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Notes: Abstract PURPOSE: The postradiation preoperative staging results of 25 patients with rectal cancer who were found to have Stage T0,N0 lesions after surgery were examined. Our aim was to assess the ability of preoperative staging following radiation therapy to predict the absence of disease. METHODS: From 1983 to 1994, 25 patients treated with preoperative radiation therapy for biopsy-proven rectal cancer were found to have no pathologic evidence of disease in the resected specimen (T0,N0). The preoperative postradiation disease staging results of these patients were compared with the postoperative pathologic findings. Each patient received 4,500 to 5,580 cGy during a five-week to six-week period, and four patients had preoperative chemotherapy. Surgical resection was performed six to eight weeks after completion of radiation therapy. All 25 patients were staged by digital rectal examination before surgery. In addition, 13 patients were assessed using computed tomography, 6 by endorectal ultrasound, and 1 by magnetic resonance imaging. RESULTS: Most irradiated lesions were overstaged by radiologic assessment and physical examination. No technique could reliably distinguish between postradiation fibrosis and residual cancer. The negative predictive value for digital rectal examination was 24 percent. Computed tomography accurately staged 23 percent of lesions, and endorectal ultrasound predicted 17 percent of lesions correctly. The single patient evaluated by magnetic resonance imaging was overstaged and thought to have a T2 lesion. CONCLUSIONS: Our ability to assess local eradication of rectal cancer following radiation therapy remains poor. Conventional imaging and clinical examination techniques are unable to safely predict which patients do not require surgical excision following curative radiation therapy for rectal cancer.

Notes: Abstract PURPOSE: We have investigated the use of anorectal manometry to distinguish encopretic-constipated children (n=88) from sibling controls (n=16) and nonsibling controls (n=11). METHODS: Study variables included manometrically determined resting and maximum voluntary anal sphincter pressure, depth and speed of rectoanal inhibitory reflex, minimum rectal volume sensation, critical distending volume for fecal urgency, rectal and anal pressure responses during attempted defecation, and ability to defecate a water-filled balloon. RESULTS: Change in anal sphincter pressure during attempted defecation (P=0.03), gradient between rectal and sphincter pressure during attempted defecation (P=0.02), critical distending volume for fecal urgency (P=0.02), and ability to defecate a water-filled balloon (P=0.05) distinguished encopretic-constipated from control children. The change in rectal pressure associated with the rectoanal inhibitory reflex just escaped significance at P=0.07. CONCLUSIONS: Anal sphincter spasm and megacolon are pathophysiologic abnormalities associated with pediatric constipation-encopresis.

Notes: Abstract This article reports the observation that there is a left/right asymmetry of the anterior columns of the fornix in the human brain. This asymmetry is present in the position of the two columns of the fornix in relation to the septum pellucidum. The left columna fornicis was found to be located caudal to the right, and this can be readily visualized on axial MRI scans. This difference was seen in most of the subjects, but in some subjects there was no left/right-difference and in a few the asymmetry was inverse. The asymmetry of the fornix with respect to the anterior-posterior axis was independent of the well-known dissimilar lateral ventricular volumes. However, the left/right difference in the position of the fornix was evident in subjects with or without differences in ventricular volumes. This suggests that the mechanism underlying the development of asymmetry of the fornix is independent of the mechanism leading to ventricular asymmetry. So far, no functional relevance has been ascribed to such differences in location. The finding is gaining interest in connection with recent reports of asymmetries in hippocampal subfields. Studies of fornical lesions should therefore give attention to possible side-to-side differences.

Notes: Summary This article reports the observation that there is a left/right asymmetry of the anterior columns of the fornix in the human brain. This asymmetry is present in the position of the two columns of the fornix in relation to the septum pellucidum. The left columna fornicis was found to be located caudal to the right, and this can be readily visualized on axial MRI scans. This difference was seen in most of the subjects, but in some subjects there was no left/right-difference and in a few the asymmetry was inverse. The asymmetry of the fornix with respect to the anterior-posterior axis was independent of the well-known dissimilar lateral ventricular volumes. However, the left/right difference in the position of the fornix was evident in subjects with or without differences in ventricular volumes. This suggests that the mechanism underlying the development of asymmetry of the fornix is independent of the mechanism leading to ventricular asymmetry. So far, no functional relevance has been ascribed to such differences in location. The finding is gaining interest in connection with recent reports of asymmetries in hippocampal subfields. Studies of fornical lesions should therefore give attention to possible side-to-side differences.

Notes: Abstract We describe neural foraminal enlargement as a radiologic sign indicating bilateral interfacetal dislocation or subluxation injuries (BID/S) of the lower cervical spine on axial images. Axial neural formainal width was measured by computed tomography or magnetic resonance imaging in 10 patients with BID/S and compared with measurements in 20 control patients. In both the BID/S patients and controls, the size of the foramina at the injured level was compared with the foramina at levels immediately above and below the injury. These measurements were compared for statistical significance using Student's t-test. The width of the injured foramina averaged 10.4 mm in BID/S and 5.3 mm in controls (P〈0.001). The enlargement in the BID/S cases relative to foramina above and below the injured level was statistically significant. The sign was also assessed qualitatively for detection of BID/S by three reviewers blindly analyzing 19 cases (9 BID/S and 10 controls) for signs of widened neural foramina. The pooled blinded review yielded a sensitivity of 81% and a specificity of 83% for neural foraminal enlargement as a sign indicative of BID/S. We describe enlargement, of neural foramina as a radiologic sign corroborative of BID/S on axial images.

Notes: Abstract This article attempts to answer the most common questions on the use of diffusion magnetic resonance imaging to distinguish between acute cerebral infarction and transient ischemic attack in patients who present with the symptoms of stroke.

Notes: Abstract Pneumatoceles are an infrequent manifestation of blunt chest trauma in children. They are important to present immediately after trauma and can be mistaken for more serious surgical conditions, such as diaphragmatic rupture. In this case report, we present one such case in which we present one such case in which posttraumatic pneumatoceles were mistaken for diaphragmatic injury with bowel herniation.

Notes: Abstract The objective of this study was to investigate the value of ultrasound to establish alternative diagnoses in pediatric patients with clinical suspicion of acute appendicitis. A total of 206 consecutive patients with right lower abdominal pain were evaluated by ultrasound. For each case, the sonographic findings documented on the report at the time of examination were compared with the final diagnosis. Our results demonstrated that ultrasound was 94% accurate in the diagnosis of acute appendicitis. Among all 42 of the 108 truenegative cases of appendicitis, there was a variety of sonographic findings other than appendiceal abnormality, comprising intestinal and/or mesenteric, gallbladder, and ovarian abnormalities. Sonographic findings were well correlated with clinical and/or surgical diagnoses. Only 3 of these 42 cases were treated by surgical intervention. Ultrasound of pediatric right lower abdominal pain is a reliable diagnostic tool not only for appendicitis but for alternative diagnoses mimicking appendicitis.

Notes: Zusammenfassung Kernspintomogramm (MRI) und Computertomogramm (CT) sind geeignete Methoden zur objektiven Erfassung der Zentrierung nach offener oder geschlossener Reposition einer Hüftgelenkluxation und können in besonderen Situationen die Arthrographie ersetzen. Bis zur ausreichenden Ossifikation der Hüftkopfkerne, d. h. im 1. Lebensjahr, ist dem MRI der Vorzug zu geben. Vom 2. Lebensjahr an ist für die Beurteilung des Repositionsergebnisses das CT gleichwertig. Beim älteren Kind kann das MRI aber auch indiziert sein für die Beurteilung der knorpeligen Hüftstrukturen oder zur Diagnose einer Femurkopfnekrose. In höherem Alter können durch eine aufwendige CT-Untersuchung die knöchernen Verhältnisse auch dreidimensional dargestellt werden, z. B. zur Planung komplexer Korrekturosteotomien.

Notes: Zusammenfassung Hintergrund: Chronischer Tinnitus tritt bei Kindern und Jugendlichen mit einem normalen Ton- und Sprachhörvermögen nur selten auf. Die nosologische Einordnung dieser Störung ist auch anhand der Kriterien des ICD-10 schwierig. Aktuelle epidemiologische Daten sowie allgemein anerkannte diagnostische und therapeutische Konzepte fehlen. In der Erwachsenenmedizin haben psychosomatische Aspekte des chronisch-komplexen Tinnitus einen hohen Stellenwert. Gegenstand dieser Arbeit ist die Frage, ob dies auf Kinder und Jugendliche übertragen werden kann. Es soll ein rationelles diagnostisches Vorgehen im Hinblick auf die in dieser Altersgruppe naturgemäß schwierige Indikationsstellung für eine invasive Therapie diskutiert werden. Die Ergebnisse nach parenteraler Lidocain-Gabe als organbezogener medikamentöser Therapie werden vorgestellt. Patienten und Methode: Im Zeitraum vom Januar 1992 bis zum Dezember 1995 wurden insgesamt 31 Kinder und Jugendliche im Alter zwischen 6 und 17 Jahren ambulant und stationär wegen eines chronischen Tinnitus aurium bei normalem Ton- und Sprachhörvermögen behandelt. Der Tinnitus war in 2/3 der Fälle beidseitig lokalisiert. Bei 24 Kindern konnte anhand der systematischen Anamnese und Befundung eine in der Folge des Tinnitus aufgetretene psychische Störung ausgeschlossen werden. In diesen Fällen wurde als alleinige therapeutische Maßnahme ein Tinnitus-Counselling durchgeführt. In 7 Fällen, bei 3 Mädchen und 4 Jungen im Alter von 10–17 Jahren, lag eine Dekompensation der Beschwerden vor: Bei ihnen waren die diagnostischen Kriterien einer depressiven Episode nach ICD-10 erfüllt. In diesen Fällen erfolgte unter stationären Bedingungen für 10 Tage eine auf das organische Symptom bezogene Behandlung mit Lidocain-haltigen Infusionen in einer dem Körpergewicht angepaßten Dosierung. Die Patientendaten wurden retrospektiv-katamnestisch anhand der Krankengeschichten ausgewertet. Ergebnisse: Bei normalem Ton- und Sprachhörvermögen konnten die transitorisch evozierten otoakustischen Emissionen im gesamten Krankheitsverlauf nur unregelmäßig nachgewiesen werden. Die Ableitung der auditorisch evozierten Hirnstammpotentiale ergab keine auffälligen Befunde. Die Tinnitusbestimmung und die audiometrische Prüfung der Tinnitusverdeckung gelang nur bei den älteren Jugendlichen. In allen Fällen ohne eine Dekompensation schwanden die Tinnitusbeschwerden. Im Nachbeobachtungszeitraum von 12–44 Monaten konnte bei den mit Lidocain behandelten Patienten in 4 Fällen eine komplette Remission verzeichnet werden, in 3 Fällen eine Verminderung der Beschwerden und der Klagsamkeit bei adäquater Krankheitsbewältigung. Bei einer Patientin der Lidocain-Gruppe war wegen einer komplexeren Somatisierungsstörung die Einleitung einer Phsychotherapie erforderlich. Unerwünschte Wirkungen des Lidocains wurden nicht beobachtet. Schlußfolgerungen: Bei normalem Ton- und Sprachhörvermögen und fehlenden weiteren körperlichen Auffälligkeiten kann sich beim chronischen Tinnitus im Kindes- und Jugendalter die Organdiagnostik auf ein Basisprogramm beschränken. Die Primärdiagnostik sollte also ein Ton- und Sprachaudiogramm umfassen und nur bei einer unsicheren Hörschwelle auch die Ableitung der autitorisch evozierten Hirnstammpotentiale. Die Messung der otoakustischen Emissionen sowie die Tinnitusbestimmung und Maskierungsuntersuchungen haben nach den hier erhobenen Befunden in dieser Altersgruppe keine diagnostische oder prognostische Bedeutung. Bei fehlenden weiteren Symptomen sind weitergehende diagnostische Maßnahmen (Bildgebung, Serologie) nicht erforderlich. Die Einordnung des chronischen Tinnitus aurium als eine psychosomatische Störung bewährt sich auch im Kindes- und Jugendalter. Die diagnostischen Kriterien des ICD-10 psychischer Störungen sind eine wertvolle Strukturierungshilfe im Hinblick sowohl auf die nosologische Zuordnung wie auch insbesondere im Hinblick auf die Entscheidung zu einer invasiven Therapie, sofern nämlich die diagnostischen Kriterien einer depressiven Episode erfüllt sind. In diesen Fällen sollte eine Lidocain-Infusionsbehandlung versucht werden. In allen anderen Fällen ist ein Tinnitus-Counselling ausreichende Therapie.

Notes: Abstract  The various aspects of supportive care in children suffering from cancer, diagnosed at the Children's Hospital Aglaia Kyriakou, in Athens, are presented from the diagnosis to each step of therapy, and afterwards. It is stressed that cultural, religious and social parameters are crucial in defining the way to approach the family as a whole.

Notes: Tränenwegsoperationen an der Universitäts-Augenklinik Münster von 296 Kindern aus den Jahren von 1987 – 1992 wurden untersucht. Patienten und Methode: Alter der Patienten, Ätiologie und die Operationsmethoden wurden retrospektiv ausgewertet. Um die Operationsergebnisse und die subjektive Zufriedenheit zu erfassen, wurden an die Eltern der Patienten Fragebögen verschickt. Ergebnisse: 181 Fragebögen (Ergebnisse von 210 Operationen, davon 24 Voroperationen) wurden zurückgeschickt. 124 Kinder waren bis zu 3 Jahre alt. 197 Stenosen wurden kongenital, 7 traumatisch, 4 entzündlich und 2 iatrogen bedingt. 184 Tränenwegsspülungen mit Intubationen (I) der Tränenwege, 7 Canaliculusrekonstruktionen und 19 Dakryozystorhinostomien (DCR) wurden durchgeführt. Praktisch beschwerdefrei waren 87,5% der Patienten mit Tränenwegsintubation, 85,7% nach Canaliculusrekonstruktion und 78,9% nach DCR. 5 Patienten mußten wegen anhaltender starker Beschwerden ein zweites Mal operiert werden.

Notes: Between 1972 and 1994 21 children up to 14 years old sustained complex pelvic trauma treated at the Trauma Department of the Hannover Medical School. Sixteen of the 17 survivors were followed at an average of 8.9 years. In 8 patients operative treatment of the disrupted pelvic ring (external or internal fixation) was performed; in 8 patients the treatment was conservative. At follow-up 9 patients (56%) were pain-free; 4 reported slight, 2 moderate and one patient severe pain (at rest). There were no neurological deficits. Four patients had disturbed micturia, and 1 had bowel incontinence. Radiological evaluation showed anatomic reconstruction of the pelvic ring in 9 cases (56%). Residual maximum displacement of 12 mm was detected in 2 patients. In 3 cases osteoarthritis or ancylosis of the SI joint was present. In 3 cases a clinically not disturbing heterotopic ossification was found. Another 2 cases had ossifications of the pubic symphyses. A post-traumatic acetabular dysplasia was detected in 2 cases; a hypoplasia of the hemipelvis was seen in 3 patients. In a retrospective analysis of the primary radiographs, 13 pelvic lesions were not detected during the primary clinical course (sacral fracures, lesions of the triradiate cartilage). Despite this finding the pelvic outcome was rated good and excellent in 10 patients (63%), moderate in 1 patient (hypoplasia of the hemipelvis, and poor in 5 patients (31%) with severe pain or urogenital disturbancies. The maximum ratings in social reintegration was given to 9 patients, a medium rating to 7 patients. All patients were socially integrated.

Notes: Zur Analyse möglicher Unfallfolgen an vertebralen Bewegungssegmenten und insbesondere der Bandscheiben nach instabilen, mittels Fixateur interne versorgten thorakolumbalen Wirbelfrakturen führten wir bei 33 Patienten eine Magnetresonanztomographie (MRT) der Verletzungsregion nach der Implantatentfernung durch. Bei der Bandscheibendegeneration nach instabilen thorakolumbalen Wirbelfrakturen sind strukturelle von biochemischen Veränderungen zu unterscheiden. Die mittels MRT bestimmbare biochemische Degeneration findet sich deutlich seltener als ein morphologischer Schaden des Nucleus pulposus. Verletzungen des Anulus fibrosus, der eigentlich stabilisierenden Struktur der vertebralen Bewegungssegmente, wurden bei keinem Patienten beobachtet. Im Vergleich zur frakturbenachbarten oberen Bandscheibe ist der untere Diskus nach Ausheilung der Fraktur zwar weniger von Veränderungen der Form, jedoch häufiger von biochemischer Abnutzung und etwa gleich stark von Höhenverlust betroffen. Die Befürworter einer Resektion der oberen Bandscheibe bei der operativen Behandlung thorakolumbaler Wirbelfrakturen begründen ihr Procedere u. a. mit dem strukturellen Bandscheibenschaden, wie z. B. Höhenverlust und Impaktierung von Bandscheibengewebe in den Wirbelkörper, die in der Summe häufiger bei dem frakturbenachbarten oberen Diskus beobachtet werden. Unsere Beobachtungen, wonach eine Bandscheibe trotz morphologischer Schädigung des Gallertkerns einen dennoch biochemisch intakten Nucleus pulposus und strukturell unversehrten Anulus fibrosus aufweisen kann, sprechen eher für den Erhalt des betreffenden Diskus. Für die frakturbenachbarte obere Zwischenwirbelscheibe (seltener biochemisch degeneriert) bedeutet dies, daß die weitverbreitete Ansicht von der regelmäßigen Zerstörung und demzufolge obligaten Resektion zu überdenken ist. Die Frage, ob die untere Bandscheibe bei nachgewiesenermaßen ausgeprägter biochemischer Degeneration entsprechend häufiger reseziert werden sollte, kann durch die vorliegende Studie allein nicht beantwortet werden. Neben der statischen Beurteilbarkeit sämtlicher anatomischer Strukturen der Wirbelsäule in der MRT liefert die Wiederholung der Untersuchung in Bauchlage eine dynamische Information über das Verhalten des Rückenmarkes bei Verdacht auf dorsale Adhäsion.

Notes: Zusammenfassung Fragestellung: Das Ziel der Studie war die Validierung einer kindgerechten Modifikation des bei Erwachsenen angewandten 13C-Harnstoff-Atemtests zur Diagnose einer Helicobacter-pylori-Infektion. Methode: Bei 30 asymptomatischen Kindern wurden die Ergebnisse des Atemtests mit dem eines Helicobacter-pylori-ELISA-Tests verglichen. Bei 10 symptomatischen Kindern konnte das Atemtestergebnis mit einem histologischen Befund der Magenschleimhaut verglichen werden. Mittels Ultraschall wurde bei 7 Probanden untersucht, ob gekühlter Orangensaft als Testmahlzeit geeignet ist. Ergebnisse: Gegenüber dem serologischen bzw. histologischen Untersuchungsergebnis als Referenzmethode wies der für Kinder ermittelte Grenzwert ( δ -Wert = 5 ‰) eine Sensitivität bzw. Spezifität von 70 bzw. 95 % und 100 bzw. 100 % auf. Kalter Orangensaft war in der Lage, die Magenentleerung signifikant für die Dauer des Atemtests zu hemmen und erfüllt damit die Kriterien einer Testmahlzeit. 30 min nach 13C-Harnstoff-Einnahme war der optimale Zeitpunkt für den 2. Probesammelzeitpunkt. Schlußfolgerung: Auch in seiner kindgerechten Modifikation ist der 13C-Harnstoff-Atemtest zur Diagnose einer Infektion mit Helicobacter pylori geeignet.

Notes: Zusammenfassung Eine junge Patientin mit subakuter schmerzhafter Diplopie als Folge von entzündlichen Veränderungen der äußeren Augenmuskeln, die sequentiell beide Augen betrafen, wird vorgestellt. Das orbitale Kernspintomogramm zeigte eine Schwellung, Signalhyperintensität und Kontrastmittelanreicherung einzelner Augenmuskeln. Die Behandlung mit Kortikosteroiden führte zu einer vollständigen Remission der Symptomatik innerhalb weniger Tage. Aufgrund der Daten von insgesamt 52 gut dokumentierten Patienten mit okulärer Myositis aus der Literatur stellen bewegungsabhängige retrobulbäre Schmerzen (94%) mit Diplopie (85%) die Kernsymptome des Krankheitsbildes dar. Ein weiterer klinischer Hauptbefund ist die konjunktivale Injektion (73%), meistens an der Insertionsstelle des betroffenen Muskels. Die exophthalmische Form mit zusätzlichem Lidödem, Ptose, Chemose und Exophthalmus tritt seltener auf (24%), eine Visusabnahme ist die Ausnahme. Ein beidseitiger Augenmuskelbefall, typischerweise sequentiell, wird in 40% beobachtet. Frauen sind häufiger betroffen (73%), das mittlere Erkrankungsalter liegt bei 34 Jahren. Der M. rectus medialis ist der am häufigsten betroffene Muskel (70%). Die Diagnosesicherung gelingt heute mit der Kernspintomographie auch in leichteren Fällen. Der durch die Entzündung erhöhte Wassergehalt der befallenen Muskeln erklärt die gegenüber der Computertomographie erhöhte Sensitivität der Kernspintomographie. Therapie der Wahl ist die Behandlung mit Kortikosteroiden, hierunter kommt es in 90% innerhalb von Tagen zur Abheilung. Bei zu kurzer Therapiedauer sind Rezidive häufig. Bei Therapieresistenz ist die niedrigdosierte Bestrahlung meist effizient.

Notes: During the war period 1991–1992 in Croatia, ten wounded children (16 years of age or younger) with war injuries to the brain were admitted to the Division of Neurosurgery, Osijek Clinical Hospital. Six of them had been wounded by shrapnel and four by pistol or rifle bullets. All but one were managed surgically (i.e. by craniotomy). The outcome was: as follows three children had a good recovery, four retained a moderate neurological deficit, and three died (injured by shrapnel). Five of the wounded (four injured by shrapnels and one by bullets) had associated injuries (fractures of the leg bones, eye lesion, amputation of the right leg) which influenced morbidity, and in one case mortality. Children wounded with shrapnel had brain edema on admission to hospital. Our experience indicates that the thermal effect from heated shrapnel, as well as velocity, mass, size and shape of the shrapnel, could be an additional factor for the development of severe brain edema.

Notes: Zusammenfassung Die erfolgreiche Rehabilitation hörgestörter Kinder hängt wesentlich von der Qualität der Hörgeräteversorgung mit einer gezielten Vorauswahl des Hörgeräts ab. Daher wurden insgesamt 253 Hörgeräteüberprüfungen bei 39 schwerhörigen Kindern mit 71 HdO- Gerät-versorgten Schallempfindungsschwerhörigkeiten aller Schwerhörigkeitsgrade ausgewertet. Wir untersuchten, wie viele Hörgerätetypen angepaßt worden sind, sowie welche Fehler in welcher Häufigkeit bei der Hörgeräteüberprüfung festgestellt wurden. Gleichzeitig wurde die Effektivität und Regelmäßigkeit der in der Göttinger Klinik durchgeführten Hörgerätekontrollen ausgewertet. Eine Reduktion der zunächst 33 – meist anderenorts verordneten – Hörgerätetypen auf 23 fand statt, nachdem die entsprechenden Geräte die Erfordernisse für Art und Grad der jeweiligen Schwerhörigkeit nicht zufriedenstellend erfüllten bzw. die Akzeptanz schlecht war. Eine weitere Reduzierung dieser nicht erforderlichen, unüberschaubaren Anzahl an nicht bestmöglich ausgewählten Hörgeräten war wegen der bereits erfolgten Endverordnungen nicht mehr durchführbar. Bei 60% aller Überprüfungen konnten insgesamt 222 Mängel nachgewiesen werden: Am häufigsten war mit 59% die dynamische Hörgerätanpassung [Verstärkung (37%), Verzerrung (18%) und Dynamik (4%)], seltener mit jeweils 16% die Hörgerätfunktion und die Otoplastik [Form (9%), Material (4%) und Schlauch (3%)] und am seltensten mit 9% die Potentiometerabdeckung betroffen, die etwa zu gleichen Anteilen beim Tragen verlorengegangen war bzw. primär nicht angebracht wurde. Die qualitative Hörgerätkontrolle erfolgte nicht nur in 76% der Fälle regelmäßig, sondern war in 73% auch erfreulich effektiv. Unsere Ergebnisse weisen auf die Wichtigkeit der pädaudiologischen Qualitätssicherung kindlicher Hörgerätversorgungen hin.

Notes: Zusammenfassung Fragestellung: Ist die Bestimmung der kreatininbezogenen Ausscheidung von Deoxypyridinolin (DPD/Cr) und Pyridinolin (PYD/Cr) bei Kindern mit Störungen des Kalziumphosphatstoffwechsels sinnvoll? Patienten/Methoden: DPD/Cr und PYD/Cr wurden mittels 2er kompetitiver Enzymimmunoassays im Morgenurin bei 102 gesunden Kindern und 50 Patienten mit Knochenstoffwechselerkrankungen (Vitamin-D-Mangel-Rachitis; Phosphatdiabetes; Pseudohypoparathyreoidismus) zusammen mit der kreatininbezogenen Hydroxyprolinausscheidung untersucht. Ergebnisse: Bei Gesunden korrelierten PYD/Cr und DPD/Cr (r=0,59; p≤0,001), PYD/Cr und kretatininbezogene Hydroxyprolinausscheidung (r=0,72; p≤0,001) sowie DPD/Cr und kreatininbezogene Hydroxyprolinausscheidung (r=0,42; p≤0,001). PYD/Cr und DPD/Cr waren altersabhängig mit hohen Werten bei Säuglingen und Kleinkindern, die bis zur Pubertät abfielen. Bei allen 3 Krankheitsbildern waren PYD/Cr und DPD/Cr mit der kreatininbezogenen Hydroxyprolinausscheidung korreliert (Vitamin-D-Mangel-Rachtitis: r=0,54, p≤0,05 bzw. r=0,72 p≤0,01; Phosphatdiabetes: r=0,36, p≤0.05 bzw. r=0,60, p≤0,001; Pseudohypoparathyreoidismus: r=0,67, p≤0,01 bzw. r=0,84, p≤0,001). Bei einigen Patienten mit Vitamin-D-Mangel-Rachitis, Phosphatdiabetes und Pseudohypoparathyreoidismus konnten z.T. erheblich erhöhte Werte für PYD/Cr (Vitamin-D-Mangel-Rachitis: n=6, Phosphatdiabetes: n=14, Pseudohypoparathyreoidismus: n=4), DPD/Cr (Vitamin-D-Mangel-Rachitis: n=7, Phosphatdiabetes: n=14, Pseudohypoparathyreoidismus: n=4) und die kreatininbezogene Hydroxyprolinausscheidung (Vitamin-D-Mangel-Rachitis: n=8, Phosphatdiabetes: n=17, Pseudohypoparathyreoidismus: n=5) gefunden werden. Schlußfolgerung: Bei Kindern kann der Knochenumsatz durch die Bestimmung von PYD/Cr, DPD/Cr und der kreatininbezogenen Hydroxyprolinausscheidung bestimmt werden. Die alleinige Messung von DPD/Cr ist jedoch sehr viel einfacher, spezifischer und sensitiver als die Bestimmung der kreatininbezogenen Hydroxyprolinausscheidung und könnte in Zukunft ein wertvolles diagnostisches Werkzeug für Kinder mit Kalziumphosphatstoffwechselstörungen werden.

Notes: Zusammenfassung Fragestellung: Mittels ambulanter 24-h-Blutdruckmessung soll bei jungen Patienten mit Diabetes mellitus frühzeitig eine arterielle Hypertonie erkannt werden. Methode: Es wurden bei 48 Kindern und Jugendlichen mit Diabetes mellitus (24 Mädchen, 24 Knaben) im Alter von 9–18 Jahren ambulante 24-h-Blutdruckmessungen durchgeführt. Langzeitmessungen wurden ebenso bei einer Kontrollgruppe von 48 gesunden Kindern und Jugendlichen durchgeführt, die nach Geschlecht, Alter, Größe und Gewicht den Patienten mit Diabetes mellitus zugeordnet wurden (Matched-pairs-Technik). Ergebnisse: Keines der Kinder und Jugendlichen mit Diabetes mellitus hatte eine arterielle Hypertonie. Überraschenderweise lagen jedoch sowohl der systolische als auch der diastolische Blutdruck um 4 bzw. 6 mm Hg über den Werten der gesunden Kontrollgruppe (Methode der gleitenden Mittelwerte). Mittels konventioneller Auswertung (Bildung des arithmetischen Mittels) wurden diese Ergebnisse bestätigt (p 〈 0,001, t-Test für unverbundene Stichproben). Bezüglich der Stoffwechseleinstellung fanden wir bei Kindern und Jugendlichen mit HbA1c-Werten 〉 7 % (Median) um 5 mm Hg höhere diastolische Blutdruckwerte. Im Gegensatz zur Literatur zeigte sich in dieser Studie, daß bereits Kinder und Jugendliche mit Diabetes mellitus trotz Normotonie höhere Blutdruckwerte im Vergleich zur gesunden Kontrollgruppe haben. Schlußfolgerungen: Langzeitmessungen müssen in multizentrischen, longitudinal angelegten Studien durchgeführt werden, um Aufschlüsse über pathogenetisch bedeutsame Zusammenhänge bei der Entwicklung einer arteriellen Hypertonie bei Kindern und Jugendlichen mit Diabetes mellitus zu erhalten.

Notes: Zusammenfassung Die Heparin-induzierte Thrombozytopenie (HIT) Typ II ist ein lebensbedrohliches Krankheitsbild, bei der es unter Heparintherapie zu venösen Thrombosen und arteriellen Gefäßverschlüssen kommen kann. Bei Kindern wurde eine HIT Typ II sehr selten beschrieben. Wir berichten über einen 12jährigen Patienten, der aus völliger Gesundheit an einer tiefen Bein- und Beckenvenenthrombose ungeklärter Genese erkrankte. Er entwickelte im Rahmen einer thrombolytischen Therapie mit Streptokinase und Urokinase sowie einer begleitenden Behandlung mit unfraktioniertem Heparin eine Thrombozytopenie, multiple Embolien der linken A. femoralis und eine Thrombose der V. cava inferior. HIT-Antikörper wurden durch ELISA gegen Plättchenfaktor-4-(PF4)-Heparin-Komplexe und den Heparin-induced-platelet-activation-(HIPA)-Assay nachgewiesen. Nach sofortiger Beendigung der Heparinzufuhr wurde die Antikoagulation zunächst kurzzeitig mit einem Heparinoid und dann mit rekombinantem Hirudin fortgeführt. Danach kam es zu einer weitgehenden Besserung der Symptomatik, allerdings ließ sich eine Vorfußamputation nach Chopart nicht vermeiden. Unser Fallbericht zeigt, daß eine HIT Typ II nicht auf das Erwachsenenalter beschränkt ist. Auch bei pädiatrischen Patienten sollte bei jeder Heparinapplikation eine regelmäßige Kontrolle der Thrombozytenzahl erfolgen und bei entsprechendem Verdacht geeignete Maßnahmen eingeleitet werden.

Notes: Zusammenfassung In der retrospektiven Studie untersuchten wir 27 Kinder mit akuter Immunthrombozytopenie (ITP), die bei Diagnosestellung Blutungszeichen und Thrombozytenwerte von 〈 20 000/μl aufwiesen. Die Behandlung erfolgte mit i. v. gegebenen Immunglobulinen (IVIG) in einer Dosis von 0,4 g/kgKG. 24 h nach einer Einzeldosis IVIG hatten 19 von 27 Kindern (70 %) Thrombozytenwerte von 〉 20 000/μl erreicht. Bei 12 der 19 Kinder wurde daraufhin die Behandlung beendet und bei 7 Kindern über 2–4 Tage fortgeführt. Der Thrombozytenverlauf der 12 Kinder mit nur einer Einzeldosis IVIG unterschied sich nicht von dem der 7 Kinder, die länger behandelt wurden. Patienten, deren Thrombozyten nach der ersten IVIG-Gabe nicht innerhalb von 24 h über 20 000/μl anstiegen, zeigten trotz weiterer Therapie einen protrahierteren Krankheitsverlauf. Eine Einzeldosistherapie mit 0,4 g/kgKG IVIG erscheint uns ausreichend für die meisten Kinder mit akuter ITP, Blutungszeichen und niedrigen Thrombozytenwerten.

Notes: Abstract  A case of a mobile, calcified, intrapelvic mass representing an autoamputated ovary in an asymptomatic girl is presented. There was no previous history of acute abdominal pain indicating possible torsion of the adnexa. The diagnosis should be suspected when radiological investigations show a freely mobile, calcified intraperitoneal mass incidentally in young females with absent adnexa. The diagnostic and therapeutic role of laparoscopy in this case is emphasised.

Notes: Abstract Two children with fungal urinary tract infections (UTI) were treated with fluconazole. One had bilateral megaureters, an almost non-functioning right kidney, and a moderately functioning left kidney because of severe vesicoureteric reflux. He underwent a ureteric reimplantation procedure but developed a fungal UTI postoperatively. The other child had bilateral refluxing megaureters, one of which appeared to open ectopically into the urethra. He acquired a fungal infection during intensive and prolonged antibiotic therapy for a bacterial UTI and septicaemia in the neonatal period. Both fungal infections were cured successfully. We recommend fluconazole as a safe and effective antifungal agent that may be used as a drug of choice for this difficult problem.

Notes: Abstract Our initial experience over the last 3 years with laparoscopic and thoracoscopic surgery in children and adolescents is reported. Between September 1992 and August 1995, a total of 215 laparoscopic and thoracoscopic procedures were performed: 32 appendectomies for acute appendicitis, 10 cholecystectomies for symptomatic gallstones, 11 procedures for adnexal pathology, 6 laparoscopies in children with nonpalpable testes, 3 diagnostic laparoscopies, and 153 thoracoscopic sympathectomies in children suffering from primary palmar hyperhidrosis. The post-operative course was uneventful in all cases. In 2 children with acute appendicitis we converted to the open technique due to technical difficulties. We are encouraged by the results of our initial experience. There is no doubt that laparoscopic cholecystectomy, laparoscopic surgery of adnexal pathology, and thoracoscopic sympathectomy, because of their numerous benefits - shorter operative time, hospitalization, and convalescence as well as less postoperative pain and improved cosmetic results - are replacing the open techniques. We are not convinced as yet of the advantages of laparoscopic appendectomy in children; we are presently performing both laparoscopic and conventional techniques and studying the various parameters in order to reach a more definite conclusion. Various other endoscopic surgical procedures will be carefully considered in the near future.

Notes: Abstract  Twenty patients aged 5 months – 4 years (mean 14 months) with Hirschsprung’s disease were operated upon. In all cases a pull-through resection and stapled circular coloanal anastomosis was performed, in 11 with a single-stapling technique and in 9 with double-stapling. Isopaque rectography 4 – 10 days postoperatively showed an intact anastomosis in all 20 patients, i. e., there were no clinical or subclinical leaks. On palpation 4 – 6 weeks postoperatively, there were signs of stenosis in 4 patients. However, no. 9 – 11 Hegar dilators passed easily and there was no residual stenosis at follow-up 3 months after surgery. The technique can be recommended in children over 6 months of age.

Notes: Abstract The authors evaluated the relationship between drug intake and upper gastrointestinal (GI) bleeding. The endoscopic files of the previous 2 years were reviewed and the incidence and age and sex distribution recorded. GI bleeding is indication for a high percentage of lower endoscopies and a low percentage of upper endoscopies. On the other hand, although rarer upper GI bleeding is more severe and frequently related to drug ingestion. About 50% of cases showed gastric erosions secondary to drug intake. A relation between gastric bleeding and paracetamol is considered, as is the possibility of preventing secondary severe bleeding by pharmacologic gastric protection in children with risk factors such as chronic use of other drugs or portal hypertension.

Notes: Abstract Twenty-one consecutive laparoscopic cholecystectomies (LC) were compared with 29 consecutive open cholecystectomies (OC). Sickle-cell disease (SCD) was the most common reason for cholecystectomy in both groups. The average length of operative time for LC was significantly longer than that of OC (P=0.0149). In 1 patient there was conversion from LC to OC due to severe adhesions. Common bile duct (CBD) stones were diagnosed in 8 (27.6%) of the OC group; in 4 of them the diagnosis was made preoperatively by ultrasound, in 4 by intraoperative cholangiogram. All 8 patients required CBD exploration, and 2 had additional transduodenal sphincteroplasties. In the LC group 5 patients (23.8%) had CBD stones. All had (ERCP) endoscopic retrograde cholangiopancreatography sphincterotomy, and stone extraction followed by LC. ERCP is a necessary adjunct to treatment if LC is to be contemplated. Six patients in the OC group developed complications, while only 4 patients in the LC group developed minor complications. The length of hospitalization after LC was significantly shorter than after OC (P=0.0150). LC is the procedure of choice in the management of cholelithiasis in children, especially those with SCD.

Notes: Abstract Twenty-one consecutive laparoscopic cholecystectomies (LC) were compared with 29 consecutive open cholecystectomies (OC). Sickle-cell disease (SCD) was the most common reason for cholecystectomy in both groups. The average length of operative time for LC was significantly longer than that of OC (P = 0.0149). In 1 patient there was conversion from LC to OC due to severe adhesions. Common bile duct (CBD) stones were diagnosed in 8 (27.6%) of the OC group; in 4 of them the diagnosis was made preoperatively by ultrasound, in 4 by intraoperative cholangiogram. All 8 patients required CBD exploration, and 2 had additional transduodenal sphincteroplasties. In the LC group 5 patients (23.8%) had CBD stones. All had (FRCP) endoscopic retrograde cholangiopancreatography sphincterotomy, and stone extraction followed by LC. FRCP is a necessary adjunct to treatment if LC is to be contemplated. Six patients in the OC group developed complications, while only 4 patients in the LC group developed minor complications. The length of hospitalization after LC was significantly shorter than after OC (P = 0.0150). LC is the procedure of choice in the management of cholelithiasis in children, especially those with SCD.

Notes: Abstract The hospital records of patients who underwent splenectomy during the last 2 years were reviewed to compare the advantages of the laparoscopic approach with traditional open splenectomy (OS). Between March 1994 and March 1996, 16 children underwent splenectomy, in 8 using an open approach and 8 by a laparoscopic procedure. Of the patients who underwent laparoscopic splenectomy (LS), 2 had a concomitant cholecystectomy. Ages ranged between 4 and 11 years (mean 6.4 years); there were 9 girls and 7 boys. The indications for splenectomy were: hereditary spherocytosis (7 cases); idiopathic thrombocytopenic purpura (4); sickle-cell disease (3); and β-thalassemia (2). The average operating time for OS was 100 min (range, 50–155), for LS 170 min (range 125–240). The hospital stay for patients who had OS ranged from 3 to 9 days (mean 4.7), for those who had LS from 2 to 5 days (mean 3). One OS patient developed a wound infection. In 3 of the LS patients, the spleen was removed via a 7-cm Pfannenstiel minilaparotomy in the suprapubic region; in 5 cases the spleen was captured into an extraction bag, crushed, and removed through the umbilical orifice. The authors believe that LS must be performed only when it is possible to use the extraction bag to remove the spleen from the umbilical orifice (spleens weighing less than 700 g) and when a concomitant procedure such as cholecystectomy is indicated; in other cases OS is preferable.

Notes: Abstract A case of a mobile, calcified, intrapelvic mass representing an autoamputated ovary in an asymptomatic girl is presented. There was no previous history of acute abdominal pain indicating possible torsion of the adnexa. The diagnosis should be suspected when radiological investigations show a freely mobile, calcified intraperitoneal mass incidentally in young females with absent adnexa. The diagnostic and therapeutic role of laparoscopy in this case is emphasised.

Notes: Abstract Two children with fungal urinary tract infections (UTI) were treated with fluconazole. One had bilateral megaureters, an almost non-functioning right kidney, and a moderately functioning left kidney because of severe vesicoureteric reflux. He underwent a ureteric reimplantation procedure but developed a fungal UTI postoperatively. The other child had bilateral refluxing megaureters, one of which appeared to open ectopically into the urethra. He acquired a fungal infection during intensive and prolonged antibiotic therapy for a bacterial UTI and septicaemia in the neonatal period. Both fungal infections were cured successfully. We recommend fluconazole as a safe and effective antifungal agent that may be used as a drug of choice for this difficult problem.

Notes: Abstract The hospital records of patients who underwent splenectomy during the last 2 years were reviewed to compare the advantages of the laparoscopic approach with traditional open splenectomy (OS). Between March 1994 and March 1996, 16 children underwent splenectomy, in 8 using an open approach and 8 by a laparoscopic procedure. Of the patients who underwent laparoscopic splenectomy (LS), 2 had a concomitant cholecystectomy. Ages ranged between 4 and 11 years (mean 6.4 years); there were 9 girls and 7 boys. The indications for splenectomy were: hereditary spherocytosis (7 cases); idiopathic thrombocytopenic purpura (4); sickle-cell disease (3); and β-thalassemia (2). The average operating time for OS was 100 min (range, 50–155), for LS 170 min (range 125–240). The hospital stay for patients who had OS ranged from 3 to 9 days (mean 4.7), for those who had LS from 2 to 5 days (mean 3). One OS patient developed a wound infection. In 3 of the LS patients, the spleen was removed via a 7-cm Pfannenstiel minilaparotomy in the suprapubic region; in 5 cases the spleen was captured into an extraction bag, crushed, and removed through the umbilical orifice. The authors believe that LS must be performed only when it is possible to use the extraction bag to remove the spleen from the umbilical orifice (spleens weighing less than 700 g) and when a concomitant procedure such as cholecystectomy is indicated; in other cases OS is preferable.

Notes: Abstract Our initial experience over the last 3 years with laparoscopic and thoracoscopic surgery in children and adolescents is reported. Between September 1992 and August 1995, a total of 215 laparoscopic and thoracoscopic procedures were performed: 32 appendectomies for acute appendicitis, 10 cholecystectomies for symptomatic gallstones, 11 procedures for adnexal pathology, 6 laparoscopies in children with nonpalpable testes, 3 diagnostic laparoscopies, and 153 thoracoscopic sympathectomies in children suffering from primary palmar hyperhidrosis. The post-operative course was uneventful in all cases. In 2 children with acute appendicitis we converted to the open technique due to technical difficulties. We are encouraged by the results of our initial experience. There is no doubt that laparoscopic cholecystectomy, laparoscopic surgery of adnexal pathology, and thoracoscopic sympathectomy, because of their numerous benefits – shorter operative time, hospitalization, and convalescence as well as less postoperative pain and improved cosmetic results – are replacing the open techniques. We are not convinced as yet of the advantages of laparoscopic appendectomy in children; we are presently performing both laparoscopic and conventional techniques and studying the various parameters in order to reach a more definite conclusion. Various other endoscopic surgical procedures will be carefully considered in the near future.

Notes: Abstract The prevalence of cholelithiasis was studied prospectively by abdominal ultrasound (US) examination in 305 children with sickle cell disease aged 1–18 years (mean 10.45). Gallstones were present in 60 children (19.7%); an additional 50 had biliary sludge only (16.4%). On follow-up of 35 of the 50 children with sludge, 23 (65.7%) had developed gallstones and 5 had already had a cholecystectomy. Five continued to have sludge on follow-up while 7 were reported to have no sludge. Children with US evidence of sludge should be followed up regularly by US, and those who develop gallstones should undergo elective cholecystectomy. For those with biliary sludge only, we recommend elective cholecystectomy if there are hepatobiliary symptoms.

Notes: Abstract Over a period of 6 months, 48 children who underwent conventional appendectomy (CA) were compared to 34 children who had laparoscopic appendectomy (LA) for acute and recurrent subacute appendicitis. Their ages ranged from 4 to 12 years (mean 8). LA took significantly longer, 76 min, versus 50 min for CA. Less than 2 days' hospitalization was required in 95% of LA cases and 45% of CA cases. Time to return to normal activity averaged 7 days for LA and 12 days for CA (P 〈 0.001). Wound complications were fewer and the cosmetic appearance was most satisfactory in LA patients. LA is a safe operation that has the advantage of being exploratory, with shorter hospitalization time, early ambulation, and superior cosmetic results.

Notes: Abstract  Gorham syndrome is a rare disorder that is characterized by local osseous invasion and surrounding soft tissues by an angiomatous mass, eventually causing lysis of the affected bone. To date, only four cases have reported the MR imaging appearance of this disease and the findings have been variable. We present a case involving the cervical and thoracic spine and part of the osseous hemithorax with attention to the MR findings.

Notes: Abstract  We present a case of intraosseous neurilemmoma of the fibula in a 56-year-old woman. This case showed the typical radiographic appearance except for the presence of spotted calcifications that mimicked a cartilaginous tumor. Enhanced MR images revealed the heterogeneity of the tumor, which consisted of Antoni type A and B tissue.

Notes: Abstract  Objective. To correlate the MR appearance of the proximal femur marrow with clinical and blood parameters. Design and patients. The proportion of the femoral neck surface area occupied by red marrow was determined on T1-weighted magnetic resonance (MR) images of the hip in a series of 120 subjects, aged from 15 to 75 years, with ten females and ten males per decade, and correlated with clinical data. This parameter and the bulk T1 values of femoral red marrow were determined in 30 other subjects 25–46 years of age and correlated with their blood parameters. Results. In the series of 120 subjects, the proportion of red marrow surface area decreased with age (P〈10–4) and was higher in female than male subjects (P〈10–4). Within each decade, the proportion of red marrow surface area was higher in females than in males between 25 and 65 years but neither before 25 nor after 65 years. In the series of 30 subjects, the proportion of red marrow surface area and bulk T1 values of femoral red marrow were significantly negatively correlated with hemoglobin blood levels but not with blood cell counts. Conclusion. The MR appearance of proximal femur red marrow is influenced by age and sex. A relationship with hemoglobin blood level is demonstrated.

Notes: Abstract  Objective. To analyze the MR imaging features of fibrolipomatous hamartoma (FLH) of nerves. Design and patients. MR imaging studies from six patients (three men and three women) were retrospectively reviewed by three musculoskeletal radiologists. In four patients, a biopsy of the nerve lesion was performed. In two patients, biopsy data were unavailable and the diagnosis was based on the clinical history combined with the MR imaging findings. Results and conclusion. MR imaging demonstrated fusiform nerve enlargement that was caused by fatty proliferation and thickening of nerve bundles. Nerve bundles appeared as serpentine tubular structures, hypointense on both T1- and T2-weighted images. The degree of fatty proliferation varied among patients. In addition, significant variation in the distribution of fat along the course of the nerves was noted. In three patients, FLH followed the branching pattern of the nerves, a characteristic pathologic finding. In two patients, intramuscular fat deposition (biceps and tibialis posterior muscles) was present. MR imaging findings of FLH are typical, allowing a confident diagnosis. The variation of fatty proliferation among patients and involved nerves as well as the tendency of the abnormalities to follow the branching pattern of the nerves is well demonstrated with MR imaging. FLH may present as an isolated nerve lesion, may be associated with intramuscular fat deposition, or may occur as a feature of macrodystrophia lipomatosa (MDL).

Notes: Abstract Two experiments were conducted to investigate developmental changes in children's use of rime-level units of sound-to-spelling correspondence when spelling nonwords under varying task demands. In Experiment 1, nonwords were presented in isolation. Older children spelled more of the nonwords by analogy than younger children. Experiment 2 adopted versions of the clue word technique employed by Goswami (1988a) and found that significantly more analogous, rime-based responses were given to the same stimuli in both younger and older children. However, fewer analogous responses were given when the salience of the clue word was reduced by presentation of multiple target nonwords. The results suggest that while children in the early stages of development possess the ability to use rime-based units in spelling, they do not always make spontaneous use of this analogy strategy. However, when the potential to use rime-based units is highlighted by task demands, as is the case in Experiment 2 when a clue word is provided, even young children make considerable use of analogy in spelling.

Notes: Summary The aim of this study was to measure fetal ocular development and to determine a growth curve by means of measurementsin utero. Fetal ocular development was recorded by analysis of the results of magnetic resonance imaging (MRI). An anatomic study allowed definition of the best contrasted MRI sequences for calculation of the ocular surface. Biometric analysis of the values of the ocular surface in the neuro-ocular plane in 35 fetuses allowed establishment of a linear model of ocular growth curvein utero. Evaluation of ocular development may allow the detection and confirmation of malformational ocular anomalies such as microphthalmia.

Notes: Summary The proliferative potential of 45 pituitary adenomas was compared with their biological behaviour as determined by immunohistochemical studies, radiological findings, and clinical manifestations. The PCI (proliferating cell index) as measured using antibody MIB-1 in this study ranged from 0.05 to 4.80%, with an average PCI of 1.49±0.19% (mean±standard error of the mean). There was no significant correlation between proliferation and hormonal state, maximum size, intra-adenomatous haemorrhage, or invasiveness. However, a PCI ≧ 1.5% appeared to correlate with the likelihood of tumour regrowth (regrowth rate: 50%); for PCIs 〈 1.5%, the rate was 16%. Regrowth adenomas had a higher mean MIB-1 PCI than non-regrowth adenomas [2.34±0.58% (SE) versus 1.14±0.16%, p ≦ 0.05]. MIB-1 PCIs may provide information that is useful for planning follow-up studies and treatment after surgical resection.

Notes: Abstract A hemopoietin with the ability to accelerate both platelet and granulocyte recovery after intensive chemotherapy would have great clinical utility. The recombinant fusion protein composed of human granulocyte-macrophage colony-stimulating factor and interleukin-3 (PIXY321), showed some promise in early adult trials. However, studies for pediatric patients are limited, and there are no systematic data on the pharmacokinetics of PIXY321 given over prolonged periods at current dosage levels. Purpose: To determine the safety, clinical effects and plasma concentrations of increasing doses of PIXY321 in children treated with myelosuppressive chemotherapy. Methods: A total of 39 children with relapsed or high-risk solid tumors were enrolled in this phase I/II study. PIXY321 was administered once or twice daily by subcutaneous injection in total doses of 500 to 1000 μg/m2 per day for 14 days after each course of chemotherapy with ifosfamide, carboplatin, and etoposide (ICE). Pharmacokinetic studies were performed on day 1 of the first course in 33 patients and repeated on day 14 in 13 patients (once-daily schedule only). Results: Although mild local skin reactions and fever were frequent, no dose-limiting toxicity was identified at the maximum dose studied (1000 μg/m2 per day). There were no statistically significant differences in chemotherapy-induced hematologic toxicity with increasing doses of PIXY321 or with twice-daily vs once-daily dosing. On day 1, the median PIXY321 clearance was 657 ml/min per m2 (range 77–1804 ml/min per m2) and the median half-life was 3.7 h (range 2.1–20.8 h). On day 14, clearance increased in all patients studied (median increase 63%), with a corresponding decrease in the median 12-h concentration (from 1.2 to 0.25 ng/ml). Maximum concentrations were 〈1 ng/ml in 81% of patients, and only two patients had maximum plasma concentrations equivalent to those required for consistent activity in vitro. Conclusions: The recombinant fusion protein PIXY321 proved safe in children treated with myelosuppressive ICE chemotherapy but had no demonstrable clinical benefits. The pharmacokinetic studies suggest that the observed lack of hematologic benefit may be explained by low plasma concentrations resulting from increased clearance with prolonged administration. Moreover, the significant increase in PIXY321 systemic clearance in the absence of increased circulating myeloid cells suggests that the upregulation of either extravascular compartment hematopoietic progenitor cells or nonhematopoietic cells may play an important role in controlling circulating concentrations of this unique cytokine. These findings highlight the importance of a thorough assessment of the systemic disposition of cytokines when determining the dose and schedule necessary to achieve clinical activity in patients.

Notes: Abstract To assess the efficacy of a serotonin re-uptake inhibitor, sertraline hydrochloride, in preventing recurrent neurocardiogenic syncope, we studied 15 patients (10 female; mean age 12.9 ± 2 years) with positive head-upright tilt test and resistant to standard pharmacotherapy, atenolol or disopyramide. The patients were given 50 mg oral sertraline hydrochloride daily for 6 weeks. Intolerance to the drug was seen in 3 patients and 2 had syncopal episodes during the therapy. A head-upright tilt table test was then repeated in 10 patients. Six were tilt negative and asymptomatic over a mean follow up period of 7 ± 3 months while four remained tilt positive: two experienced marked hypotension and bradycardia, characterized as mixed type syncope, and two had cardiac asystole, lasting 〉10 s, during tilting, thereby exhibiting a cardio-inhibitory response. Conclusion Sertraline hydrochloride may be useful in preventing recurrent neurocardiogenic syncope resistant to standard pharmacotherapy but careful clinical studies are essential before such a treatment strategy can be recommended since serious asystole could develop.

Notes: Abstract Modern treatment of childhood acute lymphoblastic leukaemia (ALL) has dramatically improved the prognosis for children with this disease. Therapeutic approaches consist of multimodal chemotherapy and radiotherapy with significant long-term side-effects. We report on 4 children out of a group of 120 newly diagnosed patients with ALL, who survived the disease for more than 2 years and developed a cerebral haemorrhage after chemotherapy and fractionated cranial irradiation. Following a period of 2–12 years the four children presented with acute neurological signs and symptoms, i.e. seizures, ataxia and hemiparesis. CT and MRI revealed intracerebral mass lesions, interpreted as haemorrhage. After neurosurgery the patients neurological state improved. Histological examination confirmed the suspected diagnosis of bleeding cavernous haemangioma or capillary telangiectases. There are two possibilities to explain these rare alterations: they may be pre-existent to the disease and therapy or they may be caused by irradiation. Conclusion Acute neurological symptoms in patients treated for ALL may be caused by spontaneous cerebral haemorrhaging of cavernous haemangiomas or capillary telangiectases induced by chemotherapy and/or radiotherapy.

Notes: Abstract Serum total cholesterol (TC), high-density lipoprotein (HDL-C), low-density lipoprotein (LDL-C) and very low-density lipoprotein cholesterol, triglyceride, apolipoproteins A1 and B levels were studied in 57 healthy children and in 39 children with epilepsy who had been receiving carbamazepine (CBZ) (23 children) for 1.58 ± 1.10 years or valproic acid (VPA) (16 children) for 1.34 ± 1.11 years. In patients receiving CBZ, mean TC level, mean LDL-C level, mean TC/HDL-C ratio and mean LDL-C/HDL-C ratio were significantly higher than controls. None of the mean levels of serum lipids evaluated in patients receiving VPA was significantly different from the corresponding control group mean. Changes in serum lipids correlated with neither duration of therapy or plasma antiepileptic levels nor age or gender. Conclusion Our results suggested that CBZ, a hepatic-enzyme-inducing drug, affects serum lipid status. Long-term prospective studies are necessary to determine whether chronic CBZ therapy is a risk factor for atherosclerotic disorders.

Notes: Abstract Objective: To assess the efficacy of gastric intramucosal pH for the evaluation of tissue perfusion and prediction of hemodynamic complications in critically ill children. Design: Open prospective study without controls

Notes: Abstract Objective: To validate clinically cardiac output (CO) measurements using femoral artery thermodilution in ventilated children and infants by comparison with CO estimated from the Fick equation via a metabolic monitor. Design: Prospective, comparison study. Setting: Paediatric intensive care unit of a university hospital. Patients: 24 ventilated infants and children, aged 0.3 to 175 months (median age 19 months). Interventions: Oxygen consumption measurements were made and averaged over a 5-min period, at the end of which arterial and mixed venous blood samples were taken and oxygen saturations measured by co-oximetry, with CO being calculated using the Fick equation. Over this 5-min period, five sets of femoral arterial thermodilution (FATD) measurements were made and averaged. One comparison of CO values was made per patient. Results: Mean Fick CO was 2.55 l/min (range 0.24 to 8.71 l/min) and mean FATD CO was 2.51 l/min (range 0.28–7.96 l/min). The mean bias was 0.03 l/min (95 % confidence interval –0.07 to 0.14 l/min), with limits of agreement of –0.45 to 0.52 l/min. When indexed to body surface area, the mean Fick cardiac index became 3.51 l/min per m2 (1.52–6.98 l/min per m2) and mean FATD 3.49 l/min per m2 (1.74–6.84 l/min per m2). The mean bias was 0.02 l/min per m2 (95 % confidence interval –0.11 to 0.15 l/min per m2) with limits of agreement of –0.57 to 0.61 l/min per m2. The mean FATD coefficient of variation was 5.8 % (SEM 0.5 %). Conclusions: FATD compares favourably with Fick derived CO estimates in infants and children and may represent an advance in haemodynamic monitoring of critically ill children.

Notes: Abstract We describe a case of left-sided superior vena cava. The diagnosis was suggested by chest radiograph after central venous catheter placement. This was subsequently confirmed by magnetic resonance imaging.

Notes: Abstract Objective: To determine the combinations of nitric oxide (NO), oxygen (O2), minute ventilation (MV) and total gas flow (TGF) which generate toxic concentrations of nitrogen dioxide (NO2) during mechanical ventil

Notes: Abstract The study included 162 second-grade children (85 boys and 77 girls) aged 8–9 years, attending two schools in an area with a fertilizer production plant, and 59 second-graders of the same age (32 boys and 27 girls) from a small neighbouring town located 20 km west of the plant, without any particular source of pollution. During the period from December 1990 to May 1991 the incidence of acute respiratory diseases was surveyed in children and their family members, and forced expiratory volumes were measured in selected second-graders in December 1990 and April 1991. In the area with the fertilizer plant as well as in the compared area ammonia, hydrogen fluoride, nitrogen dioxide, total suspended particulate matter and smoke were measured daily in ambient air and inside the school buildings. The mean concentrations of pollutants during the study period were below the recommended limits, with only a few exceptions, but daily fluctuations, particularly of ammonia and hydrogen fluoride in the area around the plant happened to exceed these values. The observed differences in the levels of air pollution correlated to some extent with the health parameters followed up during the study period. The incidence of acute respiratory diseases corresponded to the registered differences in the exposure to measured pollutants. Forced expiratory volume values in the compared groups of children did not consistently reflect the differences in exposure levels.

Notes: Abstract. Various neoplastic, vascular, and developmental causes may lead to spinal neural foraminal widening, the most common causes of spinal dumbbell lesions being schwannomas and neurofibromas. Occasionally, some other slow-growing tumors may cause neural foraminal widening. We report an exceptional case of a low-grade spinal chondrosarcoma which apparently developed from a pre-existing osteochondroma in the neural arcus of C6. The lesion passed through the C5–C6 foramen, producing a dumbbell mass.

Notes: Abstract. Magnetic resonance imaging (MRI) of the breast brings the advantages of high resolution cross-sectional imaging to breast cancer diagnosis, treatment and research: improved cancer detection, staging, selection of therapy, evaluation of therapeutic response in vivo, detection of recurrence, and even the development of new therapies. Until now breast cancer treatment and research has been impeded by the limited means of evaluating the breast cancer in vivo: primarily clinical palpation and mammography of the breast tumor. A review of the initial studies shows that with the use of paramagnetic contrast agents, MRI has a sensitivity of 96 % for detecting breast cancers. MRI detects multicentric disease with a sensitivity of 98 %, superior to any other modality. The ability of MRI to detect recurrent local breast cancer in the conservatively treated breast is nearly 100 %. MRI is capable of monitoring tumor response to chemotherapy and actually guiding therapeutic interventions such as interstitial laser photocoagulation.

Notes: Abstract. The appearances of knee injuries on MR imaging are less well documented in children than adults. Some patterns of injury are shared by both groups of patients, e. g. meniscal damage. The frequency of specific injuries may differ, e. g. anterior cruciate ligament (ACL) tear. Congenital abnormality, coexistent pathology and previous treatment of the knee appear to be associated with meniscal problems. Discoid menisci are seen most frequently in children and have unique features on MR scans. Cruciate ligament tears are difficult to diagnose in the smallest children. The ACL may not be identified due to its small size. Normal bone marrow signal may be confused with marrow infiltration or bone microfracture. Radiographically occult fractures around the knee appear to be strongly associated with ligamentous injury as in adult patients. Osteochondral fractures, osteochondral lesions and articular cartilage damage are revealed on MR scans, but their long-term effects are uncertain. It is possible to diagnose a range of knee injuries on MR scans in children. The biggest diagnostic challenge is in pre-school children.

Notes: Summary. A double-blind, parallel-group trial was performed in 96 children in order to evaluate and compare the safety and efficacy of iopentol (Imagopaque®, Nycomed Imaging AS, Oslo, Norway) with those of routinely used contrast media. Ten children below 1 year of age received iopentol and eight received iohexol (Omnipaque®, Nycomed Imaging AS, Oslo, Norway) (both 300 mg I/ml) in a random manner. Seventy-eight children (39 in each group), between one and 10 years of age, received iopentol 300 or diatrizoate (UrografinØ, Schering AG, Berlin, Germany) 292 mg I/ml for urography. No adverse events were observed among the children below 1 year of age. In the group between one and 10 years there was a statistically and clinically significant difference (p = 0.007) in the incidence of adverse events between the iopentol (2.6 %) and diatrizoate (25.6 %) groups. All adverse events were of mild or moderate intensity. No serious reactions were encountered. The overall quality of visualization was judged to be diagnostic for all patients. This study supports the use of non-ionic contrast media in pediatric patients and confirms that iopentol is a safe and effective contrast medium well suited for children from 0–10 years of age.

Notes: Abstract We report a 44-year-old woman who developed an atypical retro-oesophageal abscess 4 years after anterior cervical surgery with fusion (ACSF). She presented with dysphagia but no fever or definite laboratory signs of inflammation. Delayed or chronic dysphagia following Cloward's operation is usually related to graft displacement. Infection may also, more rarely, be encountered in conjunction with dysphagia, but is typically associated with a classical clinical presentation and laboratory results. We recommend that in cases of delayed dysphagia without evidence of graft migration, the possibility of retropharyngeal infection should be considered, even in the absence of clinical signs or supporting laboratory evidence. MRI in this rare delayed complication is nonspecific but suggestive, and hence represents the imaging modality of choice in such situations.

Notes: Abstract We carried out fluid-attenuated inversion recovery (FLAIR) pulse sequences with long repetition and echo times in seven children with tuberous sclerosis, and compared them with conventional spin-echo (SE) sequences. FLAIR images exhibited higher sensitivity than conventional SE images to cortical and subcortical tubers. The low signal intensity of cerebrospinal fluid on FLAIR images allowed more accurate delineation of the cortical and subcortical tubers. However, T1-weighted imaging was still superior for delineation of subependymal nodules.

Notes: Abstract We present a case of isolated inferior sagittal sinus thrombosis shown on CT, MRI and angiography. This condition has not, to our knowledge, been described previously.

Notes: Abstract We report intrathecal use of gadolinium DTPA for MRI of the cerebrospinal fluid (CSF). In two patients with leptomeningeal carcinomatosis, we injected 0.01 mmol gadolinium DTPA into the lateral ventricle via an Ommaya device. Coronal T1-weighted images of the head were obtained at 0.2 T prior to and after injection. There was pronounced enhancement of CSF close to the injection site, allowing good delineation of CSF and surrounding brain tissue. No side effects occurred. MRI with intrathecal administration of highly diluted gadolinium DTPA may be a promising alternative to conventional investigation of CSF-filled cavities using iodinated X-ray contrast media or radionuclides.

Notes: Abstract We report MRI findings in a family with an autosomal-dominant, adult-onset neurological disorder. The clinical picture, the white matter changes detected on MRI and the absence of any laboratory abnormality suggested the diagnosis of leukodystrophy with an unknown biochemical defect. Autosomal-dominant inheritance is extremely rare in this kind of disease, and most reported families have not undergone MRI. We performed MRI and clinical examination of 17 members of our family; 9 affected subjects, at different stages of the disease, were detected. The most characteristic MRI findings were initially symmetrical areas of signal change in the white matter of the trigonal region; demyelination extending thereafter to the frontal and parietal regions, partially involving subcortical white matter; the temporal lobe and optic radiations were less involved; the internal capsule and corpus callosum were involved later, in a dorsoventral direction; patchy demyelination was evident in the late stages in the brain stem; the cerebellum was spared even in the latest stages of the disease. While pathological examination is essential to characterise and classify these kinds of diseases, MRI can make substantial contributions to understanding their natural history, and to detect early signs of the disease.

Notes: Abstract Our aim was to determine whether MRI reliably shows pathology in patients with active otosclerosis (otospongiosis). We studied five patients with clinical and audiometric signs of this disorder and positive findings on high-resolution CT and tympanocochlear scintigraphy. Contrast enhancement of otospongiotic lesions was found in all affected ears, and could be topographically related to demineralised otospongiotic foci on CT. In lesions in the lateral wall of the labyrinth MRI sometimes showed the pathology better than CT, where partial-volume effects could be troublesome.

Notes: Abstract Early MRI in a case of clinically established Reye's syndrome confirmed CT findings of compressed ventricles and additionally demonstrated signal alterations in the thalamus, mesencephalon and pons. On follow-up MRI the pontine lesion had vanished by 1 week later, while the thalamic lesion persisted for more than 2 months. The patient, however, recovered without neurological sequelae.

Notes: Abstract We used MRI for in vivo measurement of brain water self-diffusion in patients with intracranial tumours. The study included 28 patients (12 with high-grade and 3 with low-grade gliomas, 7 with metastases, 5 with meningiomas and 1 with a cerebral abscess). Apparent diffusion coefficients (ADC) were calculated in a single axial slice through the tumours; the sequence was sensitive to diffusion along the cephalocaudal axis. Our main finding was that ADC in contrast-enhancing areas within cerebral metastases was statistically significantly higher than ADC in contrast-enhancing areas in high-grade gliomas (P≤ 0.05). Furthermore, the ADC in oedema surrounding metastases were statistically significantly higher the ADC in oedema around high-grade gliomas (P≤ 0.02). The ADC in patients with meningiomas did not differ significantly from those seen with high-grade gliomas or cerebral metastases. The highest ADC were found within cystic or necrotic tumour areas. In one patient with a cerebral abscess, suspected of having a high-grade glioma, the ADC was similar to that in high-grade gliomas. The finding of higher ADC in cerebral metastases than in high-grade gliomas may be helpful in trying to distinguish between these tumours preoperatively; it suggests increased free extracellular and/or intracellular water fraction in cerebral metastases. The method seems to hold potential for further noninvasive characterisation of intracranial tumours.

Notes: Abstract Pituitary abscesses, rare lesions, may be divided into primary and secondary types. Primary pituitary abscesses occur within a previously healthy gland, while secondary abscesses arise within an existing lesion, such as an adenoma, craniopharyngioma, or Rathke's cleft cyst. Secondary abscesses share radiologic characteristics with the lesions from which they arise. There has been no review of the MRI characteristics of primary pituitary abscesses. We report two cases and review the literature. The typical primary pituitary abscess gives the same or slightly lower signal than brain on T1-weighted images, and could be mistaken for a solid mass or presumed to represent a pituitary adenoma. Contrast-enhanced images are useful, demonstrating absence of central enhancement, suggesting a fluid or necrotic center. In one of our cases, meningeal enhancement was obvious; this has not been reported previously and may be diagnostic, when associated with a rim-enhancing pituitary mass.

Notes: Abstract We performed MRI on 27 patients with clinically proven temporal lobe epilepsy (TLE), all with prior EEG lateralisation, and 10 volunteers, studied to evaluate disparity in size arising from biological variation (group 1). Three-dimensional spoiled GRASS (3DSPGR) sequences provided 2-mm contiguous sections of the limbic system, enabling assessment of the hippocampus (HC), fornix (FN) and mamillary body (MB). Measurements of FN and MB width were made from a workstation. Any percentage difference in size was computed. In 19 cases there was unilateral abnormality in the HC (group 2); in 18 and 19 cases respectively there was a smaller FN and MB on the same side as the abnormal HC. This percentage difference in size was significantly greater than that in group 1 in the FN and MB in 17 and 17 cases respectively. Comparison of percentage difference computations for FN and MB between groups 1 and 2 showed high statistical significance (P 〈 0.0002). In 5 patients with clinical TLE the HC was normal on MRI (group 3). Unequal FN and MB sizes were found in 4, significant in 2. Comparison of percentage difference computations for FN and MB showed statistical significance (P 〈 0.0005 and P 〈 0.0003 respectively). There was no case of discordance between the sides of hippocampal abnormality and the smaller FN or MB or between the sides of smaller FN and MB. The strong concordance between the changes in the HC and those in the FN and MB suggests that this combination will play an important role in the assessment of TLE and limbic system abnormality.

Notes: Abstract In ten patients who had undergone lumbar laminectomy, visual assessment of epidural scar enhancement and diagnostic confidence was performed after 0.1 mmol/kg gadodiamide intravenously, again after a further 0.2 mmol/kg, and once more using a fat-suppression sequence. The single-dose contrast-enhanced T1-weighted images showed clear enhancement of epidural scar in eight cases, and clearly improved diagnostic confidence as regards scar and/or disc herniation in six. Triple-dose contrast-enhanced images showed further increase in epidural enhancement clearly in only two cases and marginally in six, with no significant increase in diagnostic confidence. Fat-suppression, performed in eight cases, showed a further clear increase in epidural enhancement in seven cases, but again no increase in diagnostic confidence. In one patient with arachnoiditis contrast enhancement and diagnostic confidence increased only slightly after each contrast injection, and again with the fat-suppression sequence. Increasing contrast medium dose was thus not useful following laminectomy when epidural scarring obscures a possible recurrent disc herniation. Use of fat suppression may, however, permit reduction of the dose of contrast medium necessary to provide adequate scar enhancement.

Notes: Abstract We present a case of spontaneous spinal epidural haematoma (SSEH) with a rare clinical course of repeated spontaneous recovery and relapse. The patient suffered three episodes of upper-back pain of sudden onset followed by sensory and motor dysfunction after weight lifting. In the first two episodes, the neurological deficits recovered spontaneously and completely. In the last episode, paraplegia persisted even after emergency surgery. Serial studies with computed tomographic (CT) myelography and magnetic resonance imaging (MRI) demonstrated the remitting and relapsing course of the SSEHs. The possible causes of the SSEHs and the mechanisms of spontaneous recovery are discussed.

Notes: Abstract Neurosarcoma is a rare tumour originating from the sheath of peripheral nerves. Facial lesions have been reported in about 20 patients. We describe the MRI appearances of neurosarcoma with histological correlation in three patients. The lesions lay in the submandibular region, the left parapharyngeal space and the right orbit. MRI showed a well-defined mass with mixed components. The lesions were moderately heterogeneous on T1-weighted images in two cases and on T2-weighted images in all cases. Gadolinium enhancement occurred in all cases to variable degrees. In two cases, small high signal foci were seen on T2-weighted sequences. MRI appearances of neurosarcoma are not specific.

Notes: Abstract Precise assessment of the extent of nasopharyngeal carcinoma (NPC) represents the basic step towards optimal treatment. We compared the capacity of CT and MRI in assessing the extent of NPC in 67 patients. MRI was superior to CT in demonstrating lesions in the retropharyngeal node, skull base, intracranial area, carotid space, longus colli muscle and levator palatini muscle. Of 25 cases in which retropharyngeal adenopathy was recognised only on MRI, seven had been reported as showing oropharyngeal involvement and 18 as primary extension to the carotid space on CT. MRI showed skull-base involvement in 40 patients compared with 27 on CT and intracranial involvement in 38 patients versus 24 on CT. There was not a single case in which skull base invasion was seen on CT but not on MRI. MRI enabled improved recognition of tumour infiltration of longus colli muscles (34 cases compared with 15 on CT). It allowed us to clarify 12 questionable sinonasal opacities on CT. Overall, T-staging was changed in 18 of 67 patients (26.9 %), including upstaging in 15 cases and downstaging in 3 cases, after comparing CT with MRI. The nodel status was changed from negative on CT to positive on MRI in 4 of 67 patients (6 %). We believe that MRI allows more accurate evaluation of the extent of NPC than CT and should be the primary mode of investigation.

Notes: Abstract We examined five patients who had enucleation of one eye for inflammatory or neoplastic disease, using MRI at 1.5 Tesla. None had symptoms referable to the enucleated orbit. In addition, age- and-sex matched individuals were imaged as control subjects, and a further 15 subjects, referred for other than orbital disease, were reviewed. Measurements were made retrospectively of the dimensions of the optic chiasm to establish normal values. All five patients showed abnormalities on MRI following enucleation: abnormal signal within the optic nerve remnant on short τ inversion recovery (STIR) images, and atrophy of the nerve remnant and the chiasm. These findings were not apparent in the control or normal subjects. Such findings are to be expected following enucleation and should not be interpreted as indicating active pathology.

Notes: Abstract Spinal epidural extramedullary haematopoiesis is very rare in thalassaemia. A 27-year-old man with thalassaemia intermedia presented with symptoms and signs of spinal cord compression. MRI showed a thoracic spinal epidural mass, representing extramedullary haematopoietic tissue, compressing the spinal cord. Following radiotherapy, serial MRI revealed regression of the epidural mass and gradual resolution of spinal cord oedema.

Notes: Abstract Parry-Romberg syndrome is a poorly – understood disorder characterized by progressive hemifacial atrophy involving the skin, soft tissue, and bone. Involvement of the central nervous system with impairment in neurologic function occurs infrequently. We describe a child with this syndrome in whom central nervous system involvement, documented on serial MRI, played a prominent role. We have attempted to correlate the clinical course with the radiologic findings, and to determine the impact of prednisone and methotrexate on the intracranial lesions.

Notes: Abstract Lyme disease is a cause of illness involving multiple organ systems, including, in 10–15 % of cases, the nervous system. Peripheral radiculoneuritis, cranial neuritis, encephalitis and myelitis are among the neurological manifestations found in the second and third stages. We present the MRI findings in isolated oculomotor nerve involvement by Lyme disease and discuss the differential diagnosis.

Notes: Abstract MRI was performed in 13 patients with the adult form of myotonic dystrophy (MD) and compared with that of sex- and age-matched normal controls. There was some cerebral atrophy in the patients and marked thickening of the skull in three of them, associated with ossification of the falx cerebri in two. We found high-signal areas on T 2-weighted images in the white matter in 9 (70 %) of the patients; five showed high-signal areas in the subcortical white matter of the temporal lobes. These findings were associated with intellectual impairment in only one patient, who had a history of a difficult birth and temporal lobe epilepsy.

Notes: Abstract Most reports of MRI in Wilson's disease have been of abnormal low-signal lesions on T1-weighted images and high signal intensity on T2-weighted images. In contrast, we report three patients who had high-signal lesions in the globus pallidus on T1-weighted images, a finding seen in patients with portal-systemic encephalopathy. The possible causes include the paramagnetic effect of copper or iron and accumulation of Alzheimer type II glial cells.

Notes: Abstract We document the MRI features in seven patients with Japanese encephalitis. MRI was carried out on a 1.5 T system within 10–60 days of onset. In all the patients MRI revealed bilateral thalamic lesions, haemorrhagic in five. Signal changes were present in the cerebrum in four patients, the midbrain and cerebellum in three each, the pons in two and the basal ganglia in one. The lesions were haemorrhagic in three of the four patients with lesions in the cortex, two of the three with lesions in the midbrain and cerebellum, but the pontine lesions were haemorrhagic in both patients. Spinal cord involvement was seen in one of the three patients who underwent MRI. In two patients MRI was repeated 3 years after the onset, showing marked reduction in abnormal signal; and all the lesions gave low signal on both T1- and T2-weighted images. Bilateral thalamic involvement, especially haemorrhagic, may be considered characteristic of Japanese encephalitis, especially in endemic areas.

Notes: Abstract We present the MRI findings of cerebral and optic pathway damage in the acute and subacute stages of methanol intoxication. In the acute stage, CT and MRI showed bilateral haemorrhagic necrosis of the corpus striatum and infarcts in the anterior and middle cerebral arterial territories. MRI in the subacute stage demonstrated atrophy of the optic chiasm and prechiasmatic optic nerves in addition to the cerebral infarcts. The patient survived, with total blindness.

Notes: Abstract We report a 40-year-old woman, who initially developed cerebellar symptoms, with multiple hemorrhagic brain metastases from a papillary thyroid cancer. Intracranial masses gave heterogeneous high signal on T 1-weighted and T 2-weighted images, hemosiderin rims on the latter. Some of the tumors showed contrast enhancement. Metastatic thyroid cancer is a consideration in a patient with multiple hemorrhagic masses.

Notes: Abstract We prospectively analysed the normal contrast-enhanced MRI features of the facial nerve and determined criteria for pathological contrast enhancement. We studied 31 patients with clinically normal facial nerves with T1-weighted images before and after contrast medium. The intensity, thickness and right-left symmetry of enhancement were assessed in each segment and correlated with MRI features observed in abnormal facial nerves. Enhancement along at least one segment of the facial nerve was seen in 98 % of cases, but only within the facial canal: labyrinthine segment: 78.2 %; geniculate ganglion: 96.9 %; tympanic: 88.4 %; mastoid: 66.6 %. Marked (++) to intense (+++) enhancement was seen in the labyrinthine segment in 17.4 %, the geniculate ganglion in 36.3 %, and the tympanic (25.6 %) and mastoid (7.1 %) segments, whereas intense enhancement was only seen in the geniculate ganglion (6 %) and the tympanic segment (11.6 %). A right-left asymmetry was noted in 69 % of cases. No correlation was found between enhancement and the thickness of the nerve. No enhancement of the eighth nerve was seen. We suggest three criteria for pathological enhancement: enhancement outside the facial canal; extension of enhancement to the eighth nerve; and intense enhancement in the labyrinthine and/or mastoid segments.

Notes: Abstract Meningeal sarcomas are very rare, highly aggressive tumours affecting children more frequently than adults. The clinical course and MRI of meningeal sarcomas in two cases are discussed with special regard to possible misinterpretation. In one case MRI demonstrated a circumscribed mass in contact with the meninges, with central areas of haemorrhage. In the other, a case of primary leptomeningeal sarcomatosis, several MRI examinations over the course of almost a year were unhelpful, despite severe neurological complaints. Then MRI revealed meningeal contrast enhancement all over the brain and spinal canal, together with cerebral infarcts. MRI of meningeal sarcomas has not been discussed in the literature. MRI did not permit specific diagnosis, but enabled visualisation of the extent of the tumour and/or meningeal involvement. Early histological diagnosis is indispensable for adequate treatment.

Notes: Abstract Axial fast FLAIR images of the brains of 40 normal volunteers in four age groups between 16 and 55 years were examined and the number and size of areas of increased white-matter signal recorded. Increased signal in the corticospinal tract region was seen at the level of the internal capsule in all subjects, extending up towards the centrum semiovale and down towards the pons for 0.5–5.5 cm (median 2.5 cm). In all cases the IIIrd and IVth ventricles were outlined by a thin line of high signal. Focal areas of high signal (caps) were seen around the frontal and occipital horns in 90 % and 77 % respectively; 54 % of caps were asymmetrical. None of the above features varied with the age or sex of the subject, but the numbers of discrete white matter ’lesions' increased with age. The findings are used to suggest guidelines for the identification of areas of ’normal' high signal to be excluded in quantification of lesions on fast FLAIR images.

Notes: Abstract We report a 27-year-old woman with atypical sciatica due to a giant, rapidly growing, lumbar (T12-S1) meningioma. The unique features of this case are discussed and the importance of early investigation by MRI of patients with atypical low back pain and sciatica is highlighted.

Notes: Abstract We analysed the MRI findings in 23 patients with callosal dysgenesis in relation to their associated telencephalic anomalies to investigate the morphological significance of the development of Probst's bundles and the anterior commissure in congenital callosal dysgenesis. We classified callosal dysgenesis into three types: total defect (9 patients), partial defect (7) and hypoplasia (7). Associated anomalies were observed in 15 patients, including migration disorder (8 patients), micrencephaly (5), and lipoma (2). The remaining 8 patients had no associated anomalies. Probst's bundles were not identified in 4 patients with a severe migration disorder. An absent or hypoplastic anterior commissure was observed in 9 of the 16 patients with callosal defect and all 7 of those with callosal hypoplasia. Colpocephaly and keyhole dilatation of the temporal horns were seen in 16 and 21 patients, respectively. Callosal dysgenesis may occur not only through a defect in the callosal anlage, but also from impaired growth of axonal fibres projecting from the cerebral isocortex. Therefore, associated telencephalic anomalies may be responsible for additional features in callosal dysgenesis. Consequently, identification of Probst's bundles and the anterior commissure may be important when assessing cortical development in patients with callosal dysgenesis.

Notes: Abstract A review of 4,500 angiograms yielded 11 patients with dissection of the vertebral arteries who had MRI and (in 4 patients) MR angiography (MRA) in the acute phase of stroke. One patient with incidental discovery at arteriography of asymptomatic vertebral artery dissection and two patients with acute strokes with MRI and MRA findings consistent with vertebral artery dissection were included. Dissection occurred after neck trauma or chiropractic manipulation in 4 patients and was spontaneous in 10. Dissection involved the extracranial vertebral artery in 9 patients, the extra-intracranial junction in 1, and the intracranial artery in 4. MRI demonstrated infarcts in the brain stem, cerebellum, thalamus or temporo-occipital regions in 7 patients with extra- or extra-intracranial dissections and a solitary lateral medullary infarct in 4 patients (3 with intracranial and 1 with extra-intracranial dissection). In 2 patients no brain abnormality related to vertebral artery dissection was found and in one MRI did not show subarachnoid haemorrhage revealed by CT. Intramural dissecting haematoma appeared as crescentic or rounded high signal on T1-weighted images in 10 patients examined 3–20 days after the onset of symptoms. The abnormal vessel stood out in the low signal cerebrospinal fluid in intracranial dissections, whereas it was more difficult to detect in extracranial dissections because of the intermediate-to-high signal of the normal perivascular structures and slow flow proximal and distal to the dissection. In two patients examined within 36 h of the onset, mural thickening was of intermediate signal intensity on T1-weighted images and high signal on spin-density and T2-weighted images. MRA showed abrupt stenosis in 2 patients and disappearance of flow signal at and distal to the dissection in 5. Follow-up arteriography, MRI or MRA showed findings consistent with occlusion of the dissected vessel in 6 of 8 patients.

Notes: Abstract We present the MRI appearances of advanced degenerative changes at the atlanto-odontoid (AO) joint. Changes including obliteration of the joint space, subchondral sclerosis and osteophytosis were clearly depicted on fast gradient-echo T1-weighted MRI images. Recognition of these changes may be helpful in the diagnosis in patients with suboccipital pain.

Notes: Abstract We reviewed MRI of the brain and cervical spine in 11 patients with Morquio's disease. No abnormality was seen in the brain. The odontoid peg was abnormal in all patients, with varying degrees of cord compression due to an anterior soft tissue mass and indentation by the posterior arch of the atlas. The degree of cord compression was more marked than suggested by the symptoms and signs. We recommend MRI of the cervical spine in children with Morquio's disease before the development of neurological symptoms, to optimise the timing and type of surgical intervention.

Notes: Abstract We present six proven cases of chordoma of the clivus studied by CT and MRI, with special attention to the extent of the tumour and to the signal intensity after intravenous gadolinium. MRI is the best technique for assessing the extent of the tumour but CT is important for showing osteolysis. Our aim was to determine differential diagnostic neuroradiological criteria. Reliable signs of chordoma of the skull base are: posterior extension to the pontine cistern; a lobulated, “honeycomb” appearance after gadolinium; the swollen appearance of the bone in the early stages; bone erosion on CT and frequent extension to critical structures such as the circle of Willis, cavernous sinuses and brain stem.

Notes: Abstract The “dural tail” sign on gadolinium (Gd-DTPA)-enhanced MRI has been described in association with meningiomas. Various series with histopathological correlation have shown that in some cases there is tumour invasion into the dura mater, but in the majority of cases it represents a hypervascular, non-neoplastic reaction. While this sign was originally thought to be specific for meningioma, subsequent case reports have described the presence of a dural tail in other intra- and extra-axial lesions. We present a patient with a giant aneurysm arising from the P2 segment of the right posterior cerebral artery, adjacent to the tentorium, with a prominent dural tail on Gd-DTPA-enhanced MRI. In this location, differentiation of an aneurysm from a meningioma was critical.

Notes: Abstract We report the MRI and CT findings of an intracranial meningeal melanocytoma (IMM) arising from Meckel's cave and review the imaging characteristics of IMM. On CT, IMM constantly appear as well-circumscribed, isodense to slightly dense, extra-axial tumours with homogeneous contrast enhancement. This appearance is nonspecific and similar to that of meningiomas or small neuromas. On MRI, the signal of IMM is strongly related to the amount of melanin pigment: the more melanin, the more shortening of T1 and T2 relaxation times. Only when it shows as a homogeneous mass, bright on T1 and dark on T2 weighting, can a specific diagnosis of a melanin-containing tumour be made. However, this still cannot provide a distinction between IMM and malignant meningeal melanoma.

Notes: Abstract Extradural arachnoid cysts are uncommon expanding lesions in the spinal canal which may communicate with the subarachnoid space. Usually in the lower thoracic spine, they may cause symptoms by compressing the spinal cord or nerve roots. We report cases of thoracic and lumbar arachnoid cysts studied by cystography, myelography, CT and MRI. These techniques showed extradural cystic lesions containing cerebrospinal fluid, with variable communication with the subarachnoid space, causing anterior displacement and flattening of the spinal cord.

Notes: Abstract Demonstration of the full extent of abnormality in patients with the Sturge-Weber syndrome (SWS) is important for prognosis and in planning surgery to remove the seizure focus. We compared single-photon emission computed tomography (SPECT), MRI and CT in nine children under the age of 4 years with seizures as part of SWS, in an attempt to determine the optimal method of imaging in different clinical settings. Seven unilateral and two bilateral cases were studied by interictal 99mtechnetium hexamethylpropyleneamineoxime (HMPAO) SPECT, and contrast-enhanced CT and MRI, giving information on 11 abnormal hemispheres. All imaging modalities showed abnormalities in every child. Perfusion imaging showed focal regions of decreased uptake in 9 of 11 (82 %) abnormal hemispheres and demonstrated a widespread decrease but no focal defect in 2; it also revealed crossed cerebellar diaschisis in 2 cases. CT demonstrated typical gyriform calcification in 9 of 11 (82 %) affected hemispheres. Contrast-enhanced MRI showed more extensive involvement than contrast-enhanced CT in 5 of 11 (45 %) cases. The area of hypoperfusion shown by SPECT was smaller than the area of contrast enhancement on MRI in 6 of 11 cases (55 %), comparable in 3 (27 %) and larger in 2 cases (18 %). CT is sufficient to confirm the clinical diagnosis of SWS, but MRI frequently shows more extensive abnormal areas. 99mTc HMPAO imaging is a useful addition when it is important to know the full extent of the disease, for example prior to surgery. It is likely to detect areas of hypoperfusion, representing ischaemic regions, which may act as an epileptogenic focus and may not be shown by CT or MRI.

Notes: Abstract Extraskeletal mesenchymal chondrosarcoma is a relatively uncommon entity, an orbital location being extremely rare. A review of the literature revealed 16 reported cases of primary orbital mesenchymal chondrosarcoma demonstrated by plain film and CT. To the best of our knowledge, the MRI features of orbital extraskeletal mesenchymal chondrosarcoma have not been previously reported. We present the case of an 18-year-old man with a 2-year history of progressive proptosis of the right eye who underwent CT, dynamic CT, MRI without and with gadolinium enhancement, and magnetic resonance angiography of the orbits. CT of orbital mesenchymal chondrosarcoma demonstrates a well-defined mass with multiple areas of fine and coarse calcification and shows moderate contrast enhancement. The noncalcified portions of the mass demonstrate signal intensity lower than or equal to gray matter on T1-weighted images and are isointense to the gray matter on T2-weighted images. Dynamic CT reveals delayed contrast enhancement. MRI has proven to be a valuable diagnostic tool in the diagnosis and differentiation of well-defined intraorbital masses. By a combination of CT and MRI, it appears mesenchymal chondrosarcoma can be differentiated from other intraorbital lesions, such as cavernous hemangioma, hemangiopericytoma, orbital amyloidosis and fibrous histiocytoma.

Notes: Abstract We report MRI in a patient with portal-systemic encephalopathy, in which the high signal in the basal ganglia on T1-weighted images showed marked resolution after successful embolization of the intrahepatic portal-systemic venous shunt.

Notes: Abstract Cerebral alveolar echinococcosis is rare and has a poor prognosis. We report an unusual case presenting with disseminated intracranial lesions secondary to primary hepatic infection.

Notes: Abstract We report a rare case of miliary brain metastases presenting with symptoms similar to encephalitis (“carcinomatous encephalitis”). Contrast-enhanced MRI demonstrated miliary metastases more distinctly than other imaging methods and reproduced the pathological features.

Notes: Abstract We describe the case of a 52-year-old man, with cryptococcal meningitis and meningeal fibrosis who had undergone ventricular shunting. Gd-DTPA-enhanced T1-weighted MRI revealed diffuse meningeal enhancement. Remarkably, there was enhancement of the pia mater and posterior fossa subarachnoid space.