Ethna Phelan

Early magnetic resonance imaging findings in Menkes' disease.

6. Erba G, Cavazzuti V: Sturge-Weber syndrome: Natural history and indications for surgery. J Epilepsy 1990;3(Suppl):287-291. 7. Sperner J, Schmauser I, Bittner R, et al: MR-imaging findings in children with Sturge-Weber syndrome. Neuropediatrics 1990;21:146-152. 8. Decker T, Jones K, Barnes P: Sturge-Weber syndrome with posterior fossa involvement. AJNR Am J Neuroradiol 1994; 15:389-392. 9. Elster AD, Chen MYM: MR imaging of Sturge-Weber syndrome: Role of gadopentetate dimeglumine and gradient-echo techniques. AJNR Am J Neuroradiol 1990;11:685-689. 10. Lipski S, Brunelle F, Aicardi J, et al: Gd-DOTA-enhanced MR imaging in two cases of Sturge-Weber syndrome. AJNR Am J Neuroradiol 1990;11:690-692. 11. Schwartz R, Carvalho P, Alexander E III, et al: Radiation necrosis versus high-grade recurrent glioma: Differentiation by using dual-isotope SPECT with TI and Tc-HMPAO. AJNR Am J Neuroradiol 1991;12:1187-1192. 12. Legido A, Price ML, Wolfson B, et al: Technetium Tc-HMPAO SPECT in children and adolescents with neurological disorders. J Child Neurol 1993;8:227-233. 13. Maria B, Drane W, Quisling R, et al: Value of thallium-201 SPECT imaging in childhood brain tumors. Pediatr Neurosurg 1994;20:11-18. 14. Chugani H, Mazziotta J, Phelps M: Sturge-Weber syndrome: A study of cerebral glucose utilization with positron emission tomography. J Pediatr 1989;114:244-253. 15. Drane W, Abbott F, Nicole M, et al: Technology for FDG SPECT with a relatively inexpensive gamma camera. Radiology 1994;191:461-465. 16. Norman MG, Schoene WC: The ultrastructure of Sturge-Weber disease. Acta Neuropathol 1977;37:199-205.

Gas reduction of intussusception.

Efforts to improve the non-surgical management of childhood intussusception centre around (a) reassessment of selection criteria used to ensure as many children as possible have the advantage of hydrostatic reduction, and (b) improvements and modifications of enema technique to ensure successful and safe reductions without increased morbidity. Reports that pneumatic reduction was highly successful in treating childhood intussusception prompted the authors to evaluate this technique over an 18 month period using our previously reported technique of oxygen at 2 litres/minute and a pressure of 80 mm Hg. Pneumatic reduction was attempted in 114 of 129 consecutive cases of intussusception, and was successful in 85 (75%). Fifteen patients (8.6%) were considered unacceptable risks for gas reduction using our current selection criteria and had primary surgery. The overall success rate considering all cases of intussusception managed at our institution over this period was 66% (85/129). As with any form of hydrostatic reduction, pneumatic reduction of intussusception requires careful selection of patients, meticulous technique, and awareness of complications and their appropriate management. Because of its simplicity and improved success rate, pneumatic reduction has replaced traditional barium reduction at our institution. It may be that with further evaluation of selection criteria, higher pressures, and prolonged attempts that results will improve further.

Urinary tract abnormalities in association with oesophageal atresia: frequency, significance, and influence on management

Urinary tract abnormalities occur in about 24% of infants with oesophageal atresia. It is important in the neonatal period to recognise those that may cause renal damage if untreated so that the long-term complications of renal disease, e. g., reflux-associated nephropathy, can be prevented. Our current practice is to obtain a renal ultrasound and micturating cystourethrogram during the first admission. If the infant has not been observed to pass urine, then renal ultrasound should be performed before repair of the oesophageal atresia, because there is inadequate functioning renal tissue for long-term survival in 3% of oesophageal atresia patients. The absence of Potters syndrome in infants with both oesophageal atresia and bilateral renal agenesis means that these patients often cannot be identified pre-operatively on clinical grounds alone.

Intussusception: effectiveness of gas enema

In tertiary paediatric centres, gas enema reduction of intussusception appears to be more effective and perhaps safer than barium enema reduction. Despite the higher success rate of reduction when gas is used, there remain some patients in whom the technique fails but who at operation have an intussusception that theoretically should have been reducible with the gas enema: easy to reduce manually, no necrosis of the bowel, and no pathological lesion at the leadpoint. After a gas enema failed, manual reduction of an intussusception was achieved in 41 of 49 patients and was difficult in less than one-half. It is suggested that a significant proportion might have been reducible had the technique of gas reduction been modified, either by repeating the enema some hours after the first attempt or by using higher insufflation pressures. In practice, clinical features were not useful in predicting in which patients gas enema would be unsuccessful or whether operative reduction would be successful without resection.

Colour Doppler imaging as an aid to excision of an arteriovenous malformation

Colour Doppler ultrasound was used to localise an arteriovenous malformation (AVM) and its major feeding vessel intraoperatively. The successful ligation and division of this vessel could be confirmed with confidence on colour Doppler ultrasound. The malformation immediately became less turgid and was completely excised. Colour Doppler ultrasound was a valuable adjunct in the localisation and excision of this AVM and has application to AVM surgery in general.

Intussusception: recurrence following gas (oxygen) enema reduction

Over a 38-month period, prospective data were collected on all episodes of intussusception treated at the Royal Childrens Hospital, Melbourne. There were 170 initial episodes for which a gas enema was performed, 127 of which were reduced successfully. Recurence occured in 10 patients following initial successful gas enema reduction, a recurrence rate of 7.9%, which compares with a recurrence rate of 8.9% with barium (P = 0.896). We conclude that the gas enema does not have a higher rate of recurrence than barium and that there is no evidence of a significant incidence of incomplete reduction unrecognised at the time of gas enema.

Intracranial arteriovenous malformations in childhood: presentation, management and outcome

Cases of true intracranial arteriovenous malformations (AVMs) presenting over a 25 year period were reviewed in order to achieve a better understanding of the behaviour and management of AVMs in children. There were 69 cases, presenting with haemorrhage (78%), seizures (13%) cardiac failure (3%) and focal signs with or without headache (6%). It was less common to present under six years of age. CT scanning, where performed, always demonstrated an abnormality, but this was suggestive of an AVM in less than one third. By contrast, angiography defined the lesion in 82% of initial studies. 59 patients underwent a surgical procedure directed at their AVM or an associated haematoma. Total AVM excision was obtained in 65%, with none of these later rebleeding. Three patients presenting solely with seizures were not operated upon initially, but underwent successful resections of their lesions after later haemorrhages. There were 6 (9%) deaths in the series, with focal deficits in 52% of survivors at last follow up. In the operative group these figures were 3% and 51%, respectively. None of the eight patients operated upon prior to a clinical bleed suffered a new neurological deficit. The role of stereotactic radiosurgery, although not used in any of our cases, is discussed. We believe that our results support an aggressive surgical approach to childhood AVMs, regardless of presentation, given the significant risk of morbidity from a later bleed, and the lack of a clearly better outcome with expectant management or irradiation.

Choroid plexus angioma: A rare cause of cerebral hemorrhage in childhood

An eight year old boy, previously in good health, developed sudden generalised headache with nausea and vomiting. On examination, he was mildly drowsy with moderate meningism. His pulse rate was 92/minute, blood pressure 115/50 mmHg, and he was afebrile. There were no focal neurological signs. A lumbar puncture revealed evenly bloodstained cerebrospinal fluid. Computerised tomography (CT) revealed an intracerebral haematoma in the right parietal lobe adjacent to the lateral ventricle, with blood within the ventricles and basal cisterns (Fig. 1). Initial management was conservative, Cerebral angiography was performed seven days after presentation. Right and left carotid angiograms showed subtle asymmetry between the choroidal vasculature, with an increase in the number of small arteries, which were abnormally tortuous, and a more prominent choroidal blush in the choroid plexus of the right lateral ventricle (Figs. 2, 3). There was no evidence of early filling or enlargement of drainingveins. A left vertebral angiogram showed no abnormality. An angioma of the right choroid plexus was diagnosed on the basis of these findings. Eight days after the initial haemorrhage, a CT-guided stereotactic right parieto-occipital craniotomy was performed. The intracerebral haematoma, which extended into the right lateral ventricle, was evacuated via a posterolateral transcortical approach. Abnormal, tortuous vessels were identified in

Imaging of liver tumours in childhood

Primary liver tumours account for 6% of all paediatric neoplasms. In a child with a clinical abdominal mass, imaging (in consultation with a paediatric surgeon) aims to confirm the intrahepatic site, determine its likely resectability, exclude metastatic abdominal disease, and characterise the mass. The imaging in 44 patients with primary liver tumour over a 33-year period was reviewed and correlated with surgical/pathological findings. Characterising hepatic masses with ultrasound, computed tomography, nuclear medicine, and angiography is less important than determining its resectability and alerting the surgeon to vascular anomalies and the presence of metastatic disease. We conclude that a chest X-ray and ultrasound study are the primary methods for evaluation of a child with suspected hepatic mass. With careful attention to technique, the mass can be evaluated and an assessment made of tumour resectability preoperatively. Based on this review, we propose a schema for the initial evaluation of suspected hepatic masses in children.