A case of calcification of the umbilical vein in a premature infant with nephrocalcinosis and cholelithiasis is described. Such an association might have resulted from prematurity, bronchopulmonary dysplasia, umbilical vein catheterization, total parenteral nutrition, and furosemide therapy. Follow-up ultrasound examination at 9 months of age revealed spontaneous resolution of calcification of both the gallbladder and the umbilical vein but not of the kidneys.

Congenital hepatic arterio-venous malformations (AVM) are rare vascular anomalies and have rarely been reported in the presence of congeni-tal heart disease. The reported cases are mostly hemangiomas fed either by the hepatic artery itself or by one of its branches. We present two unique hepatic AVM cases in the presence of congenital heart defects in which the AVM was not fed by the hepatic arterial system. Transcatheter coil embolisation was successfully carried out in both of them by using non-detachable Gianturco coils. Complete occlusion was achieved without any sequel

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IntroductionIIH is characterised by raised intracranial pressure mostly affecting young females. It can lead to severe and irreversible visual loss. There is little consensus or evidence based guidance for management, especially in children. We conducted a multi-centre retrospective service evaluation to better understand current practice.MethodsChildren diagnosed with IIH from nine centres in the UK over a 3 year period were retrospectively studied. Patient demographics, clinical details at presentation and follow-up, comorbidities, investigations, and medications were recorded and analysed.Results105 patients (71 females) with IIH, median age 11 [IQR 5–13] were recruited. Overall, record keeping was suboptimal with no recorded weight in 33% (43/105), height in 64% (68/105); and visual acuity in 24% (26/105).At presentation 42/62 (67%) of the patients had weight >90 th centile, behavioural issues were reported in 15/63 (23%) and missing school in 9/43 (20%). Papilloedema was reported in 98/105 (93%) at baseline and in 87/105 (82%) at 6 months. Median GA opening CSF pressure was 34 (IQR 28 to 40) cm H2O and non-GA was 32 (IQR 25 to 39). 1/105 (0.9%) had ICP monitoring and 1/105 (1%) had shunt.73 (69%) were seen by an ophthalmologist and 48 (45%) had no record of either orbital US scan or OCT. Only 16 (15%) had MRV and/or MRA. 51 (48%) had LP under GA, of whom only 10 (19.6%) had CO2 monitoring recorded. 92/105 (87%) received acetazolamide, however 25/92 (27%) had no electrolytes checked during acetazolamide therapy.ConclusionsThis project highlights significant variation in the way that IIH is being investigated and managed in the UK. This variation is likely to reflect the paucity of evidence, but risks over-diagnosis and inappropriate management. These results should be used to form the basis for a national consensus for the diagnosis and management of IIH in young people.