Renal lymphangiomatosis, interrupted IVC with persistent primitive hepatic venous plexus and multiple anomalous venous channels: parts of an overlap syndrome?

A 5-year-old girl with cutis marmorata telangiectasia congenita (CMTC) and congenital glaucoma, who had previously presented with seizures, transient hemiplegia, upper gastrointestinal bleeding and hemihypertrophy, developed a large pleural effusion. Subsequent imaging revealed renal lymphangiomatosis, multiple anomalous intra-abdominal venous channels, an interrupted inferior vena cava with a persistent primitive hepatic venous plexus (PPHVP) and meningeal angiomas. To the best of our knowledge, the CT findings of PPHVP and the combination of the demonstrated abnormalities have not been previously reported. They may represent an overlap syndrome of CMTC, Sturge-Weber syndrome and Klippel-Trenaunay syndrome. The complexity and degree of overlap highlights the importance of an accurate clinical and anatomical description and good communication among clinicians.

High versus restricted use of home oxygen therapy, health care utilisation and the cost of care in chronic lung disease infants.

UNLABELLED: Use of home oxygen therapy for prematurely born infants with chronic lung disease (CLD) can facilitate early discharge, but affected infants might require more readmissions. Our aim was to determine if health care utilisation and associated costs in the first 2 years were greater in centres with a high compared to centres with restricted use of home oxygen therapy. A retrospective review of the hospital and general practitioner (GP) medical records of 235 infants with CLD (median gestational age 27 weeks; range 22-33 weeks) was performed to note their readmissions, outpatient attendances, community service referrals and cost of care in the first 2 years after birth. A total of 76 infants (64%) in the high use centres and 12 (10%) in the restricted use centres were discharged home on oxygen. Infants in the high use centres were discharged home from neonatal care at a younger age (median 37.7 versus 39.9 weeks; P<0.001), but subsequently had similar numbers of inpatient events, and less GP (P =0.012) and community care (P < 0.001) contacts, although their duration of home oxygen use was longer (P < 0.001). The post-discharge costs were similar in the two types of centre, but the neonatal costs (P < 0.0001) and total cost of care per infant over the first 2 years (P < 0.0001) were lower in the high use centres. CONCLUSION: Early discharge and high use of home oxygen therapy was not associated with an increased cost of care or increased morbidity.