No abstract

The aim of this study was to evaluate contemporary patterns of presentation and trends in the management and outcome of newborn infants with jejuno-ileal atresia at a regional paediatric surgical centre in the United Kingdom. The hospital neonatal surgical registry was used to identify patients with jejuno-ileal atresia (n = 83) admitted between 1976 - 1998, excluding those associated with gastroschisis. The clinical records were reviewed and antenatal information, patient demographics, associated anomalies, operative treatment, post-operative management and outcomes were analysed in three time periods to identify trends in management and survival: Group 1 1976 - 1982 (n = 32), Group 2 1983 - 1990 (n = 21), and Group 3 1991 - 1998 (n = 30). Overall survival was 90 %. The number of patients with associated anomalies were Group 1, 10 (31 %); Group 2, 7 (33 %); and Group 3, 11 (37 %). Cystic fibrosis was encountered in 4 (13 %), 1 (5 %) and 4 (13 %) patients, respectively. Resection with primary anastomosis was the definitive management in most of patients: Group 1, 25 (78 %); Group 2, 17 (81 %); and Group 3, 27 (90 %). Initial stoma followed by delayed primary anastomosis was performed in 14 infants; eight patients had divided stomas while 6 had Bishop-Koop stoma. Tapering was used in 10 patients (12 %) with proximal jejuno-ileal atresia. Parenteral nutrition was increasingly utilised over the three time periods studied. There were no deaths in Group 3 compared to 6 deaths in Group 1 and 2 in Group 2 (P = 0.02). Most of the deaths were due to overwhelming sepsis. Mortality did not correlate significantly with the TYPE of atresia, presence of associated anomalies or the need for long-term total parenteral nutrition. The overall complication rate in survivors was 18 %. In the infants undergoing Bishop-Koop operation the complication rate was 50 %. This study has shown a significant reduction in mortality from jejuno-ileal atresia, which may be attributed primarily to advances in perioperative management, including parenteral nutrition. Generous resection of the atretic segment with primary anastomosis is more frequently employed in preference to initial stoma formation. Cystic fibrosis remains an important co-morbid condition that must be excluded promptly in all newborns.

A case of spontaneous biliary perforation is reported for the following noteworthy aspects: its rare mode of presentation as an acute intestinal obstruction; its presentation in late infancy; and its association with Ivemark syndrome.

Haemangiomas and vascular malformations of the limb in children are often difficult to manage. The role of surgery and intervention in current management is still not clear. The aim of this study was to review our experience of such patients. Patients were identified using the health board database of inpatient admissions between 1999 and 2003. Clinical notes were reviewed and data collected looking at patient demographics, site of lesion, clinical findings, investigative procedures, intervention and follow-up. A total of 288 patients were identified with a diagnosis of "Haemangioma" or "vascular malformation". Thirty-eight of these patients were found to have limb haemangiomas or vascular malformations (20 boys and 18 girls). The median age at referral was 1.9 years (0-13.2). The clinical presentation included gigantism, swelling, bruising, bleeding and cosmetic concern. Cosmetic concerns and bleeding being the most frequent. Investigative modalities were used in 19 patients. These included ultrasound, CT, MRI and angiography. Many patients had more then one imaging modality employed. Treatment options included observation, steroids, laser, embolisation and surgical excision. Twenty-five patients (66%) required surgical intervention. Most patients were required one or two procedures, mainly laser or simple excision. However two patients required multiple procedures while one patient with Kasabach-Merritt syndrome required a limb amputation. Patients were followed-up for a median of 3.75 years (0.08-14). Two patients were lost to follow-up. Two patients were noted to have related psychological problems. Haemangiomas and vascular malformations of the limb can be associated with significant functional impairment, especially if they are extending to deeper tissue planes. Such patients are likely to require surgical intervention. For many patients referred to tertiary care, surgery is still the mainstay of treatment and the only curative option. Serious complications were more common in patients with arterio-venous malformations and Kasabach-Merritt syndrome. Two of 38 patients (5.3%) were referred for formal psychological assessment. Given the potential for psychological morbidity we fear this is an aspect of care that may be under-recognised and require further specialist input.

A short survey of the literature suggests that both complications are extremely uncommon. The possible mechanism of development of cerebral oedema through stretch of the cerebral vasculature before ventricular decompression is discussed. This report further adds to the list of complications following surgery for Chiari malformations.