Gastroschisis or exomphalos? An unusual abdominal defect.

A baby with an antenatal diagnosis of exomphalos was born at term. The abnormality had a highly unusual appearance, a right-sided paraumbilical defect, similar to gastroschisis, but with a sac typical of exomphalos containing both abdominal contents and over 1 L of serosanguinous fluid. The sac was drained and suspended from the top of the incubator in silo fashion. Definitive closure was achieved at day 3 of life, and the patient had a non-eventful recovery. Despite exomphalos and gastroschisis normally being discrete entities, ambiguity in the presentation this case necessitated a composite management approach.

Caregiver evaluation and satisfaction with autologous bowel reconstruction in children with short bowel syndrome.

BACKGROUND AND AIMS: Short bowel syndrome (SBS) describes the malabsorptive state seen following extensive bowel resection. Management aims to optimise nutritional intake, promote intestinal adaptation, and prevent the development of complications. Surgical options such as autologous gastrointestinal reconstruction (AGIR) attempt to restore enteral autonomy to the patient. Although the literature focuses on the objective measurements of success following AGIR techniques, subjective assessment of its effect on the quality of life (QoL) should also be sought. Because children with SBS are reliant on caregivers, caregivers' satisfaction with surgery and their perception of the effect of AGIR on their daily lives should be assessed. This is the first report of caregiver satisfaction following AGIR in children. METHODS: All children with SBS who had AGIR between January 1999 and June 2010 were identified. A questionnaire was developed. Caregivers were asked to complete the questionnaire to rate their satisfaction with surgery and the pre- and postoperative daily care, medical needs, and subjective interpretation of QoL for their child. Data were analysed using SPSS version 18, using the Friedman nonparametric test and 2-way analysis of variance by rank. Statistical significance was set at ≤0.05. RESULTS: Thirty-two children were identified. Seven assessments were done outside our centre (5 outside the UK) and were not included in the study. One patient moved to another city and was not contacted. Two children died. Twenty patients were contactable and were included in the present study. Parents were satisfied with the AGIR and saw improvements in their child's physical condition, bowel habit, and subjective interpretation of QoL postoperatively. Parents perceived significant improvements in the ease of caring and in aspects of their relationship with their child, including the quality of time spent, their enjoyment, and the level of their frustration with them. CONCLUSIONS: The present study found that AGIR improved the physical well-being of the child and gave the impression of improvements on specific QoL aspects for both the child and the parents.

Intestinal rehabilitation and bowel reconstructive surgery: improved outcomes in children with short bowel syndrome.

BACKGROUND AND AIM: Short bowel syndrome poses a great challenge to pediatric teams. Several innovative techniques in the management of total parenteral nutrition (TPN) and bowel reconstructive surgery have improved the outcomes of these children. The authors present their experience during the last decade as a specialist unit using improved techniques and multidisciplinary approaches in the management of this condition. METHODS: All of the children presenting with short bowel syndrome between 2000 and 2009 were identified. Diagnosis, length of residual gut, age at definitive surgery, length of gut prelengthening, length of gut postlengthening, TPN status, and survival were recorded. Median values were calculated. RESULTS: Twenty-seven children were identified (14 boys, 13 girls). Overall survival was 92%. Two children died. Nineteen children required bowel lengthening and 8 children had simple bowel reconstruction while on our protocol. Overall median age at definitive surgery was 12 months. Overall median residual gut length for these was 35.5 cm, whereas the median residual gut length for patients undergoing bowel lengthening was 25 cm. Postbowel lengthening, the median gut length was 90 cm. TPN data were unavailable for 2 patients. Overall, excluding the 2 patients who died and the 2 we have no TPN data on, of 23 patients, 21 (91%) are now off TPN. CONCLUSIONS: Our series shows improved results not only with survival but also in the number of patients that are off TPN. Multidisciplinary approach consisting of both medical and surgical expertise is necessary in the management of these patients. The authors advocate centralisation of short gut services to experienced centers with multidisciplinary expertise.

Safe use of segmental liver grafts from donors after cardiac death (DCD) in children with acute liver failure.

Emergency liver transplantation is a life-saving procedure in selected subset of children with acute liver failure (ALF), when most recipients receive a segmental graft from a living or heart-beating deceased donor. The increased use of full-liver grafts from donors after cardiac death (DCD) has had a beneficial impact on elective liver transplantation in adults. These grafts however are more susceptible to poor initial function, and most centres are reluctant to consider their use as segmental grafts, let alone in the situation of ALF where good initial function is imperative. In this short article, we describe the use and successful outcome in two children aged 6 weeks and 6 years with acute liver failure who received reduced-size DCD liver grafts.