Short bowel syndrome: a practical pathway leading to successful enteral autonomy.

BACKGROUND: Short bowel syndrome is a multisystemic disorder that results from the loss of a significant amount of small bowel. The goal of treatment in these patients is to achieve complete enteral autonomy while minimizing complications. Our unit has 30 years of experience in the management of short gut patients. During the past decade, our results have improved significantly, especially in children with severe short bowel syndrome. This brief communication looks at the algorithm presently used in our unit. METHODS: In this communication, the principles in management of short bowel syndrome in our unit are discussed. In addition, our algorithm is published for the first time. A brief summary of our results is provided. RESULTS: Twenty-seven children were enrolled from 2000 to 2009. In this cohort, two patients died because of significant liver disease: one after having two liver and bowel transplants. Overall, survival stands at 92%. All had autologous gastrointestinal reconstruction, and 19 patients underwent bowel lengthening (longitudinal intestinal lengthening and tailoring). The median residual length of bowel of this subgroup at first operation was 25 cm in those who had their gut measured. Two patients were lost to follow-up. Two patients remain on supplemental total parenteral nutrition (TPN), with an overall 91% of surviving patients off TPN at a median of 6 months after reconstruction. CONCLUSIONS: We believe this improvement is related to the development-over many years-of a structured pathway for managing these patients.

Value of liver herniation in prediction of outcome in fetal congenital diaphragmatic hernia: a systematic review and meta-analysis.

OBJECTIVES: Intrathoracic liver herniation (ILH) is being used to estimate prognosis and hence guide antenatal interventions in fetal congenital diaphragmatic hernia (CDH). However, the literature regarding its utility in this role is conflicting. This review systematically examines the currently available evidence of ILH use in fetal CDH. METHODS: MEDLINE and EMBASE databases were searched for the terms ((congenital diaphragmatic hernia) OR CDH) AND liver. Inclusion criteria were human case series of fetuses diagnosed with CDH using either ultrasound or magnetic resonance imaging. Included studies were required to have reported the antenatal liver position and the outcome (survival or not). Case reports, reviews and eventration series were excluded. Studies reporting similar cases from the same center over an overlapping time period were considered duplicates; only the larger of the studies were therefore included. Absolute totals were extracted and sums calculated. Fisher's exact test (FET) was used to compare survival rates in different groups. RESULTS: The original search retrieved 338 studies. Applying inclusion/exclusion criteria and removing duplicates left 21 case series in 20 studies. Retrieved studies differed in the definitions of liver herniation, survival and treatment modality. In total, there were 407 fetuses in the liver-up (herniated) and 303 in the liver-down (not herniated) groups. Survival rates were 45.4% and 73.9%, respectively. The difference was statistically significant (FET = 56.4, P < 0.005). Sensitivity analysis for cases that had only conventional postnatal treatment was still significant (FET = 52.8, P < 0.005). CONCLUSIONS: Liver herniation is associated with poorer prognosis in fetal CDH. Grading liver herniation or using it as part of a panel of markers may enhance the value of liver herniation as a prognostic test in fetal CDH.

"Hernia-itis", a case report.

We describe a case of an incarcerated hernia in which there was good evidence that infection was the primary aetiology. We propose the term hernia-itis to describe similar cases to facilitate identifying them in the literature.

How useful is the lung-to-head ratio in predicting outcome in the fetus with congenital diaphragmatic hernia? A systematic review and meta-analysis.

OBJECTIVE: Fetal surgery to improve lung growth comprises tracheal occlusion in selected 'high-risk' fetuses with congenital diaphragmatic hernia (CDH). Sonographically measured fetal lung-to-head ratio (LHR) is utilized to recruit candidates for fetal surgery. This study provides a meta-analysis of the evidence regarding the prognostic use of lung-to-head ratio measurements in fetal CDH. METHODS: MEDLINE, SCOPUS and ISI PROCEEDINGS databases were searched for MeSH terms: lung, head, hernia and ratio. References in retrieved studies were also searched. Studies were categorized as follows: Phase I studies measured normal fetal LHR; Phase II studies compared fetal LHR in CDH survivors and non-survivors (if LHR informed therapy decisions or LHR was not measured during the window for intervention (< 32 weeks' gestation), studies were excluded); Phase III studies used LHR to guide selection for fetal surgery (non-randomized trials were excluded); Phase IV studies measured CDH survival before and after LHR application in clinical practice. RESULTS: The one Phase I study showed that LHR varied substantially with gestation and technique. No complete studies met the selection criteria for Phase II: meta-analysis of subgroups revealed similar LHR in CDH survivors and non-survivors. A single Phase III study revealed no benefit for LHR-directed fetal surgery. No Phase IV studies were identified. CONCLUSION: The prognostic use of LHR in fetal CDH entered clinical practice prior to publication of robust normal data and is not supported by current evidence. Application of a structured approach to any 'new' prognostic test could improve its validity and clinical application.

Surgical management of inflammatory bowel disease.

AIM: To evaluate the outcome and morbidity after major surgical interventions for inflammatory bowel disease (IBD). METHODS: Retrospective case note analysis of 227 children referred to a tertiary referral centre between 1994 and 2002 for treatment of IBD. RESULTS: 26 of 125 children with Crohn's disease (21%) required surgical management. 13 with disease proximal to the left colon underwent limited segmental resections and primary anastomosis, without significant morbidity. Primary surgery for 13 children with disease distal to the transverse colon included 6 subtotal-colectomies or panprocto-colectomies. All seven children undergoing conservative segmental resections (three with primary anastomosis, four with stoma formation), required further colonic resection or defunctioning stoma formation. All three children undergoing primary anastomosis developed a leak or fistula formation. 22 of 102 children with ulcerative colitis (22%) required surgery. Definitive procedures (n = 17) included J-pouch ileoanal anastomosis (n = 11), ileorectal anastomosis (n = 2), straight ileoanal anastomosis (n = 3), and proctectomy/ileostomy (n = 1). Five children await restorative surgery after subtotal colectomy. Median daily stool frequency after J-pouch surgery was 5 (range 3-15), and 10 of 11 children reported full daytime continence. All three children with straight ileoanal anastomosis had unacceptable stool frequency and remain diverted. CONCLUSION: The complication rate after resectional surgery for IBD was 57% for Crohn's disease, and 31% for ulcerative colitis. In children with Crohn's disease, limited resection with primary anastomosis is safe proximal to the left colon. Where surgery is indicated for disease distal to the transverse colon, subtotal or panproctocolectomy is indicated, and an anastomosis should be avoided. Children with ulcerative colitis had a good functional outcome after J-pouch reconstruction. However, the overall failure rate of attempted reconstructive surgery was 24%, largely owing to the poor results of straight ileoanal anastomosis.

Surgical implications of the Smith-Lemli-Opitz syndrome.

Smith-Lemli-Opitz syndrome (SLOS) is a syndrome of rare multiple congenital anomalies/mental retardation associated with low plasma cholesterol levels. Prior to receiving the diagnosis of SLOS, affected children may present as a neonatal surgical emergency with ambiguous genitalia, Hirschsprung's disease, and pyloric stenosis. We present two fatal cases of SLOS with near-total Hirschsprung's disease; the surgical, anaesthetic, and medical aspects of the cases are discussed, and a literature review is presented.