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.

Controlled tissue expansion in the initial management of the short bowel state.

The treatment of short gut syndrome has evolved dramatically during the past decade. The combination of surgical techniques and medical management in the context of a multidisciplinary approach has improved the outcomes of these children. The authors describe in detail their technique of controlled tissue expansion of the bowel before lengthening procedures. Monitoring of the child and troubleshooting actions during the controlled tissue expansion program also are discussed.

Successful management of short gut due to vanishing gastroschisis - case report and review of the literature.

Vanishing gastroschisis is a rare in utero complication of gastroschisis. It is associated with a high mortality. We present a case report of an infant with vanishing gastroschisis that was managed with a combination of reconstructive bowel surgery and hepatosparing parenteral nutrition. The technique is described and a review of the literature is provided.

Infections in gastroschisis: organisms and factors.

This study aimed to define the incidence, causative organisms and predisposing factors leading to infection related morbidity in newborns with gastroschisis. All gastroschisis patients admitted over the 5-year period (1999-2004) were retrospectively reviewed. Surveillance samples, wound, blood, urine and fecal cultures were analyzed. Duration of total parenteral nutrition, antibiotic therapy, feeding regimes and demographic data were also analyzed. Multiple logistic regression was employed using the SPSS system and p < 0.05 was considered as significant. Seventy-two neonates were identified with 53% having abnormal gut carriage mostly due to Enterobacter and Klebsiella. Wound infection occurred in 20% of cases. Abnormal gut carriage predisposed to the development of wound infection. Line sepsis occurred in 21% of neonates. Endogenous coagulase negative Staphylococcus caused 74% of septic episodes. There was no correlation between abnormal gut carriage and the development of line sepsis. Overall survival was 96%. The cause of infections in gastroschisis patients appears to be multifactorial. A multidisciplinary team can play an important role in reducing the incidence of infections. Strict aseptic protocols and auditing practice can be the invaluable tools in decreasing morbidity rates.

Surgical strategies in the management of ecthyma gangrenosum in paediatric oncology patients.

Clinical presentation and microbiology profiles of neutropenic paediatric oncology patients presenting with ecthyma gangrenosum (EG) were studied. Surgical strategies deployed for these critically ill children are reported. Between 1994 and 2005, all children with EG were identified. Case notes were reviewed. Hospital course and long-term outcome were documented. Ten patients were identified. Eight had acute lymphoblastic leukaemia, one child had acute myeloid leukaemia and another had rhabdomyosarcoma. Lesions occurred in the perineal region (n = 5), buttocks (n = 2), thigh (n = 2) and the face (n = 1). Seven children had positive blood cultures for Pseudomonas aeruginosa. Surgery included (1) radical debridement, and (2) debridement with covering colostomy for four of those with perianal lesions. Ecthyma gangrenosum is a rapidly spreading and potentially lethal condition. Paediatric oncology patients with neutropenia are at a high risk. Surgical excision is crucial for progressive lesions to prevent mortality.

Less is best? The impact of urokinase as the first line management of empyema thoracis.

To assess the impact of intrapleural urokinase and small tube thoracostomy on the management of childhood empyema thoracis. The study population included 38 children presenting consecutively to a regional surgical unit with empyema thoracis from January 2001 to December 2003. Children with malignancy, immunodeficiency and complex intercurrent illness were excluded. Primary outcome variables were the need for second intervention and duration of stay, with other variables including duration of antibiotics, serial CRP and amelioration of pyrexia. Interventions were: tube thoracostomy (16-20 Fr) alone (n=2), tube thoracostomy (6-10 Fr)+urokinase (n=17), thoracoscopy (tube: 20-24 Fr) (n=9), thoracotomy (tube: 16-24 Fr) (n=10). There were no differences in age, weight or length of prodromal symptoms, between treatment groups. There were no differences in primary outcome variables, although no child undergoing thoracotomy required further intervention. The duration of intravenous antibiotics was similar in all groups. Amelioration of pyrexia was more rapid in children undergoing thoracotomy. There were no differences seen with regard to decline in CRP level. Small tube thoracostomy and intrapleural urokinase had a similar outcome to more invasive therapies such as thoracotomy or thoracoscopy thereby supporting the evidence base for urokinase and tube drainage as first line intervention.

Giant pulmonary cyst simulating tension pneumothorax.

Giant pulmonary cyst is a rare clinico-pathological entity. It was described as a "windy tumor" by John Floyer in 1726. Since that time, there have been numerous reports on this unique manifestation of emphysema by many authors. In this report, we report one such interesting case who was diagnosed as tension pneumothorax.

Clofibrate-induced muscular syndrome. Report of a case with clinical, electromyographic and pathologic observations.

In this report the clinical features of a case of clofibrate-induced muscular syndrome, as well as the electromyographic, histochemical, light- and electron-microscopic findings are described. The clinical features are similar to those of previously reported cases. Electromyography was consistent with a myopathy. Histochemical profile was normal. The myopathology included breakdown of contractile material, deranged mitochondria, dilated sarcoplasmic reticulum profiles, accumulation of membrane bound dense bodies, discontinuities in sarcolemma and thickening of capillary basement membrane. Macrophages invaded severely affected fibers.