Antonino Morabito

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.

Autologous intestinal reconstructive surgery to reduce bowel dilatation improves intestinal adaptation in children with short bowel syndrome.

Objectives: Intestinal failure (IF) is a common consequence of neonatal small bowel pathology. In our experience, bowel dilatation is often responsible for the IF state in patients who fail to adapt despite adequate residual bowel length. The aim of the present study was to investigate the role of surgery to reduce bowel dilatation, and thus favour PN independence, for these children. Methods: Data were collected prospectively for all of the patients referred to our unit for a 7-year period (2004–2011). Eight patients (2 congenital atresia, 2 gastroschisis with atresia, 1 simple gastroschisis, 3 necrotising enterocolitis) with gut dilatation who failed adaptation despite a bowel length >40 cm were identified. Preoperatively, all patients were totally dependent on parenteral nutrition (PN). Patients were managed by longitudinal intestinal lengthening and tailoring (n = 3), serial transverse enteroplasty (n = 2), or tapering enteroplasty (n = 3). Results: Median age at time of surgery was 273 days (103–1059). Mean gut length increased from 51 (35–75) to 73 cm (45–120) following surgery (P = 0.02). Incidence of sepsis (P = 0.01) and peak serum bilirubin levels (P = 0.005) were reduced postoperatively. PN was discontinued after a median of 110 days (35–537) for 7 patients; 1 patient remains on PN 497 days after surgery. Conclusions: These data indicate that reconstructive surgery to reduce bowel diameter may be an effective technique for treating IF in patients with short bowel syndrome, without sacrificing intestinal length. We suggest that this technique may reduce the need for bowel transplantation in this group of 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.

Antenatal diagnosis of bowel dilatation in gastroschisis is predictive of poor postnatal outcome

PURPOSE Although gastroschisis infants usually have a good outcome, there remains a cohort of babies who fare poorly. We inquired whether the presence of bowel dilatation in utero is predictive of postnatal course in infants with gastroschisis. METHODS We compared the clinical course of infants who had bowel dilatation with those who did not. Bowel dilatation was defined as more than 20 mm in cross-sectional diameter on ultrasound at any gestational age. Outcome measures used were length of time of parenteral nutrition, death, and surgery for intestinal failure. RESULTS A review of 170 infants with gastroschisis identified 74 who had dilatation of more than 20 mm (43.5%). There was no significant difference in the incidence of intestinal atresia in those with bowel dilatation and those without (P = .07). Those with bowel dilatation spent a longer period on parenteral nutrition. There were significantly more deaths in the group with bowel dilatation (P = .01). There was no significant difference in the number of infants requiring surgery for intestinal failure between the 2 groups (P = .47). CONCLUSIONS We found that sonographically detected bowel dilatation more than 20 mm in utero in fetuses with gastroschisis may have value in predicting clinically significant adverse postnatal outcomes.

Short bowel syndrome in children: Surgical and medical perspectives

The main cause of intestinal failure in children is due to short bowel syndrome (SBS) resulting from congenital or acquired intestinal lesions. From the first lengthening procedure introduced by Bianchi, the last three decades have seen lengthening procedures established as fundamental components of multidisciplinary intestinal rehabilitation programs. Debate on indications and timing of the procedures is still open leaving SBS surgical treatment a great challenge. However, enteral autonomy is possible only with an individualized approach remembering that each SBS patient is unique. Current literature on autologous gastrointestinal reconstruction technique was reviewed aiming to assess a comprehensive pathway in SBS non-transplant management.

Spiral intestinal lengthening and tailoring — first in vivo study

INTRODUCTION Spiral Intestinal Lengthening and Tailoring (SILT) offers a new opportunity for the surgical treatment of short bowel syndrome. SILT requires less manipulation on the mesentery than the Bianchi procedure and does not alter the orientation of the muscle fibers like serial transverse enteroplasty (STEP). This study reports the first SILT results in a surviving animal model. MATERIAL AND METHODS Vietnamese minipigs (n=6) underwent interposition of a reversed intestinal segment to produce proximal small bowel dilation. Five weeks later the reversed segment was resected, and the wall of the dilated intestine was cut spirally at 45°-60° to its longitudinal axis. The bowel was lengthened longitudinally, and the spiral shaped intestinal wound was sutured. Five weeks later, the animals were explored, and the lengthened segments were measured. Haematoxylin and eosin, picrosirius, neuron specific enolase, S-100, C-kit, and immunohistochemistry were performed. RESULTS Mean lengthening was 74.8% ± 29.5% and mean tailoring (lumen reduction) was 56.25% ± 18.8%. No instances of necrosis, perforation, suture break down, or peritonitis were observed in 6/6 animals. Four of six animals recovered uneventfully with viable lengthened segments. Statistical analysis showed no significant difference in length (p=0,078) and width (p=0,182) after 5 weeks. Two animals developed bowel obstruction due to narrowed lumen, adhesion, and strangulation after 14 and 24 days of surgery. In both animals the lumen was tailored by more than 70% to less than 1.5 cm diameter. The mucosa and the muscle layers in the operated segment had become hypertrophic, but the orientation of the circular and longitudinal muscle fibres remained normal after the SILT procedure. There were no signs of chronic ischemia or collagen accumulation after the SILT. The myenteric and submucosal plexuses and the Cajal cell network appeared normal. CONCLUSION The bowel remained viable macroscopically and microscopically after SILT, such that SILT may be an alternative or an addition to the present technical repertoire of intestinal lengthening. However the limitations of tailoring should be kept in mind.

Outcomes of congenital diaphragmatic hernia: a 12-year experience

To determine outcomes for babies with congenital diaphragmatic hernia (CDH) diagnosed prenatally and postnatally presenting to a tertiary unit.

Morbidity and mortality in total esophagogastric dissociation: a systematic review.

BACKGROUND/PURPOSE Total esophagogastric dissociation has been described as both a primary and a rescue procedure for patients with severe gastroesophageal reflux. Although most commonly used in the neurologically impaired, it has also been used in those with no neurological impairment. The main objective of this study was to determine morbidity and mortality for this procedure. METHODS All published cases of esophagogastric dissociation in children were identified. Series were updated where possible by personal communication with the author. Patient characteristics, indications, morbidity, and mortality were analyzed. RESULTS One hundred eighty-one cases were identified. One hundred seventeen were primary operations and 64 were rescue procedures. There were 29 (16.0%) early complications and 28 (15.5%) late complications with 6 (3.3%) deaths related to the procedure of a total of 35 deaths. Twenty-one patients (11.6%) required re-operation in the study periods. CONCLUSIONS Esophagogastric dissociation has an acceptable morbidity and mortality. It is useful as both a primary and a rescue procedure.

Recycling of bowel content: the importance of the right timing.

INTRODUCTION Extracorporeal stool transport (recycling of chyme discharged from the proximal stoma end to the distal end of a high jejunostomy or ileostomy) is thought to be beneficial in preventing malabsoprtion, sodium loss, cholestasis and atrophy of the distal intestine until restoration of the intestinal continuity becomes possible. However little is known about its adverse effects. Our aim was to investigate the microbiological safety of recycling. MATERIAL AND METHOD Native samples were taken from the proximal stoma in 5 premature neonates who underwent an ileostomy or a jejunostomy due to necrotising enterocolitis, for qualitative culture. The first sample was drawn immediately after the change of the stoma bag, further samples were sent from the stoma bag at 30, 60, 90, 120, 150, and 180min later. The samples were inoculated by calibrated (10 μl) loops onto blood agar (5% sheep blood), eosin-methylene blue agar and anaerobic blood agar, respectively (Oxoid). The aerobic plates were incubated for 18-20 h at 5% CO2, whereas the anaerobic plates were incubated for 24-48 h in an anaerobic chamber (Concept 400). The bacterial strains were identified to species level by specific biochemical reactions, RapID-ANA II system (Oxoid) and ID32E, Rapid ID 32 Strep ATB automatic system cards (bioMérieux). RESULTS The number of colony forming unit (CFU) of Gram-negative bacteria (mainly E. coli) exponentially increased after 30 min and reached 10(5)/ml after 120 min. Gram-positive strains (primarily E. faecalis) were detected after 60 min and CFU increased to 10(5)/ml after 120 min. The number of anaerobic (principally Bacteroides fragilis) CFU started to increase after 120 min. In two cases coagulase negative Staphylococcus strains were isolated the earliest in the chyme. The average of total CFU approached 10(5)/ml after 90 min and exceeded 10(5)/ml after 120 min. CONCLUSION The chyme in the stoma bag is colonized by commensal facultative pathogenic enteral/colonic as well as skin flora species after 120 min. Recycling of stoma bag content may be dangerous after 90 min.

Proximal large bowel volvulus in children: 6 new cases and review of the literature

BACKGROUND Proximal large bowel volvulus is considered as an extremely rare surgical emergency in children. Approximately 40 cases have been reported, and because of its rarity, the diagnosis is often missed or delayed. The purpose of this study was to review the presentation, treatment, and clinical outcome of proximal large bowel volvulus. METHODS A systematic review and analysis of the data relating to 6 patients from the authors practice and cases published in the English literature from 1965 to 2010 was performed. Detailed information regarding demographics, clinical presentation and methods of diagnosis, surgical procedure, complications, and outcome were recorded. RESULTS Thirty-six cases of proximal large bowel volvulus were retrieved from the English literature, and 6 cases, from the authors practice. The male-female ratio was 1:1, with a median age of 10 years. There were 29 (69%) cases with neurodevelopmental delay. Clinical presentation included 29 (69%) cases with constipation, 41 (98%) with colicky abdominal pain, 42 (100%) with abdominal distension, and 35 (83%) with vomiting. Plain radiography was specific in 64% (27/42) of cases, barium enema in 100% (15/15), and computed tomography in 100% (2/2). All patients underwent surgery, with resection and primary anastomosis in 24 (57%) cases, stoma formation in 11 (26%), and detorsion of volvulus without resection in 7 (17%) cases. Six patients (14%) died postoperatively. CONCLUSION A child with neurodevelopmental delay and a history of constipation presenting with an acute onset of colicky abdominal pain and progressive abdominal distension with vomiting should be suspected of having a cecal and proximal large bowel volvulus.