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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.

Background Transanal anorectoplasty was developed through the 1980s by the senior author (AB) as an alternative approach to posterior sagittal anorectoplasty for the management of imperforate anus. This study evaluates this surgical approach and its longer-term results. Methods Case notes of all patients treated from 1984 to 2005 were reviewed. Operative procedures, colostomy requirement, complications, and long-term outcome were recorded. Patients were grouped according to the status of the pelvic floor, the location of the rectal fistula, and the sacral ratio. Results A total of 245 patients (175 male, 70 female) underwent transanal anorectoplasty. The perineum was well formed in 208 patients (85%), moderately formed in 15 (6%), and poorly formed in 22 (9%) patients. Two hundred three patients (82.8%) had a visible perineal or vulval fistula, 24 (9.8%) had a prostatic urethral fistula, whereas 18 (7.4%) had a bulbourethral fistula. Overall, 6 patients (2.5%) had wound infection or breakdown. As continence is age related, 32 patients were excluded from the study. Of the remaining 212 patients, 182 are continent with no soiling or only minimal staining. Thirty patients born with a poor perineum are incontinent. Conclusion Transanal anorectoplasty is a safe procedure with limited morbidity. It is sphincter sparing and permits accurate placement of the rectum with its internal sphincter within the anal canal. The anus lies accurately placed at the center of the external anal sphincter muscle complex. Given a well-performed surgical intervention, eventual continence relates to the original anatomy and neurology of the pelvic floor. Transanal anorectoplasty should be regarded as an alternative rather than as a substitute to posterior sagittal anorectoplasty for reconstruction of most forms of imperforate anus.

Purpose We present our experience with traction-compression-closure (TCC) for exomphalos major (EM) to achieve a safe and embryologically correct midline supraumbilical aesthetic closure with preservation of the umbilicus. Methods Nineteen neonates with EM were paralyzed and ventilated. The abdominal domain was increased by upward cord traction to assist liver-bowel reduction by gravity and sac ligation, followed by circumferential elastic body binder compression. The supraumbilical abdominal wall anomaly cicatrized spontaneously or was closed surgically as a midline scar, with preservation of the umbilicus. Results Over 7 years (1998-2004), 19 patients with EM were treated by TCC, 18 of whom survived. The patients' median gestational age was 36 weeks (range, 24-40 weeks); their median birth weight was 2312 g (range, 890-3000 g). The median time to reduction was 4 days (range, 3-5 days), whereas that to full enteral feeds was 6 days (range, 4-6 days). Mechanical ventilation for 7 days (range, 6-8 days) was not associated with any morbidity, and the time to home discharge was 11 days (range, 8-12 days). Five patients did not require any surgery. There was no episode of sac rupture or infection. Conclusion Abdominal expansion by vertical cord traction followed by compression reduction (TCC) under muscle relaxation and ventilation is time well spent toward a safe and aesthetic midline abdominal wall closure without tension for EM.

Purpose Neurologically impaired children run a 12% to 45% risk of recurrent gastroesophageal reflux (GER) after fundoplication. Elimination of the reflux by "rescue" total esophagogastric dissociation (TEGD) encouraged us to use it also as a "primary" form of antireflux surgery in this group of patients. Methods Twenty-six (14 male, 12 female) patients underwent TEGD between 1994 and 2004, of which 16 were primary and 10 were rescue procedures for failed fundoplication. Results There was no operative mortality and postoperative complications were limited to one subphrenic collection, one esophagojejunal dehiscence, and one small bowel hernia beneath the jejunal Roux loop. Gastrostomy feeding was usually established by 3 to 5 days and the mean hospital stay was 10.2 days (range, 6-18 days). At follow-up of 7 months to 11 years, there was no recurrence of GER. Four late deaths were unrelated to the surgery. The children's nutritional status improved with the mean weight standard deviation score showing a statistically significant increase from −2.63 preoperatively to −0.96 postoperatively (Wilcoxon's signed rank P value ≤.005). Conclusions Total esophagogastric dissociation is a safe definitive solution for GER because it eliminates all risk of recurrent reflux. We therefore feel that TEGD can be used as a primary treatment of choice for severely neurologically impaired patients who are experiencing GER and are completely dependant on tube feeds.

Introduction : Traditionally Hirschsprung's disease has been treated by a three-stage procedure. This approach has been associated with stoma problems, three episodes of hospitalization, and a theoretical disadvantage, because of the delayed passage of feces via the rectum, in controlling defecation. The aim of this study was to assess the results of one-stage neonatal pull-through for the treatment of Hirschsprung's disease. Methods : This was a 10-year retrospective review of all patients treated with a neonatal pull-through for Hirschsprung's disease. Information was obtained from the patients' records. All descriptive data are expressed as mean ± standard deviation. Results : Fifty-one patients were treated in the 10-year period studied. The mean gestational age was 39.6 ± 1.7 weeks, weight was 3.3 ± 0.54 kg, with 76% being boys. The surgery was performed at 10.3 ± 5.8 days of age. A Swenson procedure was carried out in 38 patients and a Soave in 13. The extent of the disease was rectosigmoid in 39, and the remaining were sigmoid, descending colon, and total colonic. The mean length of the surgery was 181 ± 40 minutes. Blood transfusions were required in 13 patients with a mean volume of 55 mL given. The complications observed were: an anastomotic leak in two cases, requiring laparotomy and colostomy (both in patients with Down's syndrome), and wound infection in three cases. There were two delayed deaths both associated with other congenital anomalies. In total, three patients required further surgery; two had colostomies, one requiring a redo pull-through and one had an ileostomy for enterocolitis. The total length of hospital stay was 20.4 ± 11.2 days. Mean follow-up was 3.73 ± 2.9 years. In 21 patients the follow-up was greater than 4 years and in this group none were totally incontinent. Four patients complained of soiling at least once a week, and 17 had normal bowel control. Conclusion : Hirschsprung's disease can be successfully treated in the neonatal period with a one-stage pull-through. The short- and long-term results are as good as those with the three-stage procedure, with the child usually benefitting by not having a stoma and a shorter hospital stay.

Background/Purpose Neurologically impaired (NI) children have an increased incidence of gastroesophageal reflux and many will require surgery. Methods The case notes of 50 NI children who underwent total oesophagogastric dissociation (TOGD) were reviewed. Thirty-four were done as a primary procedure, and 16 were rescues for failed fundoplications. Results There was no operative mortality. Morbidity consisted of 1 subphrenic collection, 1 oesophagojejunal dehiscence and 2 stenoses that responded to dilatation, and 2 bowel obstructions. In 1 case, partial gastric resection was needed because of transhiatal herniation of stomach. Gastrostomy feeding was established by 3 to 5 days. The mean hospital stay was 10.9 days. At 4 months to11 years of follow-up, there was no recurrence of reflux. Children who could swallow enjoyed oral feeds. Their general health and weight SD scores improved. Food aspiration, chest infections, and hospitalizations were reduced, with an improvement in quality of life. There were 5 late deaths in the "primary" and 7 in the "rescue" group from deterioration in their original condition. Conclusion Total oesophagogastric dissociation is a safe and versatile procedure without immediate mortality and limited surgery-related morbidity. Review of our practice suggests TOGD should be considered as a primary procedure in severely NI children with gastroesophageal reflux and significant oropharyngeal incoordination and dependence on enteral tube feeding. Rescue TOGD carries a greater morbidity because of previous surgery with consequent difficult dissection, poor oesophageal tissue, and higher incidence of vagal nerve injury.

Background: Tracheomalacia occurs as a primary developmental defect or may be secondary to vascular compression. It is common in association with esophageal atresia and tracheoesophageal fistula. Collapse of the weak trachea is a cause of recurrent respiratory symptoms but may be severe and life threatening. Methods: Between 1978 and 1999 at Sheffield Children's Hospital and The Royal Manchester Children's Hospital, of 16 children with clinically significant symptoms of tracheomalacia 8 underwent combined aortopexy and tracheopexy, 1 had aortopexy alone, 4 only had a tracheopexy, and 3 had tracheal reinforcement with free costal cartilage ring grafts. The surgical approach was limited to a low cervical skin crease incision with a midline manubrial split providing extrapleural access to the anterior mediastinum and allowing for all surgery under direct unimpaired vision. Results: Ten children did not require postoperative ventilatory support. Four underwent ventilation for a few hours or days. One child required CPAP for 4 months for residual tracheomalacia and a further child, who had 3 operations to insert 11 costal cartilage ring grafts, underwent ventilation intermittently for 6 months. Adequate tracheal patency could be verified by intraoperative tracheoscopy and was sustained postoperatively so that only 1 child with associated bilateral vocal cord paralysis came to tracheostomy. Four children required prolonged hospitalization because of residual tracheomalacia, 2 for bronchomalacia and 2 because of esophageal narrowing leading to further surgery. All other children were fit for discharge within 10 to 30 days of surgery. Long-term follow-up has confirmed sustained tracheal improvement and resolution of the life-threatening features of tracheomalacia. Conclusions: The authors recommend the low skin crease transmanubrial approach, as described by Vaishnav and MacKinnon, for tracheopexy, aortopexy and for tracheal reconstruction for tracheomalacia. The approach gives excellent access for surgery under direct vision through a relatively avascular plane. It is associated with less morbidity than a conventional thoracotomy and leaves a more acceptable aesthetic scar. J Pediatr Surg 35:1456-1458. Copyright © 2000 by W.B. Saunders Company.

Background/Methods: The authors report experience with five children, four of whom underwent esophagogastric dissociation with a Roux-en-Y esophagojejunal anastomosis and jejuno-jejunostomy after recurrent failure of fundoplication to control gastroesophageal reflux. A fifth child had a similar temporary gastric dissociation because of lifethreatening aspiration caused by reflux into a colonic neoesophagus. Reversal is planned when the child has achieved sufficient physical growth and accepts a substantial oral intake. Feeding into the dissociated stomach was by intermittent catheterization of a nonrefluxing gastrostomy in four children and a Stamm tube gastrostomy in one child. The postoperative course and follow-up were notable for the rapid improvement in the children's general condition and nutritional status and the reduction in the frequency of pneumonitis. Reflux did not recur. Results: Review of the postoperative course led to a restructuring of surgical management with the development of tailored ‘patient-specific' plans based on short-term and long-term prognosis. Failure to thrive, difficulty in child handling, gastroesophageal reflux, and neurological and physical impairment are particular considerations. Conclusions: The author's limited experience leads us to suggest that esophagogastric dissociation with Roux-en-Y esophagojejunostomy and a nonrefluxing feeding gastrostomy is a useful temporary or permanent ‘rescue' procedure. Furthermore, it is the author's view that the procedure may have a useful role as the primary form of management when normal oral feeding is unlikely to become established.