Multistaged extrathoracic esophageal elongation procedure for long gap esophageal atresia: Experience with 12 patients.

PURPOSE: This study evaluates the multistaged extrathoracic esophageal elongation procedure performed on 12 babies with long gap esophageal atresia over 15 years. METHODS: Eight babies had pure esophageal atresia, 2 had proximal tracheoesophageal fistula (TEF), and 2 had distal TEF. The gaps ranged between 2 and 7 vertebral bodies. Proximal esophagostomy, TEF ligation, and gastrostomy were performed initially. The proximal esophagus is elongated 2 to 3 cm each time by translocating the esophagostomy distally along the anterior chest wall at 2- to 3-month intervals. Sham-fed milk is collected in an ostomy bag and refed via the gastrostomy. The definitive esophageal reconstruction is performed at 5 to 24 months of age. RESULTS: Only one elongation was required in 4 babies, 2 were needed in 5, 3 in 2, and 5 in 1 patient. All patients tolerated sham feeding well. After esophageal restoration, 3 patients had minor leakage. All (12 of 12) patients had anastomotic stenosis requiring multiple dilatations, of which, 3 needed resection of stricture. Eleven patients had gastroesophageal reflux that required fundoplication. Follow-up was possible in 11 patients for 4 months to 14 years after esophageal restoration. Seven early patients are eating normally. CONCLUSION: Multistaged extrathoracic esophageal elongation is effective in stretching the proximal esophagus to bridge 2 to 7 vertebral bodies.

Pyocolpos: diagnosis and treatment.

Pyocolpos is a rare complication of hydrocolpos. Hydrocolpos usually presents during adolescence and is associated with an imperforate hymen. The following is a case of a 3-month-old girl with pyocolpos. Her history was significant for a urinary tract infection (UTI) at 7 weeks of age. The authors believe that her UTI was caused by urinary retention secondary to hydrocolpos. A complete evaluation may have prevented the complication of pyocolpos.

Elliptical seromuscular resection for tapering the proximal dilated bowel in duodenal or jejunal atresia.

The authors developed a surgical technique to taper the proximal dilated bowel in patients with duodenal or jejunal atresia. An appropriately wide elliptical piece of the seromuscular layer along the antimesenteric border is resected, with its underlying submucosa and mucosa kept intact. The muscular margins are approximated by sutures, with the mucosa either inverted or imbricated into the bowel lumen. This technique has the advantage of avoiding infection, leakage, or protrusion of thick bowel wall into the bowel lumen, which may produce a motility disorder.

Button device for antegrade enema in the treatment of incontinence and constipation.

Antegrade enema through an appendicostomy is effective in improving the quality of life for patients with incontinence or severe constipation. For patients without an appendix, a conventional gastrostomy button was used to create a cecostomy (or ileostomy) that served as a port for the administration of enema solutions.

Bipolar electrode implantation for myoelectrical recordings of rat bowel.

This report describes a technique to implant bipolar electrodes into the rat bowel. We implanted a total of 129 pairs of bipolar electrodes into the bowel of rats. 124 pairs of electrodes (96.3%) have continuously functioned allowing repeated myoelectrical readings. Of these, 69 electrodes functioned over 8 weeks. Only 5 electrodes (3.7%) failed. The advantage of our technique includes: (1) high success rate in implantation, (2) long term durability, and (3) less technical difficulty.

Anteriorly located anus: is constipation caused by abnormal location of the anus?

Anteriorly located anus (ALA) is frequently associated with severe constipation accompanied by defecation pain. Between 1988 and 1994, the authors treated 27 children (26 girls, 1 boy; age range, 0 to 11 years) to surgically correct ALA. The operation was performed according to a uniform protocol to longitudinally divide the internal sphincter muscle from the anal skin level to 2 cm above the dentate line on the posterior wall of the anorectum. For anal reconstruction, any of the conventional procedures was employed. Twenty-two of the 27 patients have had follow-up in our clinic for 12 months to 6 years (mean, 2.75 years). Eighteen are completely free of constipation and defecation pain and have regular spontaneous bowel movements. The other four require occasional use of enemas or laxatives. Anal incontinence did not occur in any patient. The results of this study suggest that abnormal function of the internal sphincter is the most likely cause of constipation or defecation pain in patients with ALA and that internal sphincterotomy is the cornerstone of surgical treatment.

Pyomyositis in an adolescent female athlete.

A case of pyomyositis in a healthy 13-year-old female volleyball player is presented and discussed. This case is unusual because, historically, pyomyositis has been more common in males, especially those who participate in strenuous physical activity. However, competitive sports and vigorous exercise programs are becoming more widely available to young females. Therefore, a relative increase in the number of adolescent women with pyomyositis can be expected.

Motility of isolated bowel segment Iowa model III.

The isolated bowel segment (IBS) is an amesenteric segment of bowel that is devoid of extrinsic nerves and yet is viable with motility and absorption preserved after its mesentery is completely severed. The IBS is created by initial coaptation of a loop of bowel to a host organ, such as muscle (Iowa model I), liver (Iowa model II), or intestine, and secondary severance of its mesentery several weeks later. In previous studies using Iowa models I and II, the viability, motility, and absorption of the IBS were preserved by vascular collaterals, which form across the coaptation. In rats, an IBS was created in the jejunum by initial enteroenteropexy (Iowa model III), followed by its mesenteric division five weeks later. At the second laparotomy, bipolar electrodes were implanted in the IBS and the adjacent jejunum. One week later, myoelectrical recording was performed during a fasting state. Coordinated abroad propagation of migrating motor complex (MMC) was observed in the IBS. The MMC period was 16.9 +/- 1.7 and 19.3 +/- 0.8 min in the intact bowel, and 22.8 +/- 0.8 min in the IBS (P = 0.1). After feeding, the MMC in the IBS was replaced by irregular spike burst activity similar to that observed in the intact bowel. This study concludes that the IBS Iowa model III can be used for studies of bowel physiology.

Congenital microgastria.

Congenital microgastria is an extremely uncommon dysplasic condition of the newborn stomach. With only 26 well-documented accounts of this anomaly reported in the literature, its treatment and long-term outcome have not been well elucidated. Herein, the authors report on a newborn with multiple congenital anomalies who presented with severe reflux and aspiration. The workup showed congenital microgastria. The infant's diagnostic and treatment course is described.

Multistaged extrathoracic esophageal elongation for long gap esophageal atresia.

The authors developed an extrathoracic multistaged esophageal elongation scheme for managing patients with long gap esophageal atresia. The protocol consists of (1) initial cutaneous esophagostomy of the proximal esophagus and feeding gastrostomy followed by (2) multistaged extrathoracic elongations of the proximal esophagus, translocating its cutaneous stoma down the anterior chest wall at intervals of several weeks, and (3) esophageal end-to-end anastomosis in the mediastinum. These procedures were successfully employed in an infant with esophageal atresia with an initial gap of seven vertebral bodies. Three elongation procedures, including the initial one, were performed at intervals of 2 to 6 months, which allowed a tension-free esophageal anastomosis in the mediastinum. Since the initial operation, the patient has been sham-fed with formula, which was collected in a stomal bag and refed via the gastrostomy. Immediately after the final operation, no difficulty was experienced in nipple feeding. She has had follow-up for 3 years, and the result is satisfactory.

Posterior sagittal rectal myectomy for persistent rectal achalasia after the Soave procedure for Hirschsprung's disease.

For the management of persistent rectal achalasia after the Soave endorectal pull-through procedure, we have used posterior sagittal myectomy of the remaining aganglionic rectal muscular cuff, and have had satisfactory outcomes in five patients. Via a posterior sagittal skin incision, the posterior aspect of the rectal muscular cuff is reached. With the striated muscular complex retracted downward, the level of the dentate line is identified on the posterior wall of the rectum with the aid of the surgeon's finger inserted inside the anorectum. Two parallel longitudinal incisions are made on the rectal muscular cuff to create a muscular strip which is elevated and excised; the distal end of the myectomy strip is at the level of the dentate line and includes a part of the internal and sphincter muscle. During the last 4 years, we performed this procedure in 5 patients with remarkable relief of constipation, distension, and enterocolitis. The advantages of this procedure include: (1) less technical difficulty than the transanal approach, (2) avoiding colostomy, and (3) promising results.

A new bowel elongation technique for the short-bowel syndrome using the isolated bowel segment Iowa models.

For the management of infants with the short-bowel syndrome, we developed a two-stage bowel elongation procedure based on experimental studies of what we term as an isolated bowel segment. The procedure consists of: (1) initial coaptation of the antimesenteric surface of a segment of bowel to host organs such as liver and abdominal wall, and (2) after collaterals have developed from these host organs, secondary longitudinal split of the bowel to provide two bowel loops, one from its antimesenteric half and the other from its mesenteric half. These are arranged in series by end-to-end anastomosis to double the original bowel length. The antimesenteric loop is totally free of its original mesenteric attachment but viable by vascular collaterals formed across the coaptation site. This procedure was successfully used for an infant who was born with 17 cm of duodenum and 17 cm of the distal colon from first trimester intrauterine midgut volvulus. At completion of the multistaged procedures at the age of 1 year when we reentered the abdomen for duodenoplasty, his small bowel measured 90 cm in length. He is currently taking 50% to 60% of required calories via the enteric route at 18 months of age. This procedure is suitable for elongating the duodenum of infants when other alternatives such as the Bianci procedure are not feasible because of mesenteric absence.

A staged surgical approach to save ischemic bowel.

A 15-year-old girl developed bowel strangulation of 80% of her small intestine by an omental sling. At exploration, only 100 cm of proximal jejunum remained clearly viable and the remaining small bowel looked necrotic. The transitional bowel between normal and ischemic segments was exteriorized to form a double-barreled jejunostomy. Twelve hours later a "second look" operation was performed. The bowel distal to the exteriorization appeared still seminecrotic but blood flow recovery was demonstrated along the mesenteric border by Doppler oxymeter. No bowel resection was performed. Two months later the jejunostomy was converted to a Bishop-Koop type side-to-end jejunostomy. In the ensuing 2 months, the patient passed both gas and stool per rectum, and oral feedings were gradually increased, retaining the jejunal stoma as a "safety valve." Later, the stoma was taken down, stenotic bowel segments were resected, and the bowel was finally reconstructed by an end-to-end anastomosis, preserving approximately 80% of the small intestine. This management strategy provides an alternative approach to the conventional practice of simple resection of severely ischemic bowel, allowing maximal salvage of bowel with reversible high-grade ischemic change in selected patients.

Foramen of Morgagni hernia in identical twins: is this an inheritable defect?

Twins with Down's syndrome, foramen of Morgagni hernias, and similar cardiac anomalies are described. While diaphragmatic hernias are not uncommon, the occurrence of this congenital defect in twins with very similar congenital anomalies raises the possibility that diaphragmatic hernias may result from an inheritable defect.

The isolated bowel segment (Iowa model II): motility across the anastomosis with or without mesenteric division.

In previous reports, anastomosis has been shown to disrupt the myoelectric activity of the bowel. However, these studies have failed to delineate the role of the extrinsic nerves. Using an isolated bowel segment (IBS) and an amesenteric bowel segment (ABS), motility was evaluated by myoelectric recording across a bowel anastomosis. Ten rats were divided equally into the experimental group with the IBS and the control group with the ABS. In the IBS group, an 8-cm segment of jejunum was divided, reanastomosed, and coapted to the liver margin (Iowa model II). In the ABS group, an 8-cm segment of jejunum was coapted to the liver margin without disruption of bowel continuity (Iowa model II variant). Two weeks later, bipolar electrodes were implanted in the IBS and ABS, and normal jejunum in both groups. Mesenteric division (MD) was performed 4 weeks later to eliminate extrinsic innervation. Myoelectrical recordings were taken 2 weeks before and after MD. In the control group with IBS, incoordination in the propagation of the migrating motor complex (MMC) and reduction in the frequency of slow waves (FSW) were observed across the anastomosis and were unchanged by MD. In the control group with the ABS, the MMC and FSW were identical to that in the normal jejunum and were unaffected by MD. In both groups postprandial inhibition of the MMC was the same as in the normal jejunum and was unaffected by MD. This study confirms that incoordination in propagation of the MMC and reduction in FSW occur across a bowel anastomosis, and elimination of extrinsic innervation does not affect the autonomy of these changes.(ABSTRACT TRUNCATED AT 250 WORDS)

The isolated bowel segment (Iowa Model II): absorption studies for glucose and leucine.

A model of the isolated bowel segment (IBS, Iowa Model II) was successfully created in experimental animals using a new surgical technique we developed. The IBS is completely free of its mesenteric attachment, yet its viability is preserved. The technique consists of two staged procedures: (1) initial enteropexy between the anterior margin of the liver and the antimesenteric border of the IBS with its ends forming cutaneous stomas; and (2) division of the IBS mesentery 5 weeks later. The IBS is nourished by vascular collaterals that form at the hepatoenteropexy during the interval between these two procedures. Our previous studies demonstrated preserved viability and motility in the IBS. This study was undertaken to test absorption in the IBS. In 25 rats (experimental group), the IBS (Iowa Model II) was created using an 8-cm-long isolated segment of jejunum. In 15 rats (control group), an 8-cm-long segment of jejunum was arranged to form a Thiry-Vella loop. Five weeks later, the IBS mesentery was divided in the experimental group, and sham laparotomy was performed in the control group animals. Absorption of glucose and leucine was studied in 13 rats of the experimental group and 6 of the control group using a constant single perfusion technique at 3, 8, and 11 weeks after the initial operation. The results were compared between the two groups. There was a 25% to 35% reduction in absorption of glucose and leucine in both groups with the advance of time, but no significant difference was observed between the groups except in leucine absorption at 11 weeks after the initial operation. This study concludes that absorption of glucose and leucine is preserved in the IBS after its mesentery is divided, suggesting that the IBS can be used as a functioning bowel for bowel reconstruction.

The isolated bowel segment (Iowa model II) created in functioning bowel.

In experimental rats (n = 15), an isolated bowel segment (IBS) was created by (1) initial enteropexy between an 8-cm-long jejunal segment and the liver margin (hepatoenteropexy; Iowa model II) with its proximal and distal ends divided and immediately reanastomosed in an end-to-end fashion to reconstruct the bowel; and (2) secondary division of the IBS mesentery 5 weeks later. The IBS is then completely free of its mesentric and intramural nervous and vascular communications. The viability of the IBS is preserved by vascular collaterals developed at the hepatoenteropexy. Twelve rats proceeded to the second procedure, having tolerated regular rat chow with satisfactory weight gain. The Iowa model II created in functioning bowel was evaluated by contrast studies and myoelectrical activities. Contrast studies demonstrated peristalsis in the IBS. In the myoelectrical recordings, the frequency of slow wave was 32.5 +/- 1.0 in the IBS and 36.3 +/- 0.8 in the normal bowel (P less than .05). During fasting, the migrating motor complex (MMC) was observed to propagate aborally in the IBS in a coordinated fashion. The cyclic period of the MMC was 17.2 +/- 1.1 minutes in the IBS and 15.8 +/- 0.8 minutes in the normal bowel (P = .30). We conclude from this study that (1) the IBS (Iowa model II) retains motor function as demonstrated by successful feeding, as well as contrast studies and myoelectrical recordings that were essentially identical to those in the normal bowel; and (2) the IBS (Iowa model II) has significant research potential for studies of bowel physiology.