Michel Bonnevalle

Anastomotic stricture after surgical repair of esophageal atresia: frequency, risk factors, and efficacy of esophageal bougie dilatations

AIMS The aim of this study was to evaluate the frequency and risk factors of postoperative anastomotic stricture, and the efficacy and complications of esophageal bougie dilatations for symptomatic anastomotic stricture in a population of children with esophageal atresia. PATIENTS AND METHODS The medical records of 62 children operated on for esophageal atresia type III (Ladd and Gross) over a 5-year period were retrospectively reviewed. RESULTS Anastomotic stricture developed in 23 (37%) of patients. Anastomotic tension during primary repair of esophageal atresia was associated with subsequent stricture formation (P < .05). Patients required esophageal dilation at a mean age of 149 days (range, 30-600 days). Stricture resolution occurred after a mean of 3.2 dilatations per patient (range, 1-7). Dilation was successful in 87% of patients. Three patients continued to present mild (n = 1) to severe (n = 2) dysphagia, mainly related to esophageal dysmotility. No complications were observed during or after the dilatation sessions. CONCLUSIONS Anastomotic stricture, secondary to the surgical treatment of esophageal atresia, remains a frequent complication in patients with esophageal atresia. Esophageal dilation with Savary-Gilliard bougies is a safe and effective procedure in the management of strictures.

Long-term outcome of colon interposition after esophagectomy in children.

Background and Objectives: Tissues derived from the colon, stomach, and jejunum have been used to replace the esophagus in childhood to cure esophageal atresia or stricture secondary to gastroesophageal reflux or the ingestion of corrosive agents. The outcome in adulthood of colon interposition performed at an early age has yet to be satisfactorily assessed. The aim of this single-center retrospective study was to evaluate the long-term nutritional, digestive, and respiratory outcome of all patients (n = 32) who underwent colon interposition during childhood in our hospital (1970–2001). Patients and Methods: Medical records of these subjects were reviewed and their nutritional (weight, height, 24-hour food diary), digestive (questionnaire), and pulmonary function status evaluated. Results: Of the patients, 17 had esophageal atresia (7 males, median age at surgery 11 months, range 0.5–61) and 15 had ingested corrosive substances (10 males, median age at surgery 50 months, range 22–113). Complications occurred less than 1 year postoperatively in 53% and long-term complications (occurring >1 year after surgery) in 84%. Long-term complications were common: digestive symptoms were found in 85% (most frequently observed during the first 5 years of follow-up), abnormal lung function in 7 (58%) of those tested (n = 12), feeding difficulties in 50%, scoliosis in 35%, and nutritional complications in 25%. Conclusions: Our study showed a high rate of sequelae following esophageal replacement. This highlights the need for multidisciplinary long-term follow-up into adulthood, and research into alternatives to colon interposition as treatment for esophageal strictures.

Sténose anastomotique après traitement chirurgical de lˈatrésie de lˈœsophage : fréquence, facteurs de risque et efficacité des dilatations œsophagiennes

Resume La stenose anastomotique est la principale complication apres traitement de lˈatresie de lˈœsophage. Elle est habituellement traitee par dilatation mecanique par bougie ou par ballonnet. Buts de lˈetude. – Les buts de ce travail retrospectif etaient dˈetudier dans une population dˈenfants operes a la naissance dˈune atresie de lˈœsophage de type III ou IV : 1) la frequence et les facteurs associes a la survenue dˈune stenose anastomotique ; 2) lˈefficacite des dilatations œsophagiennes par bougie de Savary. Patients et methodes. – Les caracteristiques de 52 enfants operes sur une periode de cinq ans ont ete analysees. Le terme et le poids de naissance, la duree du maintien des drains mediastinaux et trans-anastomotiques, la tension lors de la realisation de lˈanastomose, la survenue de complications precoces (fistule et/ou mediastinite) et la presence d’un reflux gastro-œsophagien ont ete notes. Les parametres concernant le groupe dˈenfants ayant eu une stenose anastomotique ont ete compares a ceux du groupe dˈenfants indemnes de stenose. En cas de survenue dˈune stenose anastomotique, lˈâge de lˈenfant lors de la premiere dilatation, le nombre de seances de dilatation realisees et lˈefficacite des seances etaient notes. Resultats. – Vingt enfants (40 %) ont eu une stenose anastomotique. Le terme, le poids de naissance, la duree du maintien des drains trans-anastomotiques ou mediastinaux n’etaient pas associes a la survenue ulterieure dˈune stenose anastomotique. Lˈexistence dˈune tension lors de la realisation de lˈanastomose etait significativement liee a la survenue dˈune stenose ( p n = 1) ou severe ( n = 1). Conclusions. – La survenue dˈune stenose anastomotique apres traitement chirurgical de lˈatresie de l’œsophage est frequente, favorisee par la longueur du segment atresique conditionnant la tension lors de la suture. La realisation de dilatations œsophagiennes par bougie de Savary est une technique efficace, mais necessite habituellement plusieurs seances pour obtenir la disparition de la dysphagie.Anastomotic stricture is the most common complication following the surgical repair of esophageal atresia, and is usually treated by esophageal dilation. Objectives. – The aims of this study were to assess in an infant population operated on at birth for type III or IV esophageal atresia: 1) the frequency of esophageal stenosis following the repair of esophageal atresia, and associated factors; 2) the efficacy of esophageal dilation by the Savary-Gaillard bougie technique. Materials and methods. – The medical records of 52 children presenting with esophageal atresia over a 5-year period were retrospectively reviewed. Gestional age and birth weight, duration of mediastinal and transanastomotic drainage, and anastomotic complications including leakage, stricture, and the presence of gastroesophageal reflux were recorded and analysed. Patients presenting with anastomotic stricture were compared with a group of children without stricture. The number of esophageal dilations, their efficacy and the complication rate were analyzed. Results. – Anastomotic stricture developed in 20 (40%) of the 50 patients undergoing primary repair for esophageal atresia. The occurrence of anastomotic stricture was related to anastomotic tension during esophageal surgical repair (p < 0.03). Young children required esophageal dilation at a mean age of 142 days (24–930 days). Stricture resolution occurred after a mean of 3.2 dilations (1–15) over an average period of 7.9 months (range: 0–30 months). Dilation was successful in 90% of the 20 patients. Seven patients required only one dilation. Perforation of the esophagus occurred in one case, and this severe complication led to the death of the child. Esophageal dilation was unsuccessful in two patients, who presented prolonged severe dysphagia. Conclusion. – Anastomotic stricture following repair of esophageal atresia is connected with the length of the gap that has to be repaired, and tension during suture. Esophageal dilation by the Savary-Gaillard bougie technique is an effective method for treating esophageal stricture. Several dilations are usually needed before the disappearance of dysphagia.

Favorable nutritional outcome after isolated liver transplantation for liver failure in a child with short bowel syndrome.

A patient with short gut syndrome presented with end-stage liver disease. She underwent an isolated liver transplantation at the age of 3.5 years. Parenteral nutrition was discontinued 1.5 years after surgery. At 7 years of age, the patient has maintained normal nutritional indices and growth while on a normal oral diet. This observation suggests that liver transplantation alone can be a valuable alternative to the combined small bowel/liver transplantation in short bowel syndrome when intestinal adaptation is expected and if the ileocecal valve is present and that improvement of gut function can occur after successful liver transplantation.

Intraoperative endoscopic diagnosis of heterotopic gastric mucosa in the ileum causing recurrent acute intussusception

Heterotopic gastric mucosa (HGM) may be found anywhere in the gastrointestinal tract, most often in a Meckels diverticulum. Approximately 30 cases of HGM, located in the small bowel beyond the ligament of Treitz and not associated with a Meckels diverticulum, have been reported. They were most often revealed by intestinal intussusception, occasionally by perforation of an intestinal ulcer or intestinal bleeding. We report a 4-year-old boy who had three attacks of acute intestinal intussusception over a 5-month period resulting in surgery. Both physical examination and barium examination of the small bowel and large intestine were found to be normal between attacks. Peroperative palpation of the small bowel was normal during the three laparotomies. During the third operation, he underwent an intraoperative endoscopy (IOE), which revealed a polypoid mass 2 cm in diameter and 0.5 cm in height, 40 cm proximal to the ileocaecal valve. Histologic examination showed HGM with fundic glands, and chief and parietal cells. This case emphasizes the interest of IOE, the main indications of which are the localization of unknown sites of gastrointestinal bleeding and the search for hamartomatous polyps of the Peutz-Jeghers syndrome for polypectomy and/or segmental resection.

A fatal case of portal hypertension complicating systemic mastocytosis in an adolescent

Portal hypertension was observed in a 17-year-old girl with urticaria pigmentosa since 2 months of age. Liver biopsies showed portal and sinusoidal infiltration with mast cells although spleen biopsies showed only fibrosis.ConclusionPortal hypertension is a complication of systemic mastocytosis that can lead to death. Treatment with interferon alpha might be effective.

Prolonged enteral feeding is often required to avoid long-term nutritional and metabolic complications after esophagogastric dissociation.

Background: Total esophagogastric dissociation (TED) was first described in 1997 by Bianchi as a new surgical procedure to treat severe gastroesophageal reflux disease (GERD) in children with neurological impairment. Recently, TED has been proposed in other conditions, such as esophageal atresia, esotracheal cleft, or caustic esophageal lesions. Although the long-term results in terms of GERD control have been previously reported, those regarding the nutritional and metabolic status have never been documented. Patients and Methods: All patients without neurological impairment with TED between 1999 and 2004 at Robert Debre Hospital and Jeanne de Flandre Hospital, France, were prospectively investigated, paying particular attention to their metabolic and nutritional status (blood concentration of iron and vitamins A, D, E, and B12; lipid malabsorption; and hyperglycemia test) and growth. Results: Seventeen children underwent TED. Six received primary procedures, whereas 11 were operated on because of severe respiratory diseases or failure to thrive. The mean follow-up was 6 years (range 3–8 years). Two children died (12%). Seven children were weaned from enteral nutrition support, but 5 of them had failure to thrive, steatorrhea, and/or malabsorption of vitamin B12 and/or fat-soluble vitamins. Eight patients had dumping syndrome, which was symptomatic in 6 cases. Conclusions: TED is an effective procedure for treatment of GERD. However, nutritional and metabolic complications including dumping syndrome and chronic digestive malabsorption are frequent after TED, especially after enteral nutrition weaning. A long-term follow-up of these patients is thus necessary and prolonged enteral nutrition support is recommended.

Favorable long-term outcome after isolated liver transplantation in a child with short bowel syndrome.

To the Editor: Hassan et al. (1) recently reported a child with short bowel syndrome who underwent a liver transplantation and then a Bianchi procedure with a good long-term nutritional outcome. The authors stated that only a few cases of isolated liver transplantation performed for liver failure in short bowel syndrome have been reported and emphasized that long-term follow-up on the clinical and nutritional status of such children is lacking. We report here on a girl with short bowel followed for 8 years after an isolated orthotopic liver transplantation. The patient underwent isolated liver transplantation at 4 years for end-stage liver disease associated with IV nutrition. Her clinical history and early follow-up after liver transplantation have been reported in detail (2). She presented at birth with distal ileal atresia and secondary midgut volvulus, and all but 78 cm of proximal small bowel were resected on day 1 of life. She was maintained on parenteral nutrition and at 3 years of age experienced cirrhosis with hepatic failure as a consequence of parenteral nutrition. An isolated liver transplantation was performed at the age of 4 years that was complicated by an abdominal wound dehiscence with evisceration and a single rejection episode. Liver function test results normalized after 5 months. Her long-term clinical condition has been favorable. Eight years after liver transplantation, her liver function test results are normal while taking cyclosporine as the sole therapy. No complication of liver transplantation (renal failure, lymphoproliferative syndrome) have occurred. Intestinal function improved gradually after transplantation. Oral nutrition was reintroduced, and parenteral nutrition was stopped 18 months after transplantation. Today the patient takes a normal diet, and serum nutritional markers (calcium, iron, albumin) are normal. She has 4-5 stool/day and still has steatorrhea (60 g/d, 30 g/d, 21 g/d at 1, 2, and 6 years, respectively, after transplantation; normal, <6 g/day). Her daily calorie intake is 3,212 Kcal/d 8 years after transplantation, which we estimate to be 112% of the recommended daily allowance. She had an acceleration of both weight and height gain 1 year after transplantation, even after parenteral nutrition was stopped (Figure 1). At 11 years, her weight was 35.6 kg (0 SD), her height was 155 cm (+1 SD), and her pubertal status was stage 2 for breast and stage 2 for pubic hair by the Tanner scale. This case confirms that isolated liver transplantation can be a good long-term therapeutic option for cirrhosis associated with short bowel syndrome. Efforts should be made to evaluate, as in the case of Hassan et al., all of the conservative measures that may promote intestinal adaptation.

Complexité de l’interprétation anatomopathologique dans le syndrome mégavessie-microcôlon-hypopéristaltisme intestinal

Resume Le syndrome megavessie-microcolon-hypoperistaltisme intestinal, tres rare, est la plus severe des pseudo-obstructions intestinales chroniques. Le diagnostic est habituellement porte en periode neonatale. Il est clinique et radiologique, et precise par les etudes manometriques. Les lesions anatomopathologiques sont variees, inconstantes et aspecifiques. Elles toucheraient plus souvent le muscle lisse que l’innervation intrinseque au niveau enterique et vesical. L’observation se caracterisait, chez une petite fille actuellement âgee de sept ans, par une megavessie decouverte a l’echographie prenatale realisee a 21 semaines d’amenorrhee. Le liquide amniotique etait d’abord en quantite normale pour l’âge gestationnel, puis un hydramnios est apparu a 30 semaines d’amenorrhee. Un microcolon etait decouvert a la naissance, avec microgrele distal et dilatation duodenale et jejunale proximale, malposition intestinale, et hypoperistaltisme severe de tout le tube digestif necessitant une enterostomie et une nutrition parenterale totale depuis la naissance. A l’examen anatomopathologique, la biopsie rectale et les plexus nerveux enteriques etaient d’aspect normal. Il existait une hypoplasie de la couche longitudinale externe de la musculeuse au niveau colique et au niveau ileal. Les cellules de Cajal n’etaient pas mises en evidence en immunohistochimie au niveau colique. Cette observation souligne la complexite et les difficultes de l’interpretation anatomopathologique dans ce syndrome, et la necessite d’une large serie de temoins a differents âges et a differents niveaux du tube digestif et de la vessie.Megacystis-microcolon-intestinal hypoperistalsis syndrome is very rare, and is the most severe of the chronic intestinal pseudoobstructions. Diagnosis is usually made in the neonatal period, is clinical and radiological, and is confirmed by manometric studies. Microscopic abnormalities are variable, inconstant and nonspecific. They involve the smooth muscle more often than the intrinsic innervation of the gut and the bladder. A girl, currently seven years old, presented with megacystis observed on prenatal ultrasound at 21 weeks of gestation. At first, amniotic fluid volume was appropriate for gestational age, and then hydramnios appeared at 30 weeks of gestation. Microcolon was discovered at birth, with microileum, dilatation of the duodenum and proximal jejunum, intestinal malposition, and severe hypoperistalsis of the entire gastrointestinal tract, which indicated enterostomy and total parenteral nutrition from birth. At pathological examination, rectal biopsy and enteric nervous plexuses were normal. There was hypoplasia of the external longitudinal layer of the muscularis propria in the colon and ileum. Cajal cells could not be demonstrated immunohistochemically in the colon. This case highlights the complexity and difficulties of pathological interpretation in this syndrome, and the necessity of a large study of controls at different ages and different levels of the digestive tract and the bladder.

Prognostic value of esophageal manometry in antireflux surgery in childhood.

To assess the predictive value of preoperative esophageal manometric study in the outcome of antire-flux surgery, 14 children with severe gastroesophageal reflux (GER) who underwent surgery were studied retrospectively. Five patients had neuromuscular disease; one had been operated on for esophageal atresia. After extended (gt; 20 h) esophageal pH monitoring and/or barium swallow study, all patients underwent preoperative manometric study. After surgery, the patients were followed for 4 months to 4 years. Functional complications were noted after mechanical complications were eliminated. All patients had normal upper esophageal sphincter pressure (UESp); the resting lower esophageal sphincter pressure (LESp) was decreased in four patients, and seven had esophageal body motility trouble. Functional complications occurred in two patients. One was a neu-rologically involved patient who had had a normal preoperative manometric study; the other was the patient who had been operated on for esophageal atresia. No complications occurred in four patients who had had abnormal preoperative manometric studies. We conclude that, in this group of patients, esophageal manometric study has no predictive value in the outcome of the surgical procedure; however, it still would be interesting to elucidate the mechanisms of GER, especially in congenital abnormalities such as esophageal atresia.