Intrinsic esophageal innervation in esophageal atresia without fistula

Esophageal atresia and tracheo-esophageal fistula (EA + TEF) are often associated with malformations of neural crest origin. Esophageal innervation is also derived from the neural crest and it is abnormal in EA + TEF in which there is motor dysfunction. Our aim was to examine the intrinsic esophageal innervation in children with isolated EA in which different embryogenic mechanisms might be involved. Specimens from the proximal and distal esophageal segments of 6/35 patients who had esophageal replacement for isolated EA between 1965 and 2006 were suitable for the study. They were sectioned and immunostained with anti-neurofilament (NF) and anti-S-100 antibodies. The muscle and neural surfaces on each section were measured with the assistance of image processing software. The surface of the ganglia and the number of neurons per ganglion were determined at high power microscopy. The findings were compared with those of six autopsy specimens from newborns dead of other diseases by means of standard statistical tests and a significance threshold of P < 0.05. Unmatched age/size of babies in isolated EA and control groups precluded comparison of the relative surfaces occupied by neural elements. Patients with pure EA had denser fibrilar network and larger ganglia than controls. The number of neurons/ganglion were similar in both groups although the cells from EA patients were larger. The findings were similar at both esophageal levels studied. In spite of methodologic biases, it seems that intrinsic esophageal fibrilar network is denser and the intramural ganglia larger with larger cells in patients with pure EA than in controls on both esophageal ends of the organ. These neural anomalies are only in part reminiscent of those described in regular EA/TEF but may as well explain esophageal dysfunction in patients with repaired isolated EA. Presented at the 20th International Symposium on Pediatric Surgical Research, Bern, Switzerland, 5–6 October 2007.     

The association of esophageal duplication cyst with esophageal atresia

Bronchopulmonary foregut malformations are a group of related disorders with a common embryologic pathogenesis. Two bronchopulmonary foregut malformations may occur concomitantly in the same patient.     

食管闭锁患儿术后远期并发症及食管功能评价

目的 分析食管吻合术远期并发症及评价食管功能.方法 本研究追踪随访了1990～2007年间27例在新生儿期接受Ⅰ期食管端端吻合术的患儿.观察记录其出院后存在的并发症,包括:生长发育、进食吞咽困难、呼吸系统感染.通过应用食管造影、24h pH监测、24h食管测压手段对食管功能进行分析和评价.结果 平均随访时间(53.1±45.2)个月,48.1%患儿无临床不适表现,48.2%出现远期食管狭窄征象,同时伴有不同程度吞咽困难,22.2%食管狭窄伴严重进食困难者接受食管扩张.22.2%有病理性反流,食管测压结果显示70.3%有食管运动障碍,其中1/3不伴有吞咽困难症状.结论 吻合口狭窄、GER、食管运动障碍、呼吸系统感染等远期并发症基本上不影响患儿的正常生长发育和日常生活,严重的吻合口狭窄行食管扩张术后可以缓解.     

Long segment esophageal atresia and possibilities of esophageal replacement

In up to 20% of newborns with esophageal atresia a primary anastomosis of the esophagus is not possible because of the long distance between the two ends. In some cases a circular myotomy can elongate the upper pouch to allow a primary anastomosis. If primary anastomosis is not possible bougienage of the upper and lower pouch may stimulate growth of the esophagus and allow direct anastomosis after several weeks. In all other cases esophageal replacement is necessary. Colonic replacement of the esophagus must be weighed against replacement by jejunum, gastric interposition and the gastric tube. Each technique for esophageal replacement involves characteristic complications, and these are discussed in detail. For about 80% of the patients esophageal replacement allows a normal life with normal oral uptake of nutrition.     

Reoperative esophageal surgery

Reoperative esophageal surgery can be a very challenging endeavor. Preoperative evaluation, planning and preparation are essential to optimize results. A general reoperative approach and the range of reconstructive options are outlined. Management of specific problems is discussed including stricture, recurrent gastroesophageal reflux, recurrent tracheoesophageal fistula, esophageal interposition, and recurrent achalasia.     

Congenital esophageal stenosis associated with esophageal atresia: new concepts

Congenital esophageal stenosis (CES) is suspected by a fixed intrinsic narrowing of the esophagus that affects the normal swallowing mechanism. The diagnosis is only confirmed by histopathologic picture, which may show fibromuscular disease (FMD) or tracheobronchial remnants (TBR). The latter involves ciliated pseudo stratified columnar epithelium, seromucous glands or cartilage each alone or in combination. The aim of this study is to document the usefulness of histologic picture of surgical specimens obtained from the lower esophageal pouch (LEP) during primary repair in detecting cases of CES associated with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF). Over an 8-year period, 57 consecutive cases operated upon for EA with or without TEF were subjected for histologic examination of surgical specimens obtained from the tip of the LEP. Cases that histologically showed FMD or TBR were included. The usefulness of this histologic picture as a diagnostic and therapeutic aid is assessed. Methods of treatment and outcome were also reviewed. Eight patients out of 57 (14%) had a histologic picture suggestive of CES, two with FMD, four with TBR without cartilage and two with cartilage. Out of 57 patients, 23 developed strictures, six of them had positive biopsies suggestive of CES. One patient with TBR without cartilage did not have stricture. Another case of pure atresia had LEP resection and gastric pull up showed cartilage involving the whole lower esophagus. Excluding the case of pure EA with gastric pull up, all patients suffered from feeding problems and recurrent aspiration. Fluoroscopic barium studies showed late onset minor dysmotility in five patients and late onset major dysmotility in two. All cases studied showed significant gastro-esophageal reflux (GER). Stricture was seen at the anastomotic site extending distally in the two fibromuscular cases and one case with cartilage, at the anastomotic site in three cases with TBR without cartilage. Anti reflux surgical procedures were performed in four patients without benefit in two patients with major dysmotility. Dilatation was successful in the three patients with TBR without cartilage. One patient with cartilage had resection of the anastomotic site and required frequent dilatations and is now doing well. A case of FMD did not improve after frequent dilatations and myotomy together with Nissen’s fundoplication and required resection while the other case of FMD responded partially to dilatations. Cartilage in cases of CES requires surgical resection. Those with TBR without cartilage may not develop stricture. If stricture develops, it responds well to dilatation and patients have good clinical outcomes. Unlike isolated CES, GER is a significant feature in CES with EA. Anti reflux procedures should be avoided before definitive surgery for the stricture and if necessary a partial wrap with gastrostomy is recommended. CES should be considered in the etiology of anastomotic stricture. Taking a surgical specimen routinely from the tip of the LEP during primary esophageal repair for histologic studies is highly recommended.     

Long-gap esophageal atresia

The management of long-gap esophageal atresia remains challenging with limited consensus on the definition, evaluation, and surgical approach to treatment. Efforts to preserve the native esophagus have been successful with delayed primary anastomosis and tension-based esophageal growth induction processes. Esophageal replacement is necessary in a minority of cases, with the conduit of choice and patient outcomes largely dependent on institutional expertise. Given the complexity of this patient population with significant morbidity, treatment and long-term follow-up are best done in multidisciplinary esophageal and airway treatment centers.     

Congenital esophageal stenosis

A series of 20 patients with congenital esophageal stenoses (CES), including 4 with tracheobronchial remnants, 6 with membranous diaphragm, and 10 with fibromuscular stenosis, is presented. The experience acquired by treating these patients is compared to 95 cases from the literature and a definition of CES is proposed. The 35% association of CES woth other anomalies, especially esophageal atresia, is analyzed and some difficulties in the treatment of this rare anomaly are discussed. Offprint requests to: C. Nihoul-Fékété     

Intramural esophageal pseudodiverticulosis

A case of intramural esophageal pseudodiverticulosis in a five-year-old with a 16-year follow up is presented.     

The treatment of post-operative and peptic esophageal strictures after esophageal atresia repair

Nine patients, 6 weeks to 17 years of age with esophageal atresia (EA), developed esophageal strictures and were treated with 26 balloon catheter dilatations over a period of 3 years; 6 are now asymptomatic. Five of the 9 patients had suspected reflux esophagitis, confirmed in 3 by biopsy and treated medically prior to dilatation. Seven of the 9 patients had a primary anastomosis, 1 a gastric tube, and 1 a colonic interposition. Most dilatations in the group of 7 were performed with balloon (B) ≥ the diameter of the distal esophagus (E) (B/E≥1). The 3 residually symptomatic patients include an infant dilated conservatively (B/E<1) to facilitate later bouginage, 1 patient with a recurrent stricture after stopping medical therapy and home bouginage, and 1 infant who had a persistent anastomotic stricture, suspected but untreated reflux esophagitis, and a perforation during the second balloon dilatation. Balloon catheter esophageal dilatation, as an alternative to bouginage, is usually a safe and effective procedure when reflux esophagitis is diagnosed and treated prior to dilatation.     

Congenital esophageal stenosis.

We report 6 new cases of congenital esophageal stenosis (CES) that presented to us with special diagnostic and management problems and review the literature on this subject. Gastroesophageal reflux and achalasia are important differential diagnoses of esophageal stenosis, they may be associated with CES making appropriate management even more difficult. Treatment is also controversial, ranging from simple dilatation to segmental resection. CES should be present in every pediatric surgeon's mind should dysphagia occur on the introduction of semisolid food or esophageal food impaction, with a segmental stenosis demonstrated at esophagogram even when more obvious diagnoses are evoked.