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Journal of Indian Association of Pediatric Surgeons
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Year : 2016  |  Volume : 21  |  Issue : 3  |  Page : 150-152

Management of long segment congenital esophageal stenosis: A novel technique

1 Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication18-May-2016

Correspondence Address:
Vishesh Jain
Department of Pediatric Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9261.182592

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Congenital esophageal stenosis (CES) is a rare cause of dysphagia in children. Diagnosis is often apparent on esophagogram. Surgical treatment for the subtype with tracheobronchial remnants (TBR) includes resection and anastomosis of the stenosed segment, myectomy, enucleation of cartilage, etc., These procedures are not suitable if the stenosed segment is long. We present a case of a 5-year-old boy who was diagnosed as CES and was successfully managed with stricturoplasty with some innovative modifications.

Keywords: Esophageal stenosis, esophageal stricture, tracheobronchial remnants

How to cite this article:
Jain V, Yadav DK, Sharma S, Jana M, Gupta DK. Management of long segment congenital esophageal stenosis: A novel technique. J Indian Assoc Pediatr Surg 2016;21:150-2

How to cite this URL:
Jain V, Yadav DK, Sharma S, Jana M, Gupta DK. Management of long segment congenital esophageal stenosis: A novel technique. J Indian Assoc Pediatr Surg [serial online] 2016 [cited 2023 Oct 2];21:150-2. Available from: https://www.jiaps.com/text.asp?2016/21/3/150/182592

   Introduction Top

Congenital esophageal stenosis (CES) is a rare condition in which a malformation of esophageal wall architecture results in stenosis of the esophagus. It has a reported incidence of about 1 in 25,000-50,000 live births. [1] Most cases reported in the literature are small segmental strictures which are managed with segmental resection and anastomosis of the esophagus. [2] The index case had a long segment stricture in which segmental resection was precluded and was managed by an innovative gastroesophagoplasty. The index case has been described with emphasis on the procedure performed.

   Case report Top

A 5-year-old boy presented with the complaints of difficulty in swallowing since infancy which was gradually increasing in severity. At the time of presentation, the child has difficulty in swallowing semi-solids also. There was no history of recurrent regurgitations, heartburn, or epigastric pain. Barium swallow was performed which revealed long segment narrowing in the lower part of the esophagus [Figure 1]. Esophagoscopy showed a narrowing in the lower esophagus approximately 20 cm from the incisors which could not be negotiated by the scope. However, the mucosa just proximal to the narrowing looked normal. A radionuclide gastroesophageal reflux scan revealed mild to moderate gastroesophageal reflux. A provisional diagnosis of CES was made. An exploratory laparotomy with transhiatal mobilization of the lower esophagus was performed. The lower esophagus was thick walled and fibrotic with gritty feeling. The full thickness of the strictured esophagus was laid open, and a fundus of stomach patch akin to that used in fundoplication post-Hellers myotomy was sutured over the esophageal defect. Feeding jejunostomy was also performed. The postoperative dye study showed no esophageal leak, but there was a persistent narrowing in the midesophagus [Figure 2]a. Symptomatically, the child initially improved with acceptance of semisolids but after 2-3 weeks was able to swallow only liquids. Esophagoscopy was repeated which showed an unyielding stenosis in the lower esophagus. Exploratory laparotomy was performed to perform further transhiatal mobilization of the strictured esophagus followed stricturoplasty. The procedure had to be abandoned due to vascular adhesions of the bowel near the hiatus with a future plan to explore again after 3 months through a thoracotomy. After 3 months, a thoracic approach was planned with an option to conversion to thoracoabdominal access. The patient was placed in supine position and incision was given across the left 7 th intercostal space till the midline. The esophagus was accessed through an extrapleural approach. The part of esophagus proximal to the previous site of myotomy was narrowed and had gritty feeling on palpation suggestive of the presence of cartilage. This part of the esophagus was laid open. The uppermost part of the stomach which was used as a patch over the previous esophagostomy was opened transversely and sutured to the newly created defect in the esophagus. This was done without dismantling the fundal patch placed previously. The postoperative period was uneventful. The postoperative contrast study revealed a patent normal looking esophagus till the midthoracic region [Figure 2]b. Distal to the midthoracic region the contrast was going down both through the native esophagus and through the gastroesophagoplasty. The child was asymptomatic in the postoperative period, and once he had an adequate oral intake of solids, feeding jejunostomy was removed. The child presented again after 3 months with difficulty in accepting solids for which he underwent esophageal dilatation. The child has now remained asymptomatic for 1 year and has gained weight.
Figure 1: Barium swallow AP view (a) and Lateral view (b) showing a stenosis in the lower esophagus at the level of 8th thoracic vertebrae (white arrowhead). The part distal to the stenosed part is also not significantly distended

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Figure 2: (a) Barium swallow following transhiatal mobilization, stricturotomy and fundal patch shows a persistent narrowing in the esophagus at T8 level (white arrowhead). The distal esophagus is normally distended (white arrow) and the fundal patch is seen in the left hemithorax (black arrowhead). (b) Barium swallow following complete stricturoplasty and esophagogastric anastomosis with no evident stricture. The contrast is passive through the native esophagus (white arrow) as well as esophagogastric anastomosis (white arrow)

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   Discussion Top

CES is most commonly classified based upon classification suggested by Nihoul-Fékété et al. [3] They classified CES into 3 types: (1) Tracheobronchial remnants (TBR) (2) fibromuscular stenosis (FMS): Segmental hypertrophy of the muscularis and diffuse fibrosis of the submucosa (3) membranous diaphragm (MD). Of these, the TBR is the most common whereas MD is the least common.

Overall CES is more common in the lower third of the esophagus and the treatment for the most common type, i.e. TBR is essentially surgical which may involve segment resection and anastomosis of the esophagus, circular myectomy or enulceation of the cartilage. [4] Most patients with FMS and MD respond to dilatation and few require surgical intervention. Preoperative diagnosis is often confusing, especially in olden children, where acquired causes such as strictures secondary to reflux may also be present. On esophagogram, TBR will often show an abrupt stenosis whereas FMS may show a gradual tapering. Esophagoscopy reveals a normal mucosa overlying the stenosed segment which rules out strictures secondary to corrosive or reflux. Lately, endoscopic ultrasonography is being used to reliably differentially between TBS and other types which helps in dictating management.

The presence of symptoms since infancy, normal mucosa on esophagoscopy, findings in esophagogram and intraoperative findings supported the diagnosis of a CES in the index case. Another aspect that is unique in this case is the long segment of the esophagus was involved rather than 1-2 cm that is usually described. An in-depth search of the literature reveals similar long segment strictures. One of the early publications by Bluestone et al. in 1969 details the presence of stenosis which continued to taper till the gastroesophageal junction. [1] Multiple CES have also been described previously. [4],[5]

The type of CES in the index case was probably TBR type. The gold standard for diagnosis the TBR type is a histopathological examination of the resected esophageal wall which shows cartilage rests. Since the latter was not performed in our case, the diagnosis can be debated on. However what is more important and clinically relevant is the management of these rare and difficult cases where the standard procedures such as resection and anastomosis of esophagus, enucleation, and myectomy are not possible. In management of severe varieties of CES, procedures like cardioplasty, [4] Collis gastroplasty-Nissen fundoplication [6] and esophageal replacement [1] have been described. Irrespective of the etiology of the esophageal stricture, which may be acquired or congenital, the aim of the treatment is to allow the patients to eat normally and be asymptomatic. Anderson et al. [7] suggested that stricturotomy can be considered in difficult cases before condemning the native esophagus in favor of replacement.

The first procedure which we performed wherein the stricture was laid open and a fundal patch was applied is a described procedure for management of peptic esophageal stricture. [8],[9] The third surgery that we performed was unpremeditated and was performed as the fundal patch could be easily brought to the laid open esophagus.

CES is a rare entity but should be suspected in children with dysphagia who are symptomatic since infancy. TBR type is the most common and needs surgical intervention. In severe or long strictures, a standard procedure like resection and anastomosis may not be feasible. In such cases, the surgeon should have an open mind and use whatever is available to him while maintaining basic surgical principles as was done in this case. The surgical procedures have to be tailored based on the individual cases for an optimum result.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Bluestone CD, Kerry R, Sieber WK. Congenital esophageal stenosis. Laryngoscope 1969;79:1095-103.  Back to cited text no. 1
Amae S, Nio M, Kamiyama T, Ishii T, Yoshida S, Hayashi Y, et al. Clinical characteristics and management of congenital esophageal stenosis: A report on 14 cases. J Pediatr Surg 2003;38:565-70.  Back to cited text no. 2
Nihoul-Fékété C, Backer AD, Lortat-Jacob S, Pellerin D. Congenital esophageal stenosis. Pediatr Surg Int 1987;2:86-92.  Back to cited text no. 3
Takamizawa S, Tsugawa C, Mouri N, Satoh S, Kanegawa K, Nishijima E, et al. Congenital esophageal stenosis: Therapeutic strategy based on etiology. J Pediatr Surg 2002;37:197-201.  Back to cited text no. 4
Ramesh JC, Ramanujam TM, Jayaram G. Congenital esophageal stenosis: Report of three cases, literature review, and a proposed classification. Pediatr Surg Int 2001;17:188-92.  Back to cited text no. 5
Chahine AA, Campbell AB, Hoffman MA. Management of congenital distal esophageal stenosis with combined Collis gastroplasty Nissen fundoplication. Pediatr Surg Int 1995;10:23-5.  Back to cited text no. 6
Anderson KD, Acosta JM, Meyer MS, Sherman NJ. Application of the principles of myotomy and strictureplasty for treatment of esophageal strictures. J Pediatr Surg 2002;37:403-6.  Back to cited text no. 7
Hollenbeck JI, Woodward ER. Treatment of peptic esophageal stricture with combined fundic patch-fundoplication. Ann Surg 1975;182:472-7.  Back to cited text no. 8
Maher JW, Hocking MP, Woodward ER. Long-term follow-up of the combined fundic patch fundoplication for treatment of longitudinal peptic strictures of the esophagus. Ann Surg 1981;194:64-9.  Back to cited text no. 9


  [Figure 1], [Figure 2]

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