Congenital Esophageal Stenosis: To Dilate or To Resect

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Annals of Pediatric Surgery, Vol 2, No 1, January 2006, PP 2-9 Original Article Congenital Esophageal Stenosis: To Dilate or To Resect Essam A. Elhalaby *, Mohamed M. Elbarbary **, Amel A.

1 Annals of Pediatric Surgery, Vol 2, No 1, January 2006, PP 2-9 Original Article Congenital Esophageal Stenosis: To Dilate or To Resect Essam A. Elhalaby *, Mohamed M. Elbarbary **, Amel A. Hashish *, Sherif N. Kaddah, Alaa F. Hamza *** Departments of Pediatric Surgery, * Tanta University, Tanta, ** Cairo University, *** Ain Shams University, Cairo, Egypt Background/Purpose: Congenital esophageal stenosis (CES) is a rare condition in children. The definitive preoperative diagnosis often is difficult to make, and a standard therapeutic protocol remains controversial. This study was carried out to determine the proper management strategy in children suffering from CES. Materials and Methods: The medical records of 17 patients with CES treated during a period of 10 years (from 1995 to 2005) were retrospectively reviewed. Each patient was evaluated as regard to the clinical presentation, pathology, management, and outcome. Results: The ages of patients at time of diagnosis ranged from 3 months to 9 years. The sites of stenosis were located more frequently at the lower third of the esophagus (n=10) than the upper third (n=3) and middle third (n=4). The diagnosis was made by esophagogram, esophagoscopy and was confirmed by histopathologic examination. Fifteen patients were diagnosed primarily, while 2 patients were diagnosed after unsuccessful surgical treatment for an initial misdiagnosed achalasia of the cardia. Six patients had confirmed tracheobronchial remnants (TBR), five had fibromuscular stenosis (FMS), and 2 had membranous web stenosis (MS). The histopathogy was unknown in 4 patients due to inadequate biopsies taken during esophagoscopy. All patients were treated initially by repeated esophageal dilatations (2-8 times) over two to thirty month period. The dilatation alone was successful in 11 patients, but was complicated by esophageal perforation in one case. Six patients required surgery; five of them were treated by resection and anastomosis, and one required esophageal replacement. The pathology of this later group was TBR in 5 patients and FMS in one. Conclusion: 1. this study emphasizes the diagnostic difficulties in some children with CES; 2. Esophageal dilatation may be tried initially 3. Resection should be reserved for cases not responding to repeated dilatation particularly those with proven TBR. Index Word: Congenital Esophageal Stenosis, dilatation, endoscopy C INTRODUCTION ongenital esophageal stenosis (CES) is a rare anomaly that usually presents in infancy and childhood. Its incidence is estimated at 1: 23,000 to 50,000 live births. 1 The reported incidence of associated congenital anomalies ranged from 16 to 33%. 1-5 CES may be defined as an intrinsic stenosis of the esophagus, which is caused by congenital malformation of the esophageal wall architecture. 2 There are 3 pathologic types of CES: tracheobronchial remnant (TBR); fibromuscular stenosis (FMS) and Correspondence: Essam Elhalaby, M.D. Department of Pediatric Surgery, Tanta University Hospital, 31111,Tanta, Egypt, Tel.: , eselhalaby@idsc.net.eg

2 membranous stenosis (MS). 2 Tracheobronchial remnant is thought to be a developmental disorder in the formation and separation of the primitive foregut into the trachea and esophagus. Membranous diaphragm is exceedingly rare. It is believed to be produced by incomplete reformation of the esophageal lumen upon recanalization of the esophagus observed in the sixth to eighth week of gestation. In many instances it is difficult to differentiate CES from achalasia and secondary esophageal stenosis, especially strictures caused by reflux esophagitis. 3 The ideal therapeutic strategy remains controversial. It has been reported that cases of FMS may respond to dilatation, but cases of MS or TBR require resection..2-5 Current consensus opinion is that less invasive methods as bougienage or balloon dilatation can be used instead of surgery. 6-8 although repeated dilatation may result in significant complications This aim of this study was to suggest a unified approach for the diagnosis and treatment of this rare condition. MATERIAL AND METHODS This study included 17 patients with CES, who were treated at three academic pediatric surgical centers at Tanta University Hospital, Cairo University Children s Hospital, and Ain Shams University Children s Hospital during a period of 10 years from 1995 to There were 10 boys and 7 girls. The diagnosis was made by esophagogaram, esophagoscopy, and was confirmed by tissue diagnosis of biopsies obtained during endoscopic examination or during surgery. Each patient was evaluated as regard to the clinical presentation, pathology, management and outcome. RESULTS The ages of patients at time of diagnosis ranged from 3 months to 9 years (average 7.6 months). Most of patients had their symptoms started during the first year of life after introduction of semisolid food patients (n=12, 70.5%). The youngest patient was 3 months old when he presented with frequent vomiting and recurrent chest infection. Associated anomalies were noted in 4 patients (23.5%). Two had esophageal hiatal hernia, one had imperforate anus, and one had previous repair of esophageal atresia and tracheoesophageal fistula (EA/ TEF). The clinical characteristics are summarized in Table 1. Vomiting, failure to thrive, dysphagia, wheezing and/or cyanosis were the main presenting features. Food impaction was noted in 4 patients, one of them presented with chocking and cyanosis on swallowing, vomiting dribbling of saliva and chest infection due to complete esophageal obstruction by a swallowed piece of meat, which was impacted at the site of stenosis (fig 1 A&B). Six patients had history of frequent hospitalization for recurrent chest infection before the correct diagnosis was made. Esophgogram was done for all patients. Gastrographin was given orally or was injected through a tube to examine the anatomic abnormalities of the esophagus. Stasis of contrast material was observed proximal to the site of esophageal narrowing (Fig1-3). Endoscopy was conducted under general anesthesia. Endoscopic esophagitis was diagnosed when there was any change in the esophageal mucosa such as: increased friability, erosion, ulceration. The severity of stenosis was assessed and biopsies were obtained from the site of stenosis. Table 1. Clinical Data in 17 patients with CES Clinical Data No of patients (%) Mean age 7.6 months Sex M=10, F =7 Clinical Presentations Vomiting 11(64.7%), Failure to thrive 8 (47%) Dysphagia 7 (41.2%) Food impaction 4 (23.5%) Wheezing and/or cyanosis 4 (23.5%) Recurrent chest infection 6(35.3%) Vol 2, No 1, January

3 Fig 1 A. Esophagogram: AP view in an 8 month old infant presented with cyanosis, chocking on swallowing, vomiting, and dribbling of saliva due to CES at the upper third. Fig 1 B. Esophagogram: Lateral view showing a filling defect due to impacted bolus of food causing complete esophageal obstruction. Fig 2. Esophagogram: a localized stenosis due to a membranous type of CES. Fig 3. Esophagogram : CES at the distal esophagus misdiagnosed initially as an achalasia of the cardia. 4 Annals of Pediatric Surgery

due to CES, membranous type, at the lower third Fig 4 B.

5. Photomicrograph showing cartilage (arrows) and other

Operative photograph showing the site of CES at distal part of

Pathology and treatment of 17 patients with CES Pathology Dilatation

Procedure (n) Tracheobronchial remnants (TBR) 1 5 Resection &

4 Fig 4 A. Upper GIT Endoscopy for a 10 month old infant presented with dysphagia, due to CES, membranous type, at the lower third Fig 4 B. Same patient in fig 4A after endoscopic vulguration and dilatation Fig 5. Photomicrograph showing cartilage (arrows) and other tracheobronchial remnants in the resected stenotic segment Fig 6. Operative photograph showing the site of CES at distal part of esophagus (arrow) operated through abdominal approach Table 2. Pathology and treatment of 17 patients with CES Pathology Dilatation +/_ endoscopic fulguration Dilatation + Surgery Definitive Surgical Procedure (n) Tracheobronchial remnants (TBR) 1 5 Resection & anastomosis ( n=4) Colon transposition( n=1) Fibromuscular stenosis (FMS) 4 1 Resection & anastomosis ( n=1) Membranous web stenosis (MS) Unknown histopathology Vol 2, No 1, January

5 Fifteen patients were diagnosed primarily, while two were diagnosed after surgical treatment for an initial misdiagnosis of achalasia of the cardia. Six patients had confirmed TBR on histopathological examination, five had FMS and two had MS. The histopathogy was unknown in 4 patients due to inadequate biopsies taken during esophagoscopy. The stenosis was located at the lower third of the esophagus in 10 patients (58.8%), the middle third in 4 patients (23.5%) and at he upper third in 3 patients (17.6%). Manometric studies were used to measure intraluminal pressure changes in the pharynx, esophageal body and lower esophageal sphincter and stomach. In this study, manometry were conducted in 4 patients, it showed another high pressure zone (HPZ) in addition to the lower esophageal sphincter in 3 patients All patients underwent conservative treatment by repeated balloon dilatations (n=10) or bougienage (n=7). The number of dilatation session ranged between 2-8 times over a period of 30 months. Dilatation alone was successful in 11 patients, but was complicated by esophageal perforation in one patient. He was managed conservatively (chest tube drainage of the pneumothorax), and has not required any further treatment. Both the two patients with MS were treated successfully by endoscopic fulguration of the membranous web. (Fig 4 A&B) Six patients required surgical treatment after the unsatisfactory response to conservative treatment (Table 2). Five of them had limited esophageal resection followed by an end to end anastomosis. In one patient the gap was long and he required colon interposition. Resection of the stenotic segment was performed through left posterolateral thoracotomy in 4 cases and through an upper abdominal approach for very low esophageal lesion in the other 2 patients (Fig 5). The stenotic segment was detected during surgery by observing the transition zone between the proximally dilated esophagus and the relatively narrow distal stenotic segment. The extent of stenotic segment was assessed by manual palpation and by insertion of a catheter with a balloon. The inflated balloon stopped at the site of stenosis. The length of the resected stenotic segments, apart from the case that needed esophageal replacement by colon replacement, ranged from 1.5 to 3 cm. An antireflux procedure (Nissen fundoplication) was done in two patients. One patient undergone myotomy for initial misdiagnosis of achalasia of the cardia. The symptoms persisted after myotomy. Excision of the stenotic segment and colon interposition was required subsequently. Pathological examination of the resected segment confirmed the presence of TBR (Fig 6). The postoperative course was uneventful in all patients, who were followed up over a period of 12 months to 5 years. One patient required 2 sessions of dilatation postoperatively. All patients had gained average body weight within a period of 8 months to 3 years after treatment. One patient had recurrent attacks of chest infection due to existing gastroesophageal reflux (GER), and probably will need fundoplication. DISCUSSION Esophageal stenosis in children is commonly caused by congenital malformation, gastroesophageal reflux or achalasia. 11 CES is rare, accounting for only 4 % of all cases of esophageal stenosis. 12 CES was first reported by Frey and Duschel in The first report of CES associated with esophageal atresia was by Dunbar in 1958, and up to 1994 less than 50 cases had been described indicating the rarity of that abnormality The current series included 17 patients, from 3 of the largest five pediatric surgical units in Egypt. It should be clear that this number does not represent the total number of CES treated at these institutions during the study period (10 years), but it does represent the cumulative personal experience of the participating surgeons in this study. We believe that CES may be more common than generally thought. The diagnosis of CES is usually difficult to make on clinical grounds only. Because of the rarity of CES and unawareness of many pediatricians about this pathology, many patients are presenting relatively late. One of the characteristic diagnostic clinical features is the presentation of food impaction in a child, who had no previous history of corrosive esophagitis or gastroesophageal reflux esophagitis as noted in 2 patients in this series. Upper gastrointestinal study of infants and children who have repeated vomiting and dysphagia after starting solid food provides the most reliable information. 14 Endoscopic examination usually identifies the cause of stenosis and may rule in /out 6 Annals of Pediatric Surgery

6 the presence of other esophageal lesions. Likewise, both the 24 hour ph monitoring and esophageal motility studies may be needed to exclude the GER as a cause for esophageal stricture which may be difficult to differentiate from CES. Recently Kouchi et al used endosonography to provide detailed information of the esophageal wall that enable the surgeon to determine the optimal intervention in the pediatric age group. 17 In a preliminary experience, both Takamizawa et al 18 and Usui et al 19 reported that endosonography could evaluate the mucosal and submucosal lesions and can pick up cases of TBR which appears as hypoechoic areas at the site of stenosis. These will help in deciding the treatment plan, as cases of TBR are more amenable to surgical intervention. Videomanometry is another recently developed technique that permits the simultaneous recording of intraluminal pressure and dynamic changes in fluoroscopic images Various incidences of CES with EA/TEF have been reported. The incidence rate varies from 0.4%to 14%. 2,3,11. Only one case (0.06%) of EA/TEF was noted in the current series. It might be beneficial to get a biopsy from the distal esophageal segment during the repair of TEF. Dilatation has been an accepted strategy for managing esophageal stenosis regardless its cause for more than 30 years. 22 In the current series; all patients were treated initially with attempted esophageal dilatations. Eleven of 17 patients responded well to dilatation. Multiple dilatations (2-8 times) were needed. There was no evidence of restenosis after follow up period ranged from 6 months to 9 years in those patients. Balloon dilatation has been considered the treatment of choice in cases of MS. 18 Sarihan and Abes showed in their series that 2 of the 3 MS type were treated successfully with esophageal dilatation. 24 Also Takamizawa et al reported that esophageal dilatation was effective in 10 of 13 of their patients with FMS. 18 Balloon dilatation was reported to be more effective and safer than bougienage 18 With balloon dilatation, expanding force is transmitted only to the stenotic segment of the esophagus, and correct placement of the balloon can be determined by direct endoscopic visualization.. In our series we used both balloon and bougienage dilatation. We had failure of dilatation in 5 of the 6 patients with TBR and in only one of 5 patients with proved FMS. In a collective study done by Amae et al, they reported that among 115 cases in different 27 studies, eighty patients (69.6%) underwent surgical treatment. Of them 48 patients (60%) had TBR type and 17(21.3%) had FMS type. This seems to indicate that TBR form of CES has a tendency to resist conservative treatment. 3 The rest of the patient in this collective study (35 patients, 30.4%) improved without surgical treatment. The pathologic features of the stenosis in most of the patients were not examined because no specimen is obtained at the time of dilatation. Based on their analysis of these studies, Amae et al concluded that, it is difficult to determine any actual relationship between the results of dilatation and its pathologic type. 3 On the other hand; our study as well as many others 5, 6,23,25-,28 confirmed the presence of correlation between the pathological nature of CES and its response to dilatation, as almost 83% of patients in this series with failed dilatation regimen proved to have TBR. The practical problem is that it is not always feasible to get enough tissue during esophagoscpy for an accurate pathologic study. 3 Esophageal dilatation is not without complications. Esophageal perforation, aspiration pneumonia and cardiac arrest are reported after dilatation in many studies. 7,9,10,29 Newman and Bender reported perforation in 6 of 7 patients treated with dilatation. In the current study, perforation occurred in one patient. Unneeded repeated dilatation or using the wrong application of dilatation may be the cause of this complication. So special care must be taken to provide most effective therapy for patients faced with this particular lesion. 30,31 Surgical intervention should be considered when sufficient dilatation is not achieved. Takamizawa et al recommended that if dilatation are required within 6 months intervals and remain ineffective after 3 years, surgical intervention should be undertaken. 18 Several techniques for surgical intervention had been reported including: complete resection of the stenotic segment and end to end anastomosis which was performed in 5 patients, resection of the stenotic segment and colon interposition may be needed in long stenotic segment as in one case in this series. Takamizawa et al reported that cases of TBR must be treated by surgical excision, either by resection of the stenotic region followed by end to end anastomosis or by enucleation of the cartilaginous remnants. 18 A complete resection of the stenotic segment and anastomosis has been a reliable treatment; however, resection and anastomosis of full Vol 2, No 1, January

7 esophageal wall may have risk of leakage or restenosis at the anastomotic site. 14 In our series all patients, who had surgery had uneventful postoperative course. One patient required 2 session of dilatation at the 6 th and 8 th months postoperatively. Another way of surgical treatment of CES is motomy. The use of myotomy for FMS is still unclear. 18 esophageal perforation developed after myotomy has been reported. 18 Circular myotomy has been reported and allowed extirpation of the cartilage segment in the esophageal wall and disarrangement of the muscular layer of the esophagus. 14 In our study myotomy was not effective in the patient who was treated as achalasia and complete resection was done subsequently. CONCLUSIONS The diagnosis of CES is usually difficult to make on clinical grounds only. A high index of suspicion is needed and a liberal use of esohphgogram is recommended.. Esophagoscopy with specimen histopathology is useful in differentiating TBR cases from FMS and MS. The type of CES usually determines the modality of treatment Hydrostatic dilatation is safe, simple and effective in many cases of CES. Dilatation should be tried initially in all patients with CES. Surgical intervention should be considered when sufficient dilatation is not achieved, if symptoms recur very shortly after dilatation, or in cases of TBR. REFERENCES 1. Rowe MI, O Neill JA, Grosfeld JL, et al: Essentials of Pediatric Surgery. St Louis, MO, Mosby Year-Book, pp , Nihoul-Fekete C, Backer A De, Lortat-Jacob S, et al: Congenital esophageal stenosis. A review of 20 cases. Pediatr Surg Int 2:86-92, Amae S, Nio M, Kamiyama T, et al: Clinical characteristics and management of congenital esophageal stenosis: A report on 14 Cases. J Pediatr Surg 38: , Yeung CK, Spitz L, Brereton RJ, et al: Congenital esophageal stenosis due to tracheobronchial remnants: A rare but important association with esophageal atresia. J Pediatr Surg 27: , Neilson IR, Croitoru DP, Guttman FM, et al: Distal congenital esophageal stenosis associated with esophageal atresia. J Pediatr Surg 26: , Shorter NA, Mooney DP, Vaccaro TJ, et al: Hydrostatic balloon dilatation of congenital esophageal stenosis associated with esophageal atresia. J Pediatr Surg 35: , Kawahara H, Imura K, Yagi M, et al: Clinical characteristics of congenital esophageal stenosis distal to associated esophageal atresia. Surgery 129:29-38, Diab N, Daher P, Ghorayeb Z, et al: Congenital esophageal stenosis. Eur J Pediatr Surg 9: , Panieri E, Millar AJW, Rode H: Iatrogenic esophageal perforation in children: Patterns of injury, presentation, management, and outcome. J Pediatr Surg 31: , Lisy J, Hetkova M, Snajdauf J: Long-term outcomes of balloon dilatation of esophageal stricture in children. Acad Radiol 5: , Sneed WF, LaGarde DC, Kogutt MS, et al: Esophageal stenosis due to cartilaginous tracheobranchial remnants. J Pediatr Surg 14: , Gray SW, Skandalakis JE. Embryology for surgeons. Philadelphia 7 Saunders , Nagae I, Tsuchida A, Tanabe Y, et al:: High-grade congenital esophageal stenosis owing to a membranous diaphragm with tracheoesophageal fistula. J Pediatr Surg 40: E11 E13, Maeda K, Hisamatsu C, Hasegawa T, et al: Circular myectomy for the treatment of congenital esophageal stenosis owing to tracheobronchial remnant. J Pediatr Surg 39: , Margarit J, Castano n M, Ribo JM, et al: Congenital esophageal stenosis associated with tracheoesophageal fistula. Pediatr Surg Int 9: , Shorter NA, Mooney DP, Vaccaro TJ, et al: Hydrostatic balloon dilation of congenital esophageal stenoses associated with esophageal atresia. J Pediatr Surge 35: , Kouchi K, Yoshida H, Matsunaga T, et al: Endosonographic evaluation in two children with esophageal stenosis. J Pediatr Surg l37: , Takamizawa S, Tsugawa C, Mouri N, et al: Congenital esophageal stenosis: therapeutic strategy based on etiology. J Pediatr Surge 37: , Usui N, Kamata S, Kawahara H, et al: Usefulness of endoscopic ultrasonography in the diagnosis of congenital esophageal stenosis. J Pediatr Surg 37: , Annals of Pediatric Surgery

8 20. Massey BT, Dodds WJ, Hogan WJ, et al: Abnormal esophageal motility: An analysis of concurrent radiographic and manometric findings. Gastroenterology 101: , Kawahara H, Kubota A, Okuyama H, et al: The usefulness of videomanometry for studying pediatric esophageal motor disease. J Pediatr Surg 39: , Boix-Ochoa J, Rehbein F: Oesphageal stenosis due to reflux oesophagitis. Arch Dis Child 40:197, Nishina T, Tsuchida Y, Saito S: Congenital esophageal stenosis due to tracheobronchial remnants and its associated anomalies. J Pediatr Surg 16: , Sarihan H, Abes M: Congenital esophageal stenosis. J Cardiovasc Surg 38: , Grabowski ST, Andrews DA: Upper esophageal stenosis: Two case reports. J Pediatr Surg 31: , Roy GT, Cohen RC, Willeams SJ: Endoscopic laser division of an esophageal web in a child. J Pediatr Surg 31: , Feng FH, Kong MS: Congenital esophageal stenosis treated with endoscopic balloon dilation: Report of one case. Acta Pediatr Tw 40: , Garau P, Orenstein SR: Congenital esophageal stenosis treated by balloon dilation J. Pediatr Gastroenterol Nutr 16:98-101, Morger R, Muller M, Sennhauser F, et al: Congenital esophageal stenosis. Eur J Pediatr Surg 1: , Newman B, Bender TM: Esophageal atresia/tracheoesophageal fistula and associated congenital esophageal stenosis. Pediatr Radiol 27: , Vasudevan SA, Kerendi F, Lee H, et al: Management of congenital esophageal stenosis. J Pediatr Surg 37: , 2002 Vol 2, No 1, January

Dysphagia after EA repair. Disclosure. Learning objectives 9/17/2013

Dysphagia after EA repair. Disclosure. Learning objectives 9/17/2013 Dysphagia after EA repair Christophe Faure, M.D. Professor of Pediatrics, Division of Pediatric Gastroenterology, Sainte-Justine University Health Center, Université de Montréal, Montréal, QC, Canada christophe.faure@umontreal.ca