Journal of Pediatric Surgery (2011) 46, 2041–2044

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Correspondence

Esophageal atresia with distal fistula and long overlapping upper esophageal pouch To the Editor, Esophageal atresia (EA) with tracheoesophageal fistula (TEF) (EA-TEF) is one of the common neonatal anomalies often presenting with excessive salivation, and inability to feed or pass a feeding tube into the stomach [1]. The present report describes an unusual case of EA-TEF with a long upper esophageal pouch that crossed over the lower esophagus allowing the passage of an infant feeding tube (IFT) to a sufficient distance to give the false impression of the tube passed into the stomach.

1. Case summary A 5-day-old neonate presented in the pediatric surgical emergency department with history of vomiting after attempted feeds and excess salivation. Suspecting esophageal atresia, an attempt was made to pass a 10F catheter IFT through the mouth, which progressed easily to a distance of 25 cm. A chest radiograph with the IFT in position surprisingly showed coiling of the tube at the eighth thoracic vertebral level with a normal abdominal gas pattern (Fig. 1A). Contrast esophagogram showed a dilated blind ending esophageal pouch extending down to the ninth thoracic vertebral level with no dye entering the stomach (Fig. 1B). A diagnosis of EA and TEF with long dilated upper esophageal pouch was made, and the infant was scheduled for thoracotomy. Thoracotomy confirmed the diagnosis of EA-TEF with a blind, hugely dilated upper esophageal pouch, which crossed over the fistulous communication between the lower esophagus and trachea. There was a hard swelling palpable at the lower end of the proximal pouch. The redundant upper pouch was excised along with the hard swelling, the TEF was ligated and divided, and an end-to-end esophageal anastomosis was performed. The postoperative course was uneventful, and the infant was discharged on 12th postoperative day. The biopsy of the excised hard swelling was consistent with elastic cartilage. 0022-3468/$ – see front matter © 2011 Elsevier Inc. All rights reserved.

2. Discussion Kluth [2] had described 96 variants of EA; however, type C EA-TEF remains the most frequently encountered esophageal anomaly [1]. The present report describes an extremely rare variant of EA-TEF in which the long upper pouch becomes hugely dilated and extends distally beyond the TEF site allowing the IFT to reach a sufficient distance giving a false impression of the tube in the stomach. Dafoe and Ross [3], in their series of 10 cases, had described 1 such similar case. They described this anomaly as partial duplication of the atretic upper esophageal pouch. Recently, we reported a similar case in which the crossed over upper esophageal pouch had extended down to the level of the diaphragm [4]. Our previous case posed a difficult diagnostic challenge because even the chest radiograph gave the impression that the IFT was in the upper part of the esophagus. The presence of this long upper esophageal pouch may be the reason for late diagnosis and delayed referral in the present case. The importance of awareness of such a variant is very important for pediatric surgeons to avoid delay in the diagnosis and complications of EA-TEF, which, in this era, is otherwise easily corrected by surgery. We hereby suggest following a strict protocol for the diagnostic workup of a patient with suspected EA. Mere passage of an IFT to an expected distance is not sufficient to rule out EA, and diagnosis should always be confirmed by acquiring a chest radiograph with the tube in place in all the patients. Diagnosis should be confirmed in confusing cases by doing an esophagogram under fluoroscopic guidance and/ or esophagobronchoscopy. Kirtikumar Jagdish Rathod Shraddha Verma Ravi Prakash Kanojia Ram Samujh Katragadda Laksmi Narasimhan Rao Department of Pediatric Surgery Post Graduate Institute of Medical Education and Research Chandigarh 160012, India E-mail address: [email protected] doi: 10.1016/j.jpedsurg.2011.07.016

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Fig. 1 A, Chest radiograph showing IFT coiled and arrested at the eighth thoracic vertebral level. B, Contrast esophagogram showing dilated long upper esophageal pouch.

References [1] Harmon CM, Coran AG. Congenital anomalies of the esophagus. In: Grosfeld JL, O'Neill Jr JA, Coran AG, Fonkalsrud EW, editors. Textbook of pediatric surgery, 6th ed.Philadelphia: MOSBY Elsevier; 2006. p. 1051-81. [2] Kluth D. Altas of esophageal atresia. J Pediatr Surg 1976;11:901-19. [3] Dafoe CS, Ross CA. Tracheo-esophageal fistula and esophageal atresia. Dis Chest 1960;37:42-51. [4] Rathod K, Khanna S, Kanojia RP, et al. A novel variant of esophageal atresia with tracheo-esophageal fistula with a crossed-over proximal esophageal pouch: a diagnostic dilemma. Dis Esophagus 2011;24doi: 10.1111/j.1442-2050.2010.01175.x.

Letter to the Editor To the Editor: We read with great interest the article “Microlithiasis, endoscopic ultrasound, and children: not just little gallstones in little adults” [1] and would like to make some comments. Skull base surgery had been performed in all 3 of your patients, which must be more than coincidental and a link with ceftriaxone use as antibiotic prophylaxis or enforced bed rest would go a long way to establish both a link with gallstone formation (macro or micro) and, therefore, the credibility of the hypothesis. Although we appreciate that different institutions may have been involved, surely, such records must be available somewhere to establish this key point. Endoscopic ultrasound (EUS) is undoubtedly a very sensitive diagnostic modality for choledocholithiasis and

more recently the more controversial microliths. We congratulate the authors in highlighting the use of this diagnostic tool in children with difficult-to-diagnosis right upper quadrant pain. However, ERCP (perhaps in combination with EUS and nasobiliary aspiration) surely must remain the preeminent interventional investigation for recurrent pancreatitis though (only really proved in case no. 3 in your series). Common channel, pancreas divisum, congenital duct strictures, and other are surely more likely as causes than any forme fruste variant of gallstones. We appreciate MRCP but feel that this has the same relationship to sensitivity as conventional ultrasound does to EUS, certainly in the younger child. Cholesterol cholelithiasis is potentially associated with underlying genetic mutations in the ABCB4 gene; this was first described in a cohort of young adults by Rosmorduc et al [2]. Genetic variations result in a reduced level of phospholipids excreted into bile, which is an essential component of micelle formation for the excretion of cholesterol. Bile subsequently becomes supersaturated with cholesterol, which precipitates out as gallstones. Genotypic variations may result in early onset of cholelithiasis presenting in adolescence. Of course, intrahepatic formation of stones or microliths could also be a cause of persistent long-term problems and are not resolved by cholecystectomy [3]. It would be interesting to know if any such genetic analysis was performed on your patients, particularly because there is a positive family history (case no. 1) and recurrent symptoms of abdominal pain in another (case no. 3).