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| CASE REPORT |
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| Year : 2020 | Volume
: 6
| Issue : 1 | Page : 96-97 |
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Long upper pouch overlapping with lower pouch associated with esophageal atresia and trachea-Esophageal fistula
Zaheer hasan1, Digamber Choubey2, Vinit Kumar Thakur1, Vijayendra Kumar3, Ramdhani Yadav4, Sandeep Kumar Rahul5, Ramjee Prasad5
1 Additional Professor, IGIMS, India
2 Assistant Professor, IGIMS, India
3 Professor & Head, IGIMS, India
4 Associate Professor, Dept. of Paediatric Surgery, IGIMS, India
| Date of Submission | 09-Dec-2019 |
| Date of Acceptance | 21-Feb-2020 |
| Date of Web Publication | 16-Nov-2020 |
Correspondence Address:
Zaheer hasan
Additional Professor Dept. of Paediatric Surgery, IGIMS, Patna
India
 Source of Support: None, Conflict of Interest: None
Esophageal atresia (EA) with tracheoesophageal fistula (TEF) is one of the common neonatal surgical emergencies presenting with excessive salivation, and inability to swallow or pass a firm cathether into the stomach. The present report describes an unusual case of EA-TEF with a long upper esophageal pouch that overlaps the lower esophageal pouch reaching just above the dome of diaphragm. The lower pouch fistula was present at the usual site (Kluth type IIIb6). The redundant upper pouch was excised and TEF was ligated and divided, and an end-to-end esophageal anastomosis was performed. In the post-operative period baby developed sepsis and could not be revived. However, the case was described due to rarity of presentation.
Keywords: Esophageal atresia,Kluth typeIIIb6, Long upper pouch,Tracheoesophageal fistula
How to cite this article:
hasan Z, Choubey D, Thakur VK, Kumar V, Yadav R, Rahul SK, Prasad R. Long upper pouch overlapping with lower pouch associated with esophageal atresia and trachea-Esophageal fistula. J Indira Gandhi Inst Med Sci 2020;6:96-7 |
How to cite this URL:
hasan Z, Choubey D, Thakur VK, Kumar V, Yadav R, Rahul SK, Prasad R. Long upper pouch overlapping with lower pouch associated with esophageal atresia and trachea-Esophageal fistula. J Indira Gandhi Inst Med Sci [serial online] 2020 [cited 2020 Nov 24];6:96-7. Available from: http://www.jigims.co.in/text.asp?2020/6/1/96/300753 |
| Introduction | |  |
Esophageal atresia (EA) with tracheoesophageal fistula (TEF) is one of the common neonatal surgical emergencies presenting with excessive salivation, and inability to swallow or pass a firm catheter through the mouth or nose into the stomach[1]. The present report describes an unusual case of EA-TEF with a long upper esophageal pouch that overlaps the lower esophagus allowing the passage of an infant feeding tube (IFT) to a sufficient distance beyond the fistula site causing diagnostic confusion.
| Case Report | | |
A two day male baby weighing 2.2 kg was brought to our surgical emergency with history of respiratory distress, drooling of saliva, refusal to feed and excess cry. Baby was born to full term, multi-gravida and delivered by lower segment Caesarean section (LSCS). Baby cried soon after birth. No antenatal sonography was done. Routine blood investigation was within normal limits. Echocardiography showed large ASD 8mm and VSD 2.4 mm. As esophageal atresia was suspected 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 ninth thoracic vertebral level with a normal abdominal gas pattern [Figure 1]A. C -Arm X-ray showed a dilated blind ending esophageal pouch extending down to the ninth thoracic vertebral level reaching near dome of diaphragm. [Figure 1] 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 dilated upper esophageal pouch, [Figure 2]which progressed beyond the fistulous communication overlapping with lower esophagus and terminating just above the diaphragm. The lower esophageal fistula with trachea was at the usual site [Figure 3]. The overall anatomy was shown in [Figure 4]. The redundant upper pouch was excised and TEF was ligated and divided, and an end-to-end esophageal anastomosis was performed. In the postoperative period baby developed sepsis and was put on ventilatory support. Unfortunately, baby could not be revived and succumbed on 5thpost-operati’ve day. | Figure 3: Photograph showing overlapping upper pouch with lower pouch fistula
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| Discussion | | |
Esophageal atresia (EA) is checked by passing a firm feeding catheter from mouth or nose into the stomach. If the tube does not pass beyond 10-13 cm, a diagnosis of EA is assumed, and radiographs are taken to establish the diagnosis.[2] On the other hand, if the tube passes beyond that length diagnosis is usually excluded.[3]
Although, Kluth[4] had described 96 variants of EA, commonest was type C EA-TEF. The overgrown upper esophageal pouch is extremely rare and is seen with Kluth types IIIb4, IIIb5, IIIb6. In type IIIb4 distal fistula site was high on trachea, in IIIb5 there was long atretic fistular strand and in the type IIIb6 the fistula from the distal segment ends at the usual site on trachea. The present report describes type IIIb6 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 just above the diaphragm [Fig 1]as demonstrated with C Arm X-ray and the distal fistula was at the level of T4 vertebra. [Fig 2] Dafoe and Ross[5] in their study of 10 cases, reported one similar case. They described this anomaly as partial duplication of the atretic upper esophageal pouch. This case was associated with straighter course of azygous vein which was relatively uncommon anomalous course of the vein. [Fig 2] .The presence of this long upper esophageal pouch may be the reason for late diagnosis and delayed referral. 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 should be following strict guideline for thediagnostic 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 by esophago - bronchoscope. Operative procedure is easy in terms of dissection and mobilization of upper pouch. Outcome of surgery is usually favourable in this type as anastomosis is tension free except when the disease is associated with other abnormalities such as cardiovascular, sepsis, and pneumonia.[7] However, a preoperative CT scan can provide good anatomical details and help in surgical decision making.
| References | | |
| 1. | Harmon CM, Coran AG.Congenital anomalies of the esophagus. In:GrosfeldJL, O’Neill Jr JA, Coran AG, Fonkalsrud EW, editors.Textbook of pediatric surgery, 6th ed.Philadelphia: MOSBY Elsevier;2006. p. 1051-81.  |
| 2. | Alexander A, Millar AJ. The passage of a nasogastric tube does not always exclude an oesophageal atresia. Afr J PaediatrSurg 2009; 6:47-8. |
| 3. | Erdo?an E.An infrequent cause of misdiagnosis in esophageal atresia. J PediatrSurg 2003; 38:1389. |
| 4. | Kluth D. Altas of esophageal atresia. J PediatrSurg 1976; 11:901-19. |
| 5. | Dafoe CS, Ross CA. Tracheo-esophageal fistula and esophageal atresia. Dis Chest 1960; 37:42-51. |
| 6. | 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 diagnostìc dilemma. Dis Esophagus 2011. |
| 7. | Kondo T, Honda S, Minato M, Fujisawa S, Miyagi H, Cho K, et al. A preoperatìve diagnostic challenge of a long overlapping upper pouch with distal tracheoesophageal fistula. J Neonatal Biol 2015; 4:200. |
[Figure 1], [Figure 2], [Figure 3]
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