Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2023  |  Volume : 28  |  Issue : 3  |  Page : 260--262

Tracheoesophageal fistula with bilateral facial cleft: A rare occurrence

Nitin James Peters, Shaswati Behera, Ramyasree Bade, Shivani Dogra, Shailesh Solanki, Ram Samujh 
 Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Correspondence Address:
Nitin James Peters
Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012


Commissural or lateral facial cleft (macrosomia), classified at Tessier number 7 craniofacial clefts, is a rare congenital anomaly usually associated with deformities of other structures developed from the first and second branchial arches. It affects the esthetics and functional aspect of the oral cavity. Bilateral transverse cleft occurring alone is uncommon and it's with tracheoesophageal fistula (TEF) has not been reported to the best of our knowledge. We report a case of esophageal atresia (EA) and TEF with macrosomia. EA was repaired, and the patient was discharged on full feeds. He is awaiting cleft repair.

How to cite this article:
Peters NJ, Behera S, Bade R, Dogra S, Solanki S, Samujh R. Tracheoesophageal fistula with bilateral facial cleft: A rare occurrence.J Indian Assoc Pediatr Surg 2023;28:260-262

How to cite this URL:
Peters NJ, Behera S, Bade R, Dogra S, Solanki S, Samujh R. Tracheoesophageal fistula with bilateral facial cleft: A rare occurrence. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Sep 30 ];28:260-262
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Full Text


Failed embryonic fusion of mandibular and maxillary processes of the first branchial arch results in lateral or commissural facial clefts with bilateral macrostomia. These are rare and usually associated with deformities of structures developed from the involved arches. Such deformities might include varying degrees of skeletal hypoplasia, discontinuity of facial bones, orofacial clefts, and airway abnormalities. Other syndromic features such as velopharyngeal insufficiencies, sensorineural deafness, cleft lip–palate complex, external auricular anomalies, colobomas, and cardiac anomalies should be anticipated and ruled out preoperatively.[1]

Our index case had esophageal atresia with tracheoesophageal fistula (EA/TEF) associated with bilateral commissural fissures. Such bilateral macrosomia with EA/TEF has not been described to the best of our knowledge. We managed this case with the repair of the EA/TEF and the patient is awaiting lateral cleft repair.

 Case Report

A full-term, 2700 g male baby, born by normal vaginal delivery was referred to our center on day 2 of life with complaints of drooling of saliva from the mouth and respiratory distress since birth. The baby had passed urine and meconium soon after birth and was not fed. The baby was hemodynamically stable and had a fair cry tone activity. On examination, the baby had dysmorphic facies with bilateral low-set ears and commissural defects [Figure 1]a. The presence of preauricular appendages (pretragal skin tags) and macroglossia [Figure 1b] was also remarkable.{Figure 1}

The patient had intermittent chest retractions on both sides without any episodes of desaturation or cyanosis. No other abnormalities were detectable on physical examination.

The bedside orogastric tube was introduced, reaching up to 12 cm. Chest radiograph showed coiling of the nasogastric tube at the level of the 3rd thoracic vertebra [Figure 2]a. A preoperative echocardiogram showed a structurally normal heart with normal biventricular function. Ultrasonography of the cranium and renal system was grossly normal.{Figure 2}

After adequate resuscitation, the baby was taken up for surgery on day 3 of hospitalization.

Under general anesthesia, intubation was done with the help of a concurrent media access control video laryngoscope anticipating a difficult airway. The baby underwent a right posterolateral thoracotomy with an extra-pleural approach. Intraoperatively, a wide TEF of 1 cm arising from a distal pouch was delineated and divided between ligatures. The upper esophageal pouch was healthy and well-developed, while the lower pouch was moderately developed with an intervening gap of 1.5 cm. The repair was done primarily with the help of polyglactin 5-0 intermittent sutures in a single layer.

Postoperatively, the baby was shifted intubated to the neonatal surgical ICU. The patient was weaned off gradually from continuous positive airway pressure and extubated on the 3rd postoperative day with normal lung shadow on the chest radiograph [Figure 2]b.

Feeds in the form of expressed breast milk were started on day 2 after surgery through a nasogastric tube and gradually increased. The patient was fed by palady and eventually by a long nipple bottle, used for managing cleft babies.

The baby is doing well on a close follow-up of 1 year including visits to the otolaryngology and plastic surgery clinic. Surgical management of the lateral cleft is awaited.


Congenital macrosomia is a rare anomaly arising due to abnormal fusion of the first branchial arch during the embryonic period. Thus, associated anomalies could be the derivatives of first or second branchial arches. Lateral clefts are the next common orofacial clefts, second only to isolated cleft lips, with an incidence of 1 in every 3500–5000 live births.[2] Tessier number 7 craniofacial clefts, also referred to as temporozygomatic facial clefts or transverse facial clefts, are the most laterally located subtype of craniofacial clefts. Tessier number 7 is positioned on the line between the corner of the mouth and the ear. This is a rare congenital anomaly, with a frequency of 1/60,000–1/300,000 normal births, and is characterized by soft-tissue abnormalities, such as macrosomia, external ear deformity, and hemifacial microsomia. Bilateral clefts are further uncommon and scarcely reported in the literature.[1],[3] Sometimes, intrauterine amniotic band compression may result in such a presentation. These deformities may range from a simple widening of the oral aperture to a full-thickness oral defect.

Babies suffering from Treacher Collins syndrome or Goldenhar syndrome are reported to have wide oral apertures or facial clefts. Treacher Collins syndrome causes typical facial dysmorphism including transverse facial clefts. Goldenhar syndrome might affect the cardiovascular, renal, and central nervous systems.[4] The index case did not have the typical features or corroboration with the abovementioned clinical syndromes. Some of these cases are described with overlapping deformities as vertebral defects, anal atresia, cardiac defects, TEF, and renal and limb anomalies.[5] However, the workup in our index case had no abnormalities, thus ruling out other associated syndromes.

In case of other associated anomalies, such as EA-TEF, a well-planned surgical and anesthetic approach is mandatory along with staged repair of the cleft anomalies.


Babies with macrosomia or facial clefts may be syndromic and associated with life-threatening systemic defects that need prompt attention. Difficult or atypical airways with bony dysplasia are important from an anesthetic point of view. Aerodigestive anomalies such as TEF and EA should be kept in mind while examining these patients. Treatment options are variable and individualized, ranging from simple closure of orofacial muscles to formal cleft repair and surgical repair of other anomalies such as TEF in the index case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


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