Type III laryngo-tracheo-oesophageal cleft repair without tracheostomy in neonate with long-gap oesophageal atresia| ACTA Otorhinolaryngologica Italica

Article

Dear Editor,

Laryngo-tracheo-oesophageal cleft (LTEC) is a rare congenital anomaly that involves a communication between the upper respiratory and digestive tracts as a consequence of an alteration in tracheo-oesophageal septum development ¹. LTEC is divided into 4 types depending on its extension according to the Benjamin and Inglis classification: type I (supraglottic interarytenoid involvement), type II (partial involvement of the cricoid lamina), type III (total involvement of the cricoid lamina and cervical trachea) and type IV (intrathoracic trachea involvement) ². This congenital malformation is frequently associated with other anomalies in up to 60% of cases, of which tracheoesophageal fistula, oesophageal atresia, and tracheomalacia stand out ¹. Mortality has been attributed directly to associated comorbidities such as poor feeding and aspiration pneumonia, regardless of surgical treatment ³. In terms of post-operative complications, fistula re-formation, recurrent aspiration and tracheomalacia are common, and require close follow-up for early diagnosis and treatment ⁴.

We describe a male born at 36 + 5 weeks of gestational age (2,350 grams), delivered by emergency caesarean section due to loss of foetal well-being, with a prenatal diagnosis of severe polyhydramnios. After patient stabilisation, fibrescopic airway examination revealed a LTEC with cervical tracheal involvement up to 2 cm from the carina (type III) and an atresic oesophageal pouch 2 cm in length, compatible with long-gap oesophageal atresia. Distal gas in abdominal X-ray confirmed the diagnosis of tracheo-oesophageal fistula (TEF) type C according to the Gross classification (type A, isolated oesophageal atresia; type B, proximal fistula with distal atresia; type C, proximal atresia with distal fistula; type D, double fistula with intervening atresia and type E, isolated fistula) ⁵. Physical exam showed anal atresia with bilateral thumb amputation. In addition, abdominal ultrasound found left renal agenesis, all these findings compatible with VACTERL polymalformative syndrome (vertebral defects, anal atresia, cardiac defects, TEF, renal anomalies, and limb abnormalities). The TEF was ligated on the first day of life through an abdominal approach (supraumbilical midline laparotomy), and a 10 Fr Pezzer gastrostomy tube was placed following Stamm’s technique. In the same intervention, a sigmoidostomy was performed, with meconium passing through it 24 hours after surgery.

The LTEC was repaired at two months of age, when a weight of 4500 grams had been reached. An anterior cervical approach was performed, with anterior tracheal opening, followed by reconstruction of the upper oesophageal pouch by separation from surrounding structures to achieve closure of the anterior oesophageal wall by continuous suture in one plane. After tubular reconstruction, a left cervical oesophagostomy was performed. Subsequently, a 30 x 10 mm anterior tibial periosteal graft was interposed between the oesophageal wall and the posterior tracheal wall. Finally, the posterior and anterior tracheal walls were sutured, achieving complete repair of the defect, with extubation on the 7th post-operative day without complications (Fig. 1). Bronchoscopic exams were periodically performed in which the tracheoplasty remained satisfactorily repaired, although a moderate degree of tracheomalacia and partial collapse of the airway were observed. For this reason, a biodegradable tracheal stent (30 x 6 mm) was placed, which was replaced on 2 subsequent occasions, every 3 months until the present day. At present, the patient is 12 months old (10.5 kg), and is growing properly at home, with follow-up bronchoscopies every 6-8 weeks.

Different approaches have been described for the repair of type III-IV LTEC. Anterior cervical approach allows reconstruction from an extended cervicotomy. In this case, the most difficult step was the separation of the posterior tracheal and anterior oesophageal walls. After complete reconstruction of upper oesophageal pouch, a left cervical oesophagostomy was performed, in order to allow adequate saliva outflow after swallowing, reducing the risk of aspiration pneumonia and refistulisation. In fact, one of the major problems after LTEC repair is the occurrence of fistulas between the trachea and the oesophagus. Therefore, several techniques have been described to try to reduce this risk, such as the interposition of sternocleidomastoid muscle flap and three layers of closure ⁶. Our patient also had an associated type III TEF, which had been previously ligated. We decided to perform a tibial periosteal graft interposition between the oesophagus and trachea to prevent possible refistulisation. This avoided the use of other cervical structures such as the sternocleidomastoid muscle, which would have made oesophagostomy performance more difficult.

Tracheotomy is widely used for type III and IV LTEC for protection of the lung against aspiration during enteral feeding. In our case, we chose to avoid performing initial tracheostomy. Therefore, once the anterior tracheal dissection was completed, the anterior tracheal wall was opened longitudinally and a ventilatory endotracheal tube was placed in the healthy trachea distal to the LTEC. Airway management of neonates with tracheo-oesophageal anomalies presents a challenge for neonatologists and anaesthesiologists. Because neonates with this condition are usually at risk of life-threatening respiratory problems, difficult intubation must be generally expected ³. In our patient, the presence of tracheomalacia posed an added difficulty, which is being managed by biodegradable tracheal stents. In most cases, tracheomalacia resolves spontaneously at around 18-24 months, after completion of tracheal cartilage maturation. For this reason, repair of the oesophagus will be performed once definitive airway stability is achieved. The type of oesophageal reconstruction with stomach or colon (gastric pull-up or oesophagocoloplasty respectively) will be also influenced by the associated anorectal malformation (ARM). In the next operation, ARM correction will be performed initially and, subsequently, the oesophageal reconstruction option will be decided upon depending on the availability of the colon after ARM repair.

To sum up, repair of types III-IV LTEC should be considered a surgical challenge that requires complex airway management, and should therefore be performed in experienced centres with paediatric aerodigestive surgery programmes.

Acknowledgements

We would like to thank all the hospital staff who have contributed to the care of the patient from birth to the present day.

Conflict of interest statement

The authors declare no conflict of interest.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author contributions

CDM: designed and conceptualised the study and wrote the draft manuscript; CDT, LC: performed data adquisition and data analysis; CDT, FHO: critically revised the manuscript. All authors approved the final version of the manuscript.

Ethical consideration

This study obtained the approval of the institutional ethics committee and the hospital review board (PI-502788). Child’s parents signed a written informed consent form, which included the publication of the images of this case.

Figures and tables

Figure 1.Bronchoscopic features before LTEC repair (A-B) and 2 weeks after surgical repair (C-D).

References

1. Seidl E, Kramer J, Hoffmann F. Comorbidity and long-term clinical outcome of laryngotracheal clefts types III and IV: systematic analysis of new cases. Pediatr Pulmonol. 2021; 56:138-144. DOI
2. Benjamin B, Inglis A. Minor congenital laryngeal clefts: diagnosis and classification. Ann Otol Rhinol Laryngol. 1989; 98:417-412. DOI
3. Roth B, Rose KG, Benz-Bohm G. Laryngo-tracheo-oesophageal cleft. Clinical features, diagnosis and therapy. Eur J Pediatr. 1983; 140:41-46. DOI
4. Kawaguchi AL, Donahoe PK, Ryan DP. Management and long-term follow-up of patients with types III and IV laryngotracheoesophageal clefts. J Pediatr Surg. 2005; 40:158-164. DOI
5. Yang S, Yang R, Ma X. Detail correction for Gross classification of esophageal atresia based on 434 cases in China. Chin Med J (Engl). 2021; 135:485-487. DOI
6. Geller K, Kim Y, Koempel J. Surgical management of type III and IV laryngotracheoesophageal clefts: the three-layered approach. Int J Pediatr Otorhinolaryngol. 2010; 74:652-657. DOI