A study examined the rare type of tracheocutaneous sinus after repair of a tracheocutaneous fistula and to review management strategies. A tracheocutaneous fistula is a common sequela of pediatric tracheostomy and can occur in as many as one in three pediatric patients. There is a debate in the literature regarding optimal surgical management. Advances in perinatal medicine and healthcare access have increased the number of premature infants requiring the assistance of prolonged ventilation.

Pediatric tracheostomies are performed most commonly to address an upper airway obstruction, ventilator dependence, or central hypoventilation. A tracheocutaneous sinus is a rare sequela following a pediatric tracheostomy relative to TCF, which is a common late complication occurring with a frequency of 6–55%. Subcutaneous emphysema is an early complication after TCF repair and has been reported within the first few weeks following TCF repair.

While TCF is common, there are no documented pediatric cases of delayed subcutaneous emphysema or TCS in the literature. Although exceedingly rare, a TCS, or “tracheocele,” is a variant of a TCF and may be addressed similarly. Surgical repair of a TCF is important as it directly contributes to poor hygiene, poor phonation, intolerance to submersion, social isolation, and risk of aspiration and infection.

TCF can be treated conservatively for the first 3–6 months after decannulation using local curettage, silver nitrate, and cauterization. There is some debate regarding the exact timing of repair; some authors recommend at least a 6-month trial of conservative management to ensure that there is no spontaneous closure, whereas other authors may offer a repair after only three months.

A four-layered closure is then performed, including the tracheal defect, strap muscles, subcutaneous fat, and skin. A portion of the strap muscles is often included in the scarred track, and careful mobilization with the realignment of the strap muscles is critical. In these cases, it is reasonable to discuss standard fistulectomy with healing by secondary intention or fistula tract obliteration via electrocautery since both methods have reported success. 

A pediatric TCS following tracheostomy is a rare sequela of a pediatric tracheostomy. The prolonged cannulation in conjunction with an underlying collagen disorder; Stickler syndrome, may explain this rare TCS Although there were no specific inciting events identified, we hypothesize that slowly developing tracheal micro perforations and a low-grade upper respiratory infection with a cough were likely the initiating factors. While a pediatric TCS is exceedingly rare, the diagnosis is relatively straightforward and the management closely parallels that of a TCF.