Popliteal pterygium syndrome (PPS) with intra-alveolar syngnathia: A discussion of anesthetic and surgical considerations

Syngnathia and obstructive apnea in a case of popliteal pterygium syndrome

“…A majority of cases reported in the literature undergo lysis of these adhesions shortly after birth. While the use of GA during these procedures has prompted many to secure the airway with intubation during and immediately after the procedure [3], we are aware of only one report of airway compromise in a PPS patient. Steinberg et al reported a 5-day-old infant with PPS who underwent surgical release of intra-oral adhesions under GA; the infant developed respiratory distress shortly after completion of the procedure, and he briefly required an oral airway for respiratory support [10].…”

“…A unique pyramidal fold of skin overlying the nail of the hallux is a hallmark of the diagnosis [2]. Syngnathia seen in nearly 40% of PPS patients are usually fibrous in type, limiting jaw excursion and creating difficulties in breathing and feeding [2,3]. …”

Airway Management of an Infant Presenting with Syngnathia for Surgical Correction

“…Etiology is unclear although several mechanisms have been proposed. Although surgery has been reported as being relatively simple in case of uncomplicated fibrous strands, airway management is a main challenge and various invasive and non-invasive techniques have been described [3][4][5].…”

“…Reported airway management in case of surgery for syngnathia varies from observation or sedation only, up to full control of the airway via various techniques [2][3][4]. One of those described is with the aid of a fiberoptic bronchoscope (FOB).…”

Popliteal Pterygium Syndrome

“…4. Management (Gahm et al 2007) 1. Early surgical treatment is of importance to enable and preserve oral feeding.…”