ABSTRACT
Although early complication of airway obstruction following pharyngoplasty is well recognised, there have been few reports of late modifications following this procedure. We retrospectively review cases with late complications which have required either revision or division of an existing pharyngoplasty at the Australian Craniofacial Unit over the last twenty-five years. We assess the outcome of further surgical intervention in each case, with case note and nasendoscopy video review. Fourteen cases were identified where records were complete. There were 12 males and 2 females. The cases are a heterogeneous group of cleft lip and palate patients and include three cases with a diagnosis of Pierre-Robin sequence and one case with a cleft palate as part of an underlying syndrome. Those cases requiring flap division had undergone either superiorly or inferiorly based pharyngeal flaps in contrast to dynamic (Orticochea) pharyngoplasties which required revision. This series of cases demonstrates the need for thorough assessment and planned tailoring of the pharyngoplasty procedure, with ongoing review of speech and airway function. This management philosophy results in the acceptance that a pharyngoplasty may only be required for a limited period of time and ultimately may be redundant.
ABSTRACT
Linear scleroderma is a variant of localized scleroderma which may occur in the head, trunk and extremities, affecting variously the skin, subcutaneous tissue, muscle, cartilage and bone. Particularly, scleroderma en coup de sabre is the descriptive term denoting linear scleroderma of the frontoparietal area of the scalp and face. We report a case of severe scleroderma en coup de sabre with ipsilateral body atrophy(total hemiatrophy) who underwent multidisciplinary assessment and required both two-jaw surgery and groin free flap to correct the underlying skeletal deformities and augment the facial soft tissues. The reason for classifying this as a case of scleroderma en coup de sabre in preference to Parry-Romberg syndrome is because of the striking clinical features seen in this patient.