PMY Tang, EKW Chan, NSY Chao, KH Lee

Abstract

Introduction: Congenital hypoplasia of depressor angularis oris muscle (CHDAOM) is a rare cause of asymmetric crying facies in newborn. The association of CHDAOM with bilateral cleft lip and palate has not been reported. Aims: We report a case of a neonate with bilateral cleft lip and palate and CHDAOM. Methods: A full term baby girl with bilateral cleft lip and palate was transferred to our hospital for centralised care of cleft anomalies. She was born by spontaneous vaginal delivery with a birth weight of 2.4 kg and no history of birth trauma. Clinical examination showed complete bilateral cleft lip and palate. Upon further evaluation, while her facial features remained symmetrical at rest, there was drooping (downward and lateral movement) of the left sided lower corner of the mouth on crying, with preserved upper face symmetry. Magnetic resonance imaging brain confirmed normal brainstem structure. Genetic test did not reveal any microdeletion of the chromosome 22q11. Echocardiogram showed no significant cardiac structural anomalies. As the baby was unable to tolerate oral feeding with significant choking and recurrent aspiration, endoscopy was performed and it showed right partial vocal cord paralysis. Ultrasound of the lips showed thinner and smaller right depressor angularis oris muscle, confirming the diagnosis of CHDAOM on the right. Results: As the baby remained tube - feeding dependent, elective laparoscopic gastrostomy and surgical repair of the bilateral cleft lip were performed at 4 months of age. Post-op recovery was uneventful. Conclusions: While surgeons might be well familiar with the pre-operative management of baby with cleft lip anomalies, it is important to observe the baby for other unusual facial anomalies which in this case only become apparent at crying while manifesting a contralateral pathology. Timely documentation, appropriate investigations and anomalies screening, counselling on expectations on surgical outcomes are important when managing patient with cleft lip/palate anomalies and CHDAOM.