Pediatrics & Therapeutics
Open Access
ISSN: 2161-0665
+44 1478 350008
ISSN: 2161-0665
+44 1478 350008
Ali Ozcan
Icahn School of Medicine at Mount Sinai / Elmhurst Hospital, USA
Posters-Accepted Abstracts: Pediat Therapeut
Case Report: A female neonate was born at term via vaginal delivery to a 28-year-old G2P000 mother. The mother had no significant past medical history, unremarkable prenatal sonograms and denied any alcohol/drug abuse, medications or infection during pregnancy. The delivery was complicated by thick meconium-stained amniotic fluid and, upon birth the infant was limp, with poor muscle tone and cry. An attempt at deep tracheal suctioning was unsuccessful because the mouth could not be opened. The baby started to breathe spontaneously shortly after and did not require further resuscitative effort. On physical examination the baby was noted to have wide adhesion of the tongue��?s tip to the primary palate, micrognathia, absent distal phalanges of fingers one through four on the right hand, agenesis of all fingers on the left hand and agenesis of the left great toe. On digital exploration of the mouth, a significant cleft of the secondary palate was noted and later confirmed by CT scan. The scan also demonstrated bilateral, hypoplastic mandibular rami in which both condyles were anteriorly displaced from the temporal-mandibular joint. The baby was transferred to our referral center where surgical divison of the tongue defect was performed at 23 days of life. The baby required a video-assisted nasotracheal intubation for the procedure, and the tube was left in place at the end of the operation due to significant oropharyngeal swelling. Additionally, the divided tongue was noted to move posteriorly causing additional concerns about obstruction of the oropharynx. The baby underwent tracheostomy placement on day 27 of life and is currently gaining weight, feeding with a modified nipple, and undergoing range of motion exercises to improve jaw opening. Discussion: Glossopalatine ankylosis is a rare congenital condition which involves adherence of the tongue to the hard palate, and is almost inevitably associated with other defects such as cleft palate, mandibular hypoplasia and other digital anomalies (oligo- syn- or polydactyly) [1]. Given the other oral and limb deformities, glossopalatine ankylosis may belong to the group of Oromandibular Limb Hypogenesis Syndromes (OLHS), which include Charlie M syndrome, Moebius syndrome, hypoglossia-hypodactyly syndrome, Hanhart syndrome, and limb deficiency-spleno-gonadal fusion syndrome [2]. However, there is controversy whether these syndromes are varying presentations of OLHS. In this patient, it is likely that the tongue adhesion contributed to the cleft palate by interfering with palatal fusion during gestation. This principle is demonstrated in Pierre-Robin sequence where a hypoplastic mandible displaces the tongue superiorly and posteriorly resulting in a cleft palate and respiratory distress [1,2]. Fortunately in this patient, the tongue adhesion likely maintained airway patency initially by displacing both the tongue and mandible anteriorly. This case gives further support that ankyloglossum superior may belong to OLHS as opposed to a sporadic oral deformity.
Email: ali.ozcan@mssm.edu