89 - Clinical Presentation and Outcomes for Infants with Micrognathia: a report from the Children’s Hospitals Neonatal Consortium Micrognathia Focus Group.

Poster Number: 89
Publication Number: 89.438

Irfan Ahmad, CHOC Children's Hospital, Irvine, CA, United States; Tara L. Wenger, University of Washington Division of Genetic Medicine, Seattle, WA, United States; Semsa Gogcu, Wake Forest Baptist Health - Brenner Children's Hospital, Winston Salem, NC, United States; Cherrie Welch, Wake Forest School of Medicine of Wake Forest Baptist Medical Center, Winston Salem, NC, United States; Cory M. Resnick, Boston Children's Hospital, Boston, MA, United States; Michael A. Padula, Children’s Hospital Of Philadelphia and University of Pennsylvania, Philadelphia, PA, United States; Carl Coghill, University of Alabama School of Medicine, Birmingham, AL, United States; Maithilee Menezes, Washington University in St. Louis School of Medicine, St Louis, MO, United States; Raj M. Vyas, UC Irvine Department of Plastic Surgery, CHOC Children's Hospital, Orange, CA, United States; Janet Lioy, Childrens Hospital of Philadelphia, Newtown, PA, United States; Stephen A. Rottgers, Johns Hopkins All Children’s Hospitsl, Sy Petersburg, FL, United States; Christopher Cielo, Children's Hospital of Philadelphia, Philadelphia, PA, United States; Daniela Dinu, Baylor College of Medicine, Houston, TX, United States

Background: Congenital micrognathia, whether isolated or as part of a syndrome, is a rare condition that often requires tertiary care and can result in feeding difficulties and airway compromise, with some infants requiring surgical interventions. Prior studies are limited to small samples from single-hospital experiences, so the spectrum of disease and its current management is poorly understood.

Objective: To describe the spectrum of disease in infants hospitalized with congenital micrognathia utilizing the Children’s Hospitals Neonatal Database (CHND). CHND represents 34 centers with level IV neonatal intensive care units throughout North America.

Design/Methods: CHND was queried for infants with diagnosis of micrognathia or Pierre Robin Sequence from January 2010 to December 2020. Data including demographics, associated comorbidities, surgical status, and clinical outcomes were summarized.

Results: 3256 infants with congenital micrognathia were included in analysis (Table 1). Nearly 40% of the cohort (n=1273) had a cleft palate and 8% (n=256) had an underlying syndrome associated with micrognathia. Mandibular distraction osteogenesis (MDO) was the most common airway surgery (n= 856, 26%) followed by tracheostomy (n=367, 11.3%). Only 32.10% (n=1044) infants reached full oral feeds and 32.6% (n=1063) required gastrostomy tube placement at discharge from initial hospitalization. 209 (6.4%) died during the neonatal period. Maternal black race (p < 0.001) and absence of cleft palate (p < 0.001) were the only two variables positively association with death. There was significant variability in median length of stay between centers (10 to 45 days, p< 0.001).Conclusion(s): This is the largest sample of infants with congenital micrognathia and represents a wide geographic area across North America. The condition is highly variable, with a high degree of morbidity and mortality. Many infants with micrognathia require airway surgery and feeding difficulty is common. The presence of a cleft palate was associated with a lower risk of mortality, likely due to associated comorbidities and phenotype variability. Length of stay was highly variable among the centers, and health disparities associated with outcomes, including death, may be present. This study provides an overview of the spectrum of disease and outcomes for infants with micrognathia and highlights the need for standardization of practice for infants with this complex condition.
Table 1Demographics
Table 2Key Clinical Outcomes