Spectrum of Disease in Hospitalized Newborns with Congenital Micrognathia: A Cohort of 3,236 Infants at North American Tertiary-Care Intensive Care Units

J Pediatr. 2024 Feb:265:113799. doi: 10.1016/j.jpeds.2023.113799. Epub 2023 Oct 23.

Abstract

Objective: To describe the spectrum of disease and burden of care in infants with congenital micrognathia from a multicenter cohort hospitalized at tertiary care centers.

Study design: The Children's Hospitals Neonatal Database was queried from 2010 through 2020 for infants diagnosed with micrognathia. Demographics, presence of genetic syndromes, and cleft status were summarized. Outcomes included death, length of hospitalization, neonatal surgery, and feeding and respiratory support at discharge.

Results: Analysis included 3,236 infants with congenital micrognathia. Cleft palate was identified in 1266 (39.1%). A genetic syndrome associated with micrognathia was diagnosed during the neonatal hospitalization in 256 (7.9%). Median (IQR) length of hospitalization was 35 (16, 63) days. Death during the hospitalization (n = 228, 6.8%) was associated with absence of cleft palate (4.4%, P < .001) and maternal Black race (11.6%, P < .001). During the neonatal hospitalization, 1289 (39.7%) underwent surgery to correct airway obstruction and 1059 (32.7%) underwent gastrostomy tube placement. At the time of discharge, 1035 (40.3%) were exclusively feeding orally. There was significant variability between centers related to length of stay and presence of a feeding tube at discharge (P < .001 for both).

Conclusions: Infants hospitalized with congenital micrognathia have a significant burden of disease, commonly receive surgical intervention, and most often require tube feedings at hospital discharge. We identified disparities based on race and among centers. Development of evidence-based guidelines could improve neonatal care.

Keywords: cleft palate; mandibular distraction osteogenesis; micrognathia; robin sequence; tongue-lip adhesion.

Publication types

  • Multicenter Study

MeSH terms

  • Airway Obstruction* / surgery
  • Child
  • Cleft Palate* / epidemiology
  • Cleft Palate* / surgery
  • Humans
  • Infant
  • Infant, Newborn
  • Intensive Care Units
  • Micrognathism* / epidemiology
  • Micrognathism* / surgery
  • North America
  • Retrospective Studies