Neonatal Gastrointestinal Tract Malformations in a Nonsurgical Tertiary Neonatal Intensive Care Unit

Introduction: Gastrointestinal tract (GIT) malformations diagnosed in a nonsurgical tertiary neonatal intensive care unit (NICU) require immediate initial conservative medical management and urgent transfer along established clinical transfer pathways to a surgical unit as these units do not have access to pediatric surgeons on-site.

Materials and Methods: We conducted a retrospective study over a period of 6 years—January 2010 to December 2016 to ascertain cases diagnosed with GIT malformations in a nonsurgical tertiary NICU. Cases were identified from patient electronic notes, imaging and Badger database.

Results: There were 4,228 total admissions over the period of which 102 (2.4%) had GIT malformations. Most of the infants were diagnosed at > 37 weeks (54%), only 21% were between 30 and 36 weeks, and 25% were between 24 and 30 weeks. Weight was > 2 kg in 60% of cases, while 21% were 1 to 2 kg and 19% were  < 1 kg; only 23% were growth restricted. These malformations were atresias (35 out of 102, 34%), obstructions (23 out of 102, 22%), malrotations (19 out of 102, 18%), perforations (22 out of 102, 21%), and others (1 of intestinal duplication and 2 of eventration). Atresias were made up of: esophageal atresia (EA) (13 out of 35), duodenal atresia (9 out of 35), intestinal atresia (6 out of 33), and imperforate anus (7 out of 35, 1 with rectocutaneous fistula and 1 with rectovulvar fistula). There were 23 infants who were transferred out with a diagnosis of intestinal obstruction; in two cases, volvulus was also identified; in four cases, Hirschsprung disease was diagnosed. In 26% of infants (27 out of 102) diagnosis was made antenatally and 74% (75 out of 102) postnatally, of which 48% (36 out of 75) were identified on Day 1, 36% (27 out of 75) during Days 2–10, and the rest of the patients (16%) between Days 11 and 99; 94% (97 out of 102) of infants were transferred to a surgical unit, 5 infants were managed in the unit—1 for suspected intestinal obstruction, 1 for left diaphragmatic eventration, 1 died before transfer (CDH), and 2 had withdrawal of care (trisomy 18 + EA; 26 weeks with spontaneous intestinal perforation with dysmorphic features and pulmonary hypoplasia). Out of the infants who were transferred to a surgical unit 79% (76 out of 97) required surgery and 21% (20 out of 97) were conservatively managed; 39% of infants required intubation and ventilation at birth. Genetic associations were identified in 14% (15 out of 102) of cases: trisomy 18 and 21 (six cases), cystic fibrosis (two cases), VACTERL (two cases), and singular cases of: Goldenhar syndrome, Donnai Barrow syndrome, CHARGE syndrome, Myotonic Dystrophy, and abnormalities of chromosomes 3 and 7. Mortality was of 10% and it was due to CDH (two cases), Staphylococcus sepsis (one case), EA and dextrocardia (one case) and in six cases after withdrawal of care.

Conclusion: GIT malformations formed a small number of admissions over the 6-year period. Majority was diagnosed postnatally, on day 1 of life, and was more common in term infants. Interestingly, one quarter was born with intrauterine growth restriction. Mortality rate in this cohort was high, but none due to delayed transfer issues, rather majority was due to withdrawal of care, which raised complex ethical issues.

Keywords: GIT malformations, nonsurgical NICU

 

MissingLinkText

Die Autoren geben an, dass kein Interessenkonflikt besteht.