Early Neurologic Complications and Long-term Neurologic Outcomes of Extracorporeal Membrane Oxygenation Performed in Children.

BACKGROUND: We aimed at evaluating acute neurologic complications (ANC) and clinical outcome at a 2-year follow-up in children after extracorporeal membrane oxygenation (ECMO). METHODS: We conducted a single-center, retrospective review of our patient cohort aged between 1 month and 18 years at the time of ECMO support (between June 2014 to January 2017). Outcome analysis included ANC and their clinical consequences.The Pediatric Overall Performance Category (POPC) and Pediatric Cerebral Performance Category (PCPC) were used for neurologic assessment performed at discharge from the hospital and at 2nd year follow-up. RESULTS: There were 35 children who required ECMO. The median ECMO time was 9 days (range 2-32 days). Decannulation from ECMO was achieved in 68.6% of patients, and overall, 42.8% survived (15 patients), The incidence of ANC in the surviving patients was 40% (6 children). ANC were intracranial hemorrhage, seizures, cerebral infarction, which occurred in one, two and three of the 15 surviving patients respectively (6.6, 13.3 and 20%). A higher rate of organ failure was related to death (p=0.043), whereas duration on ECMO was a risk factor for the development of ANC (p<0.05). At hospital discharge, the 14 patients evaluated had normal development or -mild disability in 73.2%, and at the 2-year follow-up, 93.4% had these scores. CONCLUSION: Children who receive ECMO have a risk to develop ANC, which was related to the length of ECMO treatment, while survival was related to less organ failure, Long-term neurological outcome was good in our patient cohort.

Genoa syndrome and central diabetes insipidus: a case report.

Genoa syndrome was first described by Camera et al in 1993 in two patients with semilobar holoprosencephaly (HPE), craniosynostosis and abnormal small hands with cone-shaped epiphyses and hypoplastic terminal phalanges of fingers (OMIM: 601370). In 2001, Lapunzina et al reported a case of craniosynostosis and HPE associated with several other malformations and suggested that these findings could be attributed to a severe form of Genoa syndrome or to a newly recognized syndrome. Endocrinopathies in association with HPE are frequently reported in the literature. Diabetes insipidus, hypothyroidism, hypocortisolism, and growth hormone deficiency are frequently associated with HPE. We here report a case of semilobar HPE, craniosynostosis and cleft lip/palate, possibly a case of Genoa syndrome, associated with central diabetes insipidus.