SNAPPE-II and MDAS scores as predictors for surgical intervention in very low birth weight neonates with necrotizing enterocolitis.

BACKGROUND: Necrotizing enterocolitis (NEC) is the most common life-threatening gastrointestinal emergency in preterm and term neonates, with the majority of cases affecting neonates classified as very low birth weight (VLBW, bw <1500 g). Scores for neonatal acute physiology-perinatal extension-II (SNAPPE-II) and metabolic derangement acuity score (MDAS) have been developed and utilized to assess neonatal morbidity and mortality including the subset of VLBW neonates. Serial SNAPPE-II and MDAS scores have been reported in neonates with necrotizing enterocolitis to assist in surgical management, yielding mixed results. OBJECTIVE: To determine the relationship between clinical and/or laboratory deterioration using SNAPPE-II and MDAS scores measured at the time of NEC diagnosis and surgical management of NEC. METHODS: We retrospectively evaluated preterm neonates ≥23 weeks gestational age who developed pneumatosis intestinalis on radiographic imaging coupled with clinical signs of NEC. SNAPPE-II and MDAS scores were calculated within twelve hours of birth and within twelve hours of initial finding of pneumatosis intestinalis. Baseline characteristics and clinical variables between those who did and did not require surgical intervention were compared. Logistic regression and receiver - operator characteristics (ROC) curve analyses were also performed, and areas under the curve (AUC) computed, to assess the performance of SNAPPE-II and MDAS scoring systems to differentiate neonates with NEC in the two groups. RESULTS: Sixty-four neonates were evaluated in our study of which 20 required surgical management of NEC. While the baseline SNAPPE-II and MDAS scores did not differ between the surgical management and medical management only groups, when rescored within 12 h of NEC diagnosis, the surgical management group had significantly higher SNAPPE -II (38 (18.5-69) vs. 19 (10-34.5), p = .04) and MDAS (2.5 (1-3) vs. 1 (0-2), p = .0004) scores. The AUCs for MDAS 0.77 (95% CI 0.65-0.89 and 0.71 (95% CI 0.57-0.85) for SNAPPE-II, indicating an acceptable level of diagnostic ability of both scoring systems to differentiate between those who did and did not need surgical management. CONCLUSION: SNAPPE II and MDAS scores performed within 12 h of NEC diagnosis may be useful in predicting which preterm VLBW neonates will require surgical intervention.

A Rare Case of Lethal Neonatal Rigidity and Multi-Focal Seizure Syndrome.

We present a case of lethal neonatal rigidity and multifocal seizure syndrome (RMFSL) in an early-term female infant born to non-consanguineous parents. RMFSL is a recently discovered autosomal recessive disease caused by the BRAT1 gene mutations. The BRAT1 gene encodes the BRCA1-associated protein required for ATM activation-1, a protein that interacts with BRCA1 and ATM to initiate DNA repair in response to DNA damage. The exon sequence revealed biallelic deletions of exon 1-2 of the BRAT1 gene in our patient. There are only a few cases of RMFSL reported in the literature, and all of them have died before two years, mostly in the first six months of life. Our patient died at the age of 74 days.