Spontaneous closure of myelomeningocele.

Fetal repair of myelomeningocele has been increasingly offered to mothers of children with myelomeningocele after the seminal Management of Myelomeningocele (MOMs) trial, which demonstrated decreased reliance on ventriculoperitoneal shunt following fetal closure. We present the case of a fetus diagnosed with a lumbar myelomeningocele in utero whose mother refused in utero closure and who was subsequently born with a skin-covered defect. A fetal MRI was obtained on a mother with a male fetus diagnosed with open neural tube defect at 20 weeks of gestation. The child demonstrated spinal dysraphism extending from L2 to L5 and associated Chiari II malformation with lateral and third ventriculomegaly. Based on our institutional criteria and the criteria of the MOMs trial, the parents were offered fetal repair of the myelomeningocele; however, they declined because of concerns about risks to the mother. At birth, the patient was found to have a skin-covered meningocele. He underwent elective repair of his occult meningocele and detethering of his spinal cord. Intraoperative findings demonstrated spinal nerve roots attached to the arachnoid within the defect, and a closed, tubularized neural placode. This represents a unique case in which a fetus with a clinical picture consistent with open spinal defect was found to have a lesion more consistent with meningocele on postnatal operative interrogation. Knowledge that this can occur should be taken into consideration when discussing fetal closure, although the frequency of this occurrence is not known. Additionally, identification of this case sheds light on the mechanism by which occult myelomeningoceles form.

Association of initial assessment variables and mortality in severe pediatric traumatic brain injury.

Background: Predictive scales have been used to prognosticate long-term outcomes of traumatic brain injury (TBI), but gaps remain in predicting mortality using initial trauma resuscitation data. We sought to evaluate the association of clinical variables collected during the initial resuscitation of intubated pediatric severe patients with TBI with in-hospital mortality. Methods: Intubated pediatric trauma patients <18 years with severe TBI (Glasgow coma scale (GCS) score ≤8) from January 2011 to December 2020 were included. Associations between initial trauma resuscitation variables (temperature, pulse, mean arterial blood pressure, GCS score, hemoglobin, international normalized ratio (INR), platelet count, oxygen saturation, end tidal carbon dioxide, blood glucose and pupillary response) and mortality were evaluated with multivariable logistic regression. Results: Among 314 patients, median age was 5.5 years (interquartile range (IQR): 2.2-12.8), GCS score was 3 (IQR: 3-6), Head Abbreviated Injury Score (hAIS) was 4 (IQR: 3-5), and most had a severe (25-49) Injury Severity Score (ISS) (48.7%, 153/314). Overall mortality was 26.8%. GCS score, hAIS, ISS, INR, platelet count, and blood glucose were associated with in-hospital mortality (all p<0.05). As age and GCS score increased, the odds of mortality decreased. Each 1-point increase in GCS score was associated with a 35% decrease in odds of mortality. As hAIS, INR, and blood glucose increased, the odds of mortality increased. With each 1.0 unit increase in INR, the odds of mortality increased by 1427%. Conclusions: Pediatric patients with severe TBI are at substantial risk for in-hospital mortality. Studies are needed to examine whether earlier interventions targeting specific parameters of INR and blood glucose impact mortality.