Clival fractures in children: a challenge in the trauma room setting!

OBJECTIVE: A clival fracture is a rare but life-threatening traumatic brain injury in the adult and pediatric populations. To date, there are very few conclusive recommendations in the literature concerning the diagnosis and treatment of pediatric clival fractures. METHODS: In 2014 and 2015, two pediatric patients with severe blunt head trauma and clival fractures were evaluated and treated at a level I trauma center. Both cases are documented and supplemented by an extensive review of the literature focusing on the diagnostic workup, classification, and clinical course of clival fractures in children. RESULTS: The clinical course of two children (8 and 9 years old) with clival fractures in concert with other intra- and extracranial injuries was analyzed. A total of 17 papers encompassing 37 patients (age range, 1-18 years) were included for a systematic review. The literature review revealed a mortality rate of 23% in pediatric patients with a clival fracture. Over 50% of the patients presented with cranial nerve damage, and two-thirds suffered from intracranial vascular damage or intracerebral bleeding. CONCLUSIONS: Clival fractures are a very rare but severe consequence of blunt head trauma in the pediatric population and may be challenging to diagnose, especially in cases with an unfused sphenooccipital synchondrosis. Vascular damage following clival fractures appears to be as common in pediatric patients as in adults. Therefore, contrast-enhanced CT of the cervical spine and head and/or magnetic resonance angiography is strongly recommended to rule out vascular injury of the extra- and intracranial brain-supplying vessels within the trauma room setting.

Postoperative brain volumes are associated with one-year neurodevelopmental outcome in children with severe congenital heart disease.

Children with congenital heart disease (CHD) remain at risk for neurodevelopmental impairment despite improved perioperative care. Our prospective cohort study aimed to determine the relationship between perioperative brain volumes and neurodevelopmental outcome in neonates with severe CHD. Pre- and postoperative cerebral MRI was acquired in term born neonates with CHD undergoing neonatal cardiopulmonary bypass surgery. Brain volumes were measured using an atlas prior-based automated method. One-year neurodevelopmental outcome was assessed with the Bayley-III. CHD infants (n = 77) had lower pre- and postoperative total and regional brain volumes compared to controls (n = 44, all p < 0.01). CHD infants had poorer cognitive and motor outcome (p ≤ 0.0001) and a trend towards lower language composite score compared to controls (p = 0.06). Larger total and selected regional postoperative brain volumes were found to be associated with better cognitive and language outcomes (all p < 0.04) at one year. This association was independent of length of intensive care unit stay for total, cortical, temporal, frontal and cerebellar volumes. Therefore, reduced cerebral volume in CHD neonates undergoing bypass surgery may serve as a biomarker for impaired outcome.

Left temporal plane growth predicts language development in newborns with congenital heart disease.

Congenital heart defects are the most common congenital anomalies, accounting for a third of all congenital anomaly cases. While surgical correction dramatically improved survival rates, the lag behind normal neurodevelopment appears to persist. Deficits in higher cognitive functions are particularly common, including developmental delay in communication and oral-motor apraxia. It remains unclear whether the varying degree of cognitive developmental delay is reflected in variability in brain growth patterns. To answer this question, we aimed to investigate whether the rate of regional brain growth is correlated with later life neurodevelopment. Forty-four newborns were included in our study, of whom 33 were diagnosed with dextro-transposition of the great arteries and 11 with other forms of severe congenital heart defects. During the first month of life, neonates underwent corrective or palliative cardiovascular bypass surgery, pre- and postoperative cerebral MRI were performed 18.7 ± 7.03 days apart. MRI was performed in natural sleep on a 3.0 T scanner using an 8-channel head coil, fast spin-echo T2-weighted anatomical sequences were acquired in three planes. Based on the principles of deformation-based morphometry, we calculated brain growth rate maps reflecting average daily growth occurring between pre- and postoperative brain images. An explorative, whole-brain, threshold-free cluster enhancement analysis revealed strong correlation between the growth rate of the Heschl's gyrus, anterior planum temporale and language score at 12 months of age, corrected for demographic variables (P = 0.018, t = 5.656). No significant correlation was found between brain growth rates and motor or cognitive scores. Post hoc analysis showed that the length of hospitalization interacted with this correlation, longer hospitalization resulted in faster enlargement of the internal CSF spaces. Our longitudinal cohort study provides evidence for the early importance of left-dominant perisylvian regions in auditory and language development before direct postnatal exposure to native language. In congenital heart disease patients, the perioperative period results in a critical variability of brain growth rate in this region, which is a reliable neural correlate of language development at 1 year of age.