Long-term neurodevelopmental outcome and serial cerebral magnetic resonance imaging assessment in Fontan patients at school age.

OBJECTIVES: Children with univentricular congenital heart disease undergoing staged surgical palliation are at risk for impaired neurodevelopmental (ND) outcome. Little is known about the long-term effects on brain growth until school age. METHODS: In a prospective two-centre study, consecutive patients undergoing stage I (Hybrid or Norwood) to stage III (Fontan procedure) were evaluated by 2 serial cerebral magnetic resonance imaging examinations, somatic growth and ND testing before Fontan procedure at 2 years of age (Bayley-III) and after Fontan at 6-8 years of age (Wechsler Intelligence Scale for Children-third edition). Magnetic resonance imaging findings were compared with 8 healthy controls. Medical and sociodemographic characteristics were documented and related to cerebral and ND findings. RESULTS: We examined 33 children (16 female) at a mean age of 2.3 (0.35) and 6.8 (± 0.7) years. The mean Bayley-III cognitive scales were 99.1 (9.9), language scales 98.4 (11.9) and motor scales 98.5 (13.8) at the first examination. Follow-up at school age showed a mean total IQ of 86.7 (13.6). The rate of structural brain lesions increased from 39% at 2 years to 58% at school age. Bayley-III language scale (P = 0.021) and mean Wechsler Intelligence Scale for Children-third edition (P = 0.019) were lower in children with pathological MR findings. Total brain volume (P < 0.001), total grey matter volume (P = 0.002), deep grey matter volume (P = 0.001) and white matter volume (P < 0.001) were smaller in patients compared to age- and gender-matched healthy controls. CONCLUSIONS: Smaller brain volumes and structural brain lesions in complex congenital heart defect patients at school age are associated with impaired ND outcome. For the evaluation of predictive surgical or clinical factors, larger multicentre studies are needed.

Extensive Microhemorrhages of the Cerebellar Peduncles After High-Altitude Cerebral Edema.

Pichler Hefti, Jacqueline, Philipp Hoigné-Perret, and Raimund Kottke. Extensive microhemorrhages of the cerebellar peduncles after high-altitude cerebral edema. High Alt Med Biol. 18:182-184, 2017.-Neuromagnetic resonance imaging (MRI) of subjects who suffered from high-altitude cerebral edema (HACE) typically shows cerebral microhemorrhages (MH) of the corpus callosum, in particular the splenium, and supratentorial white matter. This is a case report of a 43-year-old male, who suffered from unusually prolonged severe ataxia and amnesia after having been rescued during the ascent to Mount Everest at 6400 m. MRI of the brain 63 days after the incident showed the typical MH in the corpus callosum, but, in addition, extensive MH were found in the middle cerebellar peduncles. These infratentorial MH might reflect the pronounced atactic gait disorder. This case describes the first HACE-associated MH in the cerebellar peduncles in a high-altitude mountaineer indicating a potential vulnerability of infratentorial brain areas to hypobaric hypoxia.