Objective: Cerebellar injury is increasingly recognized as a prematurity-related brain lesion. Whereas the morphological characteristics have already been described, preterm birth and its risk factors for the cerebellar development remain to be evaluated.

The unfavorable impact of prematurity on the developing cerebellum was recently recognized, but the outcome after impaired cerebellar development as a prematurity-related complication is hitherto not adequately documented. Therefore we compared 31 preterm patients with disrupted cerebellar development to a control group of 31 gender and gestational age matched premature infants with normal cerebellar development. Supratentorial brain injuries during the neonatal period were comparable between the groups. At a minimum age of 24 months motor and mental development was assessed by standardized tests. Disrupted cerebellar development was associated with significantly poorer scores both in the subtests for neuromotor (p < 0.001) and mental development (p < 0.001), respectively. Mixed CP was diagnosed in 48% of affected patients, whereas none of the patients of the control group had mixed CP. Microcephaly and epilepsy were significantly related to disrupted cerebellar development. Preterm patients with disrupted cerebellar development exhibit poorer outcome results in all investigated variables. The role of the cerebellum in neurodevelopment after prematurity seems to be underestimated so far.

Fragestellung: Very low birth weight infants haben ein hohes Risiko für das Auftreten von zerebralen Läsionen. Während supratentorielle Läsionen häufig gefunden werden, sind Pathologien der hinteren Schädelgrube selten. Eine Serie von 28 frühgeborenen Kindern unter der 30. Schwangerschaftswoche mit auffälliger zerebellärer Entwicklung wurde retrospektiv untersucht, um typische Muster zu definieren, und Rückschlüsse auf mögliche Risikofaktoren zu ziehen.

BACKGROUND AND PURPOSEInfants with very low birth weight are at high risk for cerebral lesions. Although supratentorial brain damage is a common radiologic finding, posterior fossa pathologies are rare. We studied the morphology of cerebellar involvement in a large series of 28 premature infants born before the 30th week of gestation to define typical patterns and identify possible risk factors for this pathology.METHODSCranial sonograms were obtained in the early neonatal period. MR imaging was performed between the 2nd month and the 6th year of life. Morphologic patterns of cerebellar involvement were evaluated.RESULTSThree morphologic patterns of cerebellar involvement were recognized: (1) symmetric volume reduction of the cerebellar hemispheres, which were floating immediately beneath the tentorium, and a small vermis with preserved shape; (2) symmetrical reduction in hemispheric volume with an enlarged, balloon-shaped fourth ventricle and a small, deformed vermis; and (3) normal overall cerebellar shape with extensive reduction of its dimensions. A small brain stem with flattened anterior curvature of the pons and loss of supratentorial white matter was present in all patients.CONCLUSIONSymmetric cerebellar volume reduction was found as a consequence of extreme prematurity. Selective vulnerability of the developing cerebellum in the window of 24-30 weeks of gestation, combined with several additive perinatal risk factors (eg, hemosiderin deposits) seems to lead to destruction of immature structures and developmental arrest. Therefore, the resulting condition is the consequence of disrupted cerebellar development.

Objective: Supratentorial lesions are well known risks of extreme prematurity, while lesions of the cerebellum are rarely described. We report the delayed vanishing of the cerebellum in 21 of our patients with a birthweight less than 1500g.

Phrenic nerve lesions as a result of birth trauma have been reported as a cause of acute respiratory distress infrequently. We report recent diagnostic and therapeutic experiences in four newborns with birth-traumatic phrenic nerve injury: one bilaterally, and three unilaterally, all right-sided. In each case, mechanical ventilation was required for at least 16 days. Ultrasound examination of the diaphragm and phrenic nerve conduction studies turned out to be the diagnostic methods of choice. Spontaneous recovery occurred in two children and two became asymptomatic after operative treatment. One improved after plication of diaphragm and one after autologous nerve transplantation.

Im Rahmen histologischer Untersuchungen wurde in 7 von 15 Fällen von SIDS eine massive fettige Metamorphose der Gliazellen des periventrikulären frontalen Marklagers vorgefunden. Eine Kontrollgruppe von 6 Kleinkindern (Alter unter 1 Jahr), die infolge eines anderen natürlichen oder gewaltsamen Todes gestorben waren, wies in keinem Fall diese morphologischen Veränderungen auf. Dünnschichtchromatographische Analysen der beim SIDS in den Gliazellen gespeicherten Lipide ergab einen hohen Anteil von Cholesterinestern, während jener in der Kontrollgruppe stets nieder war. Die fettige Gliametamorphose wird als morphologisches Substrat einer geringgradigen Schädigung unterschiedlicher Ätiologie der metabolisch besonders aktiven, noch unreifen Gliazellen der periventrikulären Marksubstanz in Erwägung gezogen.

In 7 of 15 cases of sudden infant death syndrome (SIDS), distinct periventricular fatty metamorphosis of the neuroglia could be demonstrated by a histological staining technique. None of the six children (under 1 year of age) used as a control group whose cause of death was not SIDS showed this morphological change. Thin-layer chromatographic analysis of the lipids accumulated in the glial cells in SIDS established a high amount of esterified cholesterol whereas the content of esterified cholesterol in the control group was low. We presume that fatty metamorphosis of the neuroglia represents the morphological substrate of a metabolic disorder of the sensitive, immature glia of the periventricular white cerebral matter caused by various kinds of damage.