Postnatal Intracranial Findings Following Fetal Repair of Spinal Dysraphisms

Abstract

Objective Our objective is to document the imaging appearance in the intracranial compartment at the time of the infants' first postnatal brain MR imaging after fetal repair for spinal dysraphisms. Methods Twenty-nine patients were evaluated on fetal and postnatal magnetic resonance imaging for a series of features of Chiari II malformation. Results Of the 29 infants, 55% had resolution of tonsillar ectopia, and 62% showed a dorsal outpouching of the near the foramen magnum on postnatal magnetic resonance imaging. The majority had persistence of Chiari II features including: prominent massa intermedia (93%), tectal beaking (93%), towering cerebellum (55%), flattening of the fourth ventricle (90%), hypoplastic tentorium (97%), and tonsillar hypoplasia (59%). Conclusions Normally positioned or minimally descended, oftentimes hypoplastic tonsils in the presence of a posterior fossa configuration typical of Chiari II, was the most common presentation. An additional documented feature was an outpouching of the dorsal thecal sac between the opisthion and the posterior arch of C1.

What brain abnormalities can magnetic resonance imaging detect in foetal and early neonatal spina bifida: a systematic review

“…These were found to be associated with malformations of cortical development which may present with symptoms such as epilepsy, developmental delay, and focal neurological signs [ 38 ]. Other CIIM findings included flattening of the fourth ventricle and tectal beaking or hypoplastic tentorium [ 3 , 38 ]. This may also be important in terms of prognostication as it gives an indication as to the severity of the condition.…”

“…Open spina bifida (OSB) is a non-lethal condition with complex physical and neurodevelopmental sequalae [1][2][3][4][5][6]. It comprises brain abnormalities which include the Chiari II malformation (CIIM), a constellation of anomalies, principally, but not exclusively associated with the brainstem and characterised by hindbrain herniation in which the medulla, fourth ventricle, and cerebellum are displaced into the spinal canal.…”

Interpeduncular angle: A new parameter for assessing intracranial hypotension in fetuses with spinal dysraphism

“…Fetal MRI can also rule out the possibility of intrauterine surgery in fetuses with open SD when additional congenital or acquired anomalies are detected 7 . This technique can also be used to evaluate the evolution of the cerebral abnormalities of the Chiari II malformation in fetuses undergoing surgery 7,11,21 . In this regard, the IPA can potentially be used to assess the evolution of the amount of CSF volume after intrauterine repair of the spinal defect and, therefore, the effect of stopping the leakage of CSF.…”

“…10 The main indication for performing MRI in fetuses with open SD is currently a thorough evaluation of potential candidates for intrauterine neurosurgery repair. 7,11,21 Fetal MRI has proven to be superior to ultrasound in establishing abnormalities of the posterior fossa, brainstem, corpus callosum, and cortical development, as well as in the prenatal investigation of acquired brain lesions. [8][9][10]14 Fetal MRI can also rule out the possibility of intrauterine surgery in fetuses with open SD when additional congenital or acquired anomalies are detected.…”

“…7 This technique can also be used to evaluate the evolution of the cerebral abnormalities of the Chiari II malformation in fetuses undergoing surgery. 7,11,21 In this regard, the IPA can potentially be used to assess the evolution of the amount of CSF volume after intrauterine repair of the spinal defect and, therefore, the effect of stopping the leakage of CSF. If the intracranial pressure increases after the intrauterine intervention, the IPA also increases, as shown in Figure 3.…”