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Abstract：
BACKGROUND: Following the publication of the Management of Myelomeningocele study (MOMS), fetal repair of myelomeningocele (MMC) has become increasingly prevalent worldwide. However, limited case presentations exist illustrating the potential mechanical and embryological effects of fetal repair. We present a unique case report of a complex embryological cervicomedullary junction (CMJ) malformation and cerebellar hypoplasia following fetal repair of MMC.
METHODS: A 1-day-old female was referred to the paediatric neurosurgical team after having successful surgical intrauterine closure of MMC abroad at 25 weeks gestation. The patient was born by emergency caesarean section at 33 weeks gestation and had a ventricular-peritoneal shunt inserted at 25 days old due to resulting hydrocephalus. Neonatal MRI scans revealed a complex number of malformations that included a split cord located at the CMJ, hypoplasia of the cerebellum and vermis, and a Chiari type II malformation.
CONCLUSIONS: It is possible that the clefting of the upper cervical spinal cord was undetected at preoperative MRI; however, this is unlikely given the antenatal images. It is our hypothesis that the malformation may have exhibited mechanical change after the repair, as the preoperative MRI showed only a Chiari II malformation without any of the complex abnormalities being present and the split cord was already there but not obvious. There are no existing reports of such a complex malformation following antenatal surgery in the literature. This should be further explored as more cases and trials become available.
METHODS: A 1-day-old female was referred to the paediatric neurosurgical team after having successful surgical intrauterine closure of MMC abroad at 25 weeks gestation. The patient was born by emergency caesarean section at 33 weeks gestation and had a ventricular-peritoneal shunt inserted at 25 days old due to resulting hydrocephalus. Neonatal MRI scans revealed a complex number of malformations that included a split cord located at the CMJ, hypoplasia of the cerebellum and vermis, and a Chiari type II malformation.
CONCLUSIONS: It is possible that the clefting of the upper cervical spinal cord was undetected at preoperative MRI; however, this is unlikely given the antenatal images. It is our hypothesis that the malformation may have exhibited mechanical change after the repair, as the preoperative MRI showed only a Chiari II malformation without any of the complex abnormalities being present and the split cord was already there but not obvious. There are no existing reports of such a complex malformation following antenatal surgery in the literature. This should be further explored as more cases and trials become available.
摘要：
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