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Hemimyeloschisis associated with hydrocephalus
1Department of Neurosurgery, Gulhane Military Medical Academy, Ankara, Turkey. yusufizci@yahoo.com.
This study reports a rare case of hemimyeloschisis in a neonate with hydrocephalus. Hemimyeloschisis is a very rare spinal malformation where one side of the spinal cord is exposed. The patient had visible skin defects and hypertrichosis at birth. Computed tomography was used to diagnose the condition and revealed a bony septum at the L2 level. The patient also had mild hydrocephalus. Surgery was performed to close the myeloschisis and remove the septum, along with a ventriculoperitoneal shunt for hydrocephalus. The authors suggest that this case supports the hypothesis of an ontogenic basis of development and emphasizes the importance of early imaging in diagnosing this complex condition.
Area of Science:
- Neurodevelopmental anomalies in pediatric neurology
- Congenital spinal malformations in orthopedic surgery
- Hydrocephalus management in neonatal neurosurgery
Background:
Congenital spinal malformations remain poorly understood in many cases. Prior research has shown that split cord malformations occur in association with other spinal defects. However, hemimyeloschisis remains exceptionally rare. No prior work had resolved the diagnostic challenges of hemimyeloschisis combined with skin defects. Established knowledge includes the role of imaging in diagnosing spinal anomalies. This gap motivated a closer look at the diagnostic and surgical implications of this specific malformation. The relationship between hemimyeloschisis and hydrocephalus is not well established. This paper contributes new insights into the diagnostic and treatment approaches for this rare condition.
Purpose Of The Study:
The aim of this study was to describe a rare case of hemimyeloschisis in a neonate with hydrocephalus. The specific problem involved diagnosing and managing a complex spinal malformation. The motivation stemmed from the limited literature on this condition. The authors sought to highlight the importance of early imaging in such cases. They also aimed to document the surgical approach used for this malformation. No prior work had combined the treatment of hemimyeloschisis with hydrocephalus in a single report. This study provides a case example to guide future clinical decisions. The findings may suggest a broader diagnostic strategy for similar conditions.
Main Methods:
The study involved a case report of a neonate with hemimyeloschisis and hydrocephalus. Computed tomography was used to identify the bony septum and spinal malformation. The patient presented with visible skin defects and hypertrichosis at birth. A cranial CT scan was performed to assess the extent of hydrocephalus. Surgical intervention included closure of the myeloschisis and removal of the septum. A ventriculoperitoneal shunt was inserted to manage the hydrocephalus. The surgical approach was designed to prevent infection and neurological deterioration. The case was analyzed to evaluate the diagnostic and treatment strategies used.
Main Results:
Computed tomography revealed a bony septum at the L2 level and hemimyeloschisis on the right hemicord. The neonate also exhibited mild hydrocephalus in the cranial CT scan. Skin defects and hypertrichosis were observed in the upper lumbar region. Surgical closure of the myeloschisis was successfully performed in one stage. The removal of the septum was completed alongside skin closure. Ventriculoperitoneal shunt insertion was conducted for the hydrocephalus. The combined malformation was consistent with the hypothesis of an ontogenic basis of development. The case highlights the value of early imaging in diagnosing this complex condition.
Conclusions:
The combination of hemimyeloschisis and myeloschisis supports the hypothesis of an ontogenic basis of development. Early imaging is emphasized as a critical step in diagnosing this complex malformation. The authors propose that surgical closure should be performed to prevent infection and deterioration. The presence of hydrocephalus adds to the complexity of the condition. The case demonstrates the importance of a multidisciplinary approach in management. The findings may suggest a broader diagnostic and treatment strategy for similar cases. The authors highlight the need for further case reports to expand understanding. This case contributes to the limited literature on this rare spinal malformation.
Frequently Asked Questions
The authors propose that the combination of hemimyeloschisis and hydrocephalus suggests an ontogenic basis of development.
Computed tomography revealed a bony septum at the L2 level and hemimyeloschisis on the right hemicord.
Early imaging is critical for precise diagnosis and to guide surgical intervention in this complex malformation.
The shunt was inserted to manage the mild hydrocephalus diagnosed in the cranial CT scan.
Surgical closure of the myeloschisis and removal of the septum were successfully performed in one stage.
The authors suggest that this case supports the need for early diagnosis and multidisciplinary management in similar cases.

