Spinal Dysraphism- A Histopathological Study of 45 Cases

Introduction: Dysraphic conditions of spine resulting from non closure of the neural groove, consists of different types of malformations and they are called spina bifida, which have been classified into several types. Myelomeningocele (MMC) and Meningocele (MC) come under spina bifida cystica. Aim: To review the clinical and pathological findings of congenital spinal dysraphism and discuss the pathological diagnosis Materials and Methods: A descriptive cross-sectional type of study was conducted in 45 cases of spinal dysraphism during the period from October 2012 to February 2016, in the Department of Pathology of a tertiary care hospital, Hyderabad, India. Cases which were diagnosed as spinal dysraphism by clinical and radiological examination, tissue specimens were sent to the department of pathology along with clinical and radiological findings. Haematoxylin and Eosin (H&E) staining was done in all cases, followed by detailed microscopic study of slides. Histopathological findings such as epithelial, mesodermal, neuroectodermal changes were elucidated in detail and findings were compared with literature. Results: In a total of 45 cases there were 30 cases of MMC, nine cases of MC and six cases of encephalocele. Loss of epidermal appendages were seen in 91% of cases and neuropil like matrix was present in 76% cases. Conclusion: The embryogenesis of spina bifida involves ectoderm, neuroectoderm and mesoderm. A detailed definition of histopathological aspects will help in understanding these anomalies and should be a part of a detailed histopathology report.

Urinary incontinence in a young British blue cat with suspected dilatation of the ventricle terminalis, spina bifida cystica and failure of fusion of the sacral vertebrae

A seven-month-old, male neutered British blue cat presented for investigation of lifelong urinary incontinence. Clinical examination documented a flaccid tail which was deep pain-negative, and the urinary bladder was easily expressed. Voluntary urination was possible. An abnormal hair whorl was noted over the tail base. Pelvic radiography noted absent dorsal lamina to the caudal sacral and proximal caudal vertebrae. The cranial S1/S2 vertebrae were incompletely fused. Haematology, biochemistry and urinalysis were unremarkable. Ultrasonographic examination of the urinary tract was unremarkable, as was an intravenous urogram. MRI documented a large, fluid-filled cavity at the level of the conus medullaris compatible with dilatation of the ventricle terminalis or syrinx. Spina bifida cystica was also present at Cd5/Cd6. The incontinence was noted to improve with time and skeletal maturity before acutely exacerbating. The owners subsequently elected for tail amputation, resulting in a marked improvement in urinary incontinence.

Neural Tube Defects

Neural tube defects (NTDs) are congenital abnormalities that arise from the neural tube failing to close. These defects can affect the brain, spine, or spinal cord and generally happen within the first month of pregnancy. Cranial dysraphism is failure of cranial neural tube closure and includes anencephaly and encephalocele. Spinal dysraphism is failure of caudal neuropore closure and includes spina bifida cystica and occulta. Myelomeningocele is a type of spina bifida cystica where the membranous sac containing neural tissue protrudes through an opening in the back. It is the most common NTD and considered a surgical emergency due to the increased risk for infection, further neurologic damage, and dehydration. Advancements in medicine have allowed for the possibility of early diagnosis and even in utero surgical intervention. This chapter focuses more exclusively on the approach and management of myelomeningocele.