Figure - available from: Pediatric Radiology
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Three types of cervicothoracic cystic dysraphism. Meningocele with stalk (left) is the most common with expansion of the cerebrospinal fluid space to form a meningocele and a linear stalk that arises from the dorsal spinal cord and attaches to the posterior wall of the meningocele. Myelocystocele (middle) appears as a “cyst within a cyst.” The dilated central canal herniates into a meningocele, but the two cystic structures do not communicate. Finally, least common is the meningocele (right), which consists of simple expansion of the cerebrospinal fluid space
Source publication
Cystic dysraphism of the cervical and upper thoracic spine is very rare. It differs from the much more common lumbosacral dysraphism in appearance and structure, and usually portends a better prognosis due to lack of functional neurological tissue in the dysraphic sac and absent or less severe intracranial anomalies. There is ambiguity in the liter...
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Case summary
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Introduction and importance:
Benign cystic teratoma considered an extragonadal germ cell tumor that can present at any age and mostly located in the anterior mediastinum, only 3 %-8 % are in the posterior mediastinum. Meningomyelocele is an abnormal herniation of the meninges that located in most cases posteriorly in lumbosacral spine. Cervicothor...
IntroductionAdults rarely present with tethered cord syndrome, and this review examines whether it is justifiable to perform surgical intervention in this group.Methods
Between 2003 and 2017, we performed surgical intervention in 32 adults with tethered cord syndrome. The age range varied from 22 to 65 years. Twenty-six had pain, 20 had motor defic...
Objective: The study's goal is to find out how often individuals with myelomeningocele and TCS repair have leakage of cerebrospinal fluid. Study Design: Cross-sectional Place and Duration: Women medical college Abbottabad and Children hospital and the institute of child health, Multan, during from the period July, 2021 to Dec, 2021. Methods: There...
A mature cystic teratoma is a mass with heterogeneous appearance, consisting of adult tissue with two or three layers: endoderm, mesoderm, and ectoderm. It is a rare, benign transformation of somatic tissue most commonly found in the sacrococcygeal region and may resemble an uncomplicated spina bifida on prenatal ultrasonography. In this case repor...
Citations
... Valeur et al. reported on the extreme rarity of cystic dysraphism of the cervical and upper thoracic spine and the better prognosis versus lumbosacral dysraphism given the lack of functional neurological tissue in the dysraphic sac [13]. Classification and nomenclature of cervical myelomeningoceles (cMMC) have evolved through the literature [14][15][16][17][18][19] ( Table 1). ...
Neural tube defects are a group of birth defects that affect the development of the spinal cord and brain. Myelomeningocele is a type of neural tube defect that results in the protrusion of the spinal cord and meninges through a defect in the vertebral column. While myelomeningocele is a relatively rare condition, cervical myelomeningocele is extremely uncommon. The condition can lead to various neurological problems and atrophies and is typically diagnosed in the first trimester of pregnancy using an ultrasound examination. Surgical intervention is typically recommended to repair the affected vertebral column. In this report, we describe the case of a four-month-old baby boy who was diagnosed with a cervical myelocystocele and successfully treated surgically. The patient had an excellent postoperative status, and this case highlights the importance of early diagnosis and intervention in the management of this rare condition.
Background
Cervicothoracic myelomeningoceles (MMC) are rare entities with only two reported cases in literature of anterior thoracic MMC.
CaseDescription
We report a third case, a three-year-old male child, diagnosed during antenatal screening that warranted surgical intervention. Despite being asymptomatic, radiological surveillance demonstrated worsening syringomyelia, tonsillar descent and cord signal change concerning for myelomalacia. Pre-operative management involved respiratory assessment for pulmonary compromise, general paediatric consultation, gait analysis by physiotherapy and serial imaging by radiology. Surgical management involved an anterior thoracotomy approach by cardiothoracic surgeons, repair of the MMC by neurosurgeons and bone graft to ameliorate the bony defect in the vertebral body by orthopaedic surgeons. Post-operative care involved four days in the intensive care unit and fourteen days on the paediatric neurosurgical ward. Three-year follow-up demonstrated radiological improvement of the syringomyelia, tonsillar descent and kyphoscoliosis without any neurological or pulmonary complications.
Conclusion
This paper highlights the unique multidisciplinary surgical management of the rare entity of anterior thoracic MMC without scoliosis correction for radiological progression.
Background: Lumbosacral spinal lipomas and lipomyeloceles are usually identified in early childhood. Terminology, histopathology, and diagnosis for these malformations can be confusing. Materials and Methods: This is a PubMed review with comparison of embryology, gross, and histopathology, and reporting requisites for these and related closed spinal malformations. Results: The spinal lipoma group (congenital spinal lipomatous malformations) includes subcutaneous, transdural, intradural, and noncontiguous malformations stretching through the entire lower spinal region. This lipomyelocele trajectory overlaps the embryonic tail’s caudal eminence. Histopathologically, the lipomyelocele spectrum is a heterogeneous, stereotypical set of findings encountered from dermis to spinal cord. Diagnosis requires detailed correlation of images, intraoperative inspection, and histopathology. Conclusions: Appropriate terminology and clinicopathologic correlation to arrive at a diagnosis is a critical activity shared by pathologist and clinician. Prognostic and management differences depend on specific diagnoses. Familial and genetic influences play little if any role in patient management in closed spinal malformations.
