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|Year : 2018 | Volume
| Issue : 3 | Page : 879-881
Fetal meningocele manqué
MH Shabina Banu1, Rajeswaran Rangasami1, Indrani Suresh2
1 Department of Radiology, Sri Ramachandra University, Chennai, Tamil Nadu, India
2 Department of Radiology, Mediscan Systems, Chennai, Tamil Nadu, India
|Date of Web Publication||15-May-2018|
Dr. M H Shabina Banu
Department of Radiology, Sri Ramachandra University, Chennai - 600 004, Tamil Nadu
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Shabina Banu M H, Rangasami R, Suresh I. Fetal meningocele manqué. Neurol India 2018;66:879-81
A 29-year old female patient with 33 weeks of gestation was referred for fetal magnetic resonance imaging (MRI). Her routine antenatal ultrasound of the fetus had shown a well-defined cystic lesion in the posterior aspect of upper neck with few intervening bands suggesting the presence of a lymphangioma or meningocele [Figure 1]a. Further evaluation of the cystic lesion was done by MRI using half-Fourier acquisition single-shot turbo spin echo (HASTE) sequence that revealed a well-defined pedunculated cystic lesion measuring 2.7 × 3.0 × 3.8 cm in the posterior aspect of upper neck [Figure 1]b, [Figure 1]b, [Figure 1]c, [Figure 1]d. The lesion showed a communication with the spinal canal through a small defect in the C3 posterior elements and also a few intervening bands [Figure 1]b, [Figure 1]b, [Figure 1]c, [Figure 1]d. One of the bands was seen tethering the spinal cord to the inner wall of the cystic swelling through the defect in the C3 posterior elements. A radiological diagnosis of meningocele manqué with a dorsal tethering band from the spinal cord to the cystic lesion was made. The baby was delivered by elective Cesarean section, and the meningocele repair surgery was performed. The child had an uneventful recovery without any neurological deficit.
|Figure 1: Sagittal (a) sonographic image of the fetus shows the cystic lesion in posterior aspect of upper neck (arrow) with an intervening band (open arrow). Sagittal (b) and axial MRI sections (c and d) show the cystic lesion in the posterior aspect of upper neck (arrow).The lesion shows communication with the spinal canal through a small defect in the C3 posterior elements (arrowhead). A band is seen tethering the spinal cord to the inner wall of the cystic swelling through the defect in the C3 posterior elements|
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Meningocele manqué is a rare neural tube defect. It was described as a form of tethered cord, the cord being attached by atretic neural tissue or fibrous bands. Though the terminology was initially used in referring to dorsal tethering occurring in spina bifida, it has been broadened to include cases with a dorsal tethering band associated with a meningocele, as seen in our case. Although the exact cause of meningocele manqué is unknown, the main possibility includes congenital malformation of the spinal cord and vitamin B12 deficiency. These lesions can occur in the posterior fontanelle or the spine. Antenatally, they can be detected by routine/targeted ultrasound scan of the anomaly. Antenatal MRI is complementary in confirming the diagnosis and in detecting associated cord abnormalities. Dorsal tethering bands of meningocele manqué are frequently associated with diastematomyelia. These tethering bands are usually found at the site of diastematomyelia. Other less common associations include syringohydromyelia and dermoid cysts.
The differential diagnoses are meningocele and meningomyelocele. If the herniated lesion contains meninges and cerebrospinal fluid (CSF), it is termed as meningocele [Figure 2]a. If the herniated lesion contains meninges, CSF, and spinal cord, it is referred as meningomyelocele [Figure 2]b. Meningocele manqué contains atretic neural tissue [Figure 2]c and not the spinal cord as its content and carries a good prognosis. Postnatally, MRI is the investigation of choice to diagnose the presence of a meningocele manqué, though the lesion can be visualized by ultrasonography and a computed tomographic myelography also. The postnatal findings are similar to the antenatal findings with the cystic lesion or the lipoma communicating with the spinal canal and showing the tethering atretic neural tissue. Sometimes, it may just be seen as a band of tissue tethering the cord to the dura or the surrounding tissue. It can be asymptomatic and may be incidentally found while investigating for some other complaint. Surgical treatment involves the lysis of the bands.
|Figure 2: Diagrammatic representation of differential diagnoses of meningocele manqué. (a) Meningocele – the herniated lesion contains meninges and CSF. (b) Meningomyelocele – the herniated lesion contains meninges, CSF, and spinal cord. (c) Meningocele manqué – the herniated lesion contains atretic neural tissue, meninges, and CSF|
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Spinal dysraphism is a group of anomalies which shows malformations in the dorsum of the embryo. It includes neural tube defects. The incidence of spinal dysraphism is 1 to 2 per 1000 live births. The incidence is decreasing due to folate therapy supplementation during pregnancy and better antenatal diagnosis with appropriate management. Spinal dysraphism can be further divided into two different pathological entities – open spinal dysraphism and closed spinal dysraphism. Open spinal dysraphism includes meningocele, meningocele manqué, and myelomeningocele; while closed spinal dysraphism includes dorsal dermal sinus, lipomyelomeningocele, diastematomyelia, neurenteric cyst, and thickened filum terminale. In a study of 155 patients with spinal dysraphism in North India by Kumar et al., the incidence of open neural tube defect was 72% and myelomeningocele was the leading cause. The incidence of spina bifida occulta was 28% and split cord malformation was the leading cause of tethering. Dorsal dermal sinus occurs rarely and is clinically assessed only when there are cutaneous abnormalities, neurologic deficits or infection. It is an epithelium-lined tract that extends from the skin to the spinal cord, cauda equina, or arachnoid. Lipomyelomeningocele is a birth defect affecting the spine of the child. It appears as a fatty mass under the skin in the dorsolumbar region and attaches to the spinal cord, resulting in tethering of the cord. Diastematomyelia is a longitudinal split in the spinal cord. It is a type of spinal dysraphism that occurs as a result of abnormal notochord development and abnormal clefting of the spinal canal that causes the cord to split. It is also known as a split cord malformation. Neurenteric cyst is a rare congenital abnormality of the spine, a type of foregut duplication cyst. It is further classified into a spinal or an intracranial cyst and may be associated with vertebral or central nervous system abnormalities. Fatty filum terminale is a dysraphic abnormality that may or may not be associated with tethering of the spinal cord and can be an incidental finding on spine imaging. Fatty filum terminale is also known as lipoma of the filum terminale or a filar lipoma. Patients with tethered cord syndrome show a thickened filum terminale and a low-lying conus. With growth, neurological symptoms may develop due to distortion of neural structures, particularly the cord, which may come under increasing tension.
To summarize, meningocele manqué is a malformation that generally carries a good outcome and should not be confused with myelomeningocele that has a variable extent of neural involvement.,,
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Dr. R Rajkali for his illustrative diagram
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]