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CASE REPORT |
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| Year : 2007 | Volume
: 2
| Issue : 1 | Page : 26-27 |
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Double meningomyelocele: Embryogenesis
Dinesh Sarda, Paras Kothari, Ashok Laddha, Bharati Kulkarni
Department of Pediatric Surgery, Lokmanya Tilak Municipal General Hospital, Sion, Mumbai, India
Correspondence Address:
Paras Kothari
Department of Pediatric Surgery, Room No. 440, College Building, Lokmanya Tilak Municipal General Hospital, Sion, Mumbai - 400 022
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/1817-1745.32004
 Abstract | | |
We report a case where two distinct meningoceles (thoracic and lumbosacral) with a normal intervening spinal canal was encountered. It was successfully treated by operative intervention.
Keywords: Embryogenesis, meningocele
How to cite this article:
Sarda D, Kothari P, Laddha A, Kulkarni B. Double meningomyelocele: Embryogenesis. J Pediatr Neurosci 2007;2:26-7 |
| Introduction | |  |
Meningomyelocele is a severe developmental anomaly which is characterized by a protrusion of neural elements often including the spinal cord, through a defect in the vertebra into the meningeal lined sac. The coexistence of more than one meningocele of spine is an extremely rare event.
| Case Report | | |
A 2.5 kg full-term normally delivered male was referred on day 3 of life in view of swellings on the back [Figure - 1]. Mother's age was 21, and antenatal ultrasound done at 7 th month showed polyhydroamnios.
On examination, there was a midline swelling on back in thoracic region, which was approximately 4 cm x 3 cm in size. It was soft, cystic, brilliantly transilluminant; and impulse on crying was present. It was skin covered. Another similar swelling was noted in the lumbosacral region, which was of size 5 cm x 7 cm. It was also soft, cystic and brilliantly transilluminant but covered with thin membrane. Both lower limbs had normal power and sensation. Anal canal was nonpatulous. Bladder was nonexpressible. There was no hydrocephalus. There were no other congenital malformations. Ultrasound of skull and abdomen was normal. Blood investigations were normal.
The neonate was posted for surgery. Thoracic meningocele had few nerve fibers inside attached to sac, which were dissected carefully. Sac and lumbar fascia were closed. Adequate skin cover without tension could be achieved. Lumbar meningocele had neural placode attached to the sac, which was dissected from meninges and reposited back. Skin cover could be achieved after adequate mobilization. Postoperatively, patient did well. There was no CSF leak. Wounds healed well in 10 days and the patient was discharged. There was no neurodeficit in lower limbs. At 9 months follow-up, the child was healthy and had normal milestones with respect to age. There was no hydrocephalus.
| Discussion | | |
Lumbosacral region is the commonest site for meningocele. The presence of two distinct meningoceles is very unusual. Very few cases of double meningocele are reported in the literature. Earliest reports of double meningocele are by Potter [1] and Fahrenkrug. [2] Multiple meningoceles are often seen in anterior or lateral location of spine. [3],[4] They are associated with other congenital malformations like Arnold Chiari malformation More Details, Marfan's features or neurofibromatosis. [5] In our case, there was posterior protrusion of sac through the defect in neural arch.
Occipital meningoceles are often associated with spina bifida of the upper cervical segments and in severe cases may constitute an encephalomyelocele. In such cases, however, there is no segment of normal spine in between the occipital and the spinal defects. [6]
Between the third and fifth week of gestation, the neural plate invaginates and becomes the neural groove, which closes dorsally to form the neural tube. It separates from the overlying ectoderm by mesodermal elements, which later form the vertebral column and the supporting soft tissue. Developmental errors occurring early in this sequence produce deformities of the nervous elements as well as supporting system. [7] Recent genetic research on neural tube defect has focused on the different location of neural tube defects. The multi-site neural tube closure model suggests the existence of five separate closure points, or "zippers," in normal neural tube in humans. This indicates the existence of additional neuropores, which are the most frequent site for the defect. These five different "zippers" span the length of the neural tube and function to close it during development. For different levels of defects, the perturbation in the neurulation process occurs in a defined part of the neural tube. These zippers are presumably under the control of one or more genes, mutations in which would cause neural tube defect in the region of that zipper. [8],[9] That may explain the embryogenesis of concomitant double meningoceles.
| References | | |
| 1. | Potter EL. Pathology of the foetus and infant. Year Book Medical Publishers: Chicago; 1962. p. 571.  |
| 2. | Fahrenkrug A, Hojgaard K. Multiple para-vertebral lumbar meningocele. Br J Radiol 1963;36:574. [PUBMED] |
| 3. | Rainov NG, Heidecke V, Burkert W. Thoracic and lumbar meningocele in neuro gibromatosis type 1. Report of two cases and review of the literature. Neurosurg Rev 1995;18:127-34. |
| 4. | Gripp KW, Scott CI, Hughes HE, Wallerstein R, Nicholson L, States L. Lateral meningocele syndrome: Three new patients and review of the literature. Am J Med Genet 1997;70:229-39. |
| 5. | de Andrade GC, Braga OP, Hisatugo MK, de Paiva Neto MA, Succi E, Braga FM. Giant intrathoracic meningoceles associated with cutaneous neurogivromatosis type I: Case report. Arq Neuropsiquiatr 2003;61L677-81. |
| 6. | Bailey IC. Double meningocele. Arch Dis Child 1971;46:549-50. [PUBMED] |
| 7. | Milhorat TH. Spinal and cranial dysraphism. Pediatric neurosurgery. FA Davis Company: Philadelphia; p. 37-8. |
| 8. | Nasib C. Digra Primary Prevention of Neural Tube Defects. JK Science: January-March 2004;6:1-3. |
| 9. | Monika GO, Agnieszka S. Genetic basis of neural tube defects. I. Regulatory genes for the neurulation process Appl Genet 2002;43:343-50. |
[Figure - 1]
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