Presentation of a double myelomeningocele in the upper thoracic and thoracolumbar spine: A case report

Elarjani, Turki; Khairy, Sami; Al-Karawi, Sarmad

doi:10.1016/j.ijscr.2020.10.060

Cited by 2 publications

(4 citation statements)

References 10 publications

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“…In the same context, our patient was free of any associated congenital anomalies. Elarjani et al [25] reported the association of hydrocephalus and scoliosis with double upper thoracic and thoracolumbar MMC; in addition, 29.8% of NTD The patient in this case was operated at 2 months of age. However, it is recommended to perform surgery as soon as possible, particularly in patients diagnosed prenatally, the optimal time for surgery of MMC cases is known to be within the first 5 days as per Oncel et al [27].…”

Section: Discussionmentioning

confidence: 65%

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Double Lumbar Localization of Myelomeningocele: Case Report

Belfatmi¹,

Motawei²,

Rezkallah³

et al. 2023

Pediatr Neurosurg

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Introduction: myelomeningocele (MMC) is a malformation resulting from the neural tube’s failure to close during embryonic development, the majority of the cases of neural tube defects (NTDs) were prevalent as single location lesions along the spine, however multiple neural tube defects (MNTDs) are a very rare condition. Only a few cases of MNTDs were found in the literature. Case Report: we report the case of a 2-month-old male infant prenatally diagnosed with MMC, presented with two unconnected lumbar and lumbosacral epidermal, soft, dome-shaped swellings located on both sides of the midline (paravertebral) covered by intact skin. MRI revealed double myelomeningocele at the level of L4-L5, with spinal nerve roots. The patient underwent surgical repair of the defects by replacing the spinal cord and its nerve roots inside the thecal sac and recreating a covering layer around the neural structures to resemble thecal sac. The outcome was favorable, and postoperative head CT scan did not show any complication. Discussion/Conclusion: Our case report is considered the first from Algeria to report the condition and the first to report the occurrence of double lesions in the same spine region. MMC can be associated with neurological deficits or other congenital anomalies, thus it is necessary to thoroughly examine such patients. However there was no antenatal folic acid deficiency in our case, We recommend antenatal care with adequate folic acid supplementation given that its deficiency during pregnancy is considered a ubiquitous risk factor for the condition. The optimal timing for surgery of MMC cases is 8±5 days. Prenatal intrauterine repair of the condition provides favorable outcomes but carries high fetal and maternal risks. Surgical repair should include the sac removal, the reconstruction of the placode and the closure of the overlying meninges. With early diagnosis and proper repair of such cases, MMC has good prognosis and favorable outcomes.

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Section: Discussionmentioning

confidence: 65%

Section: Discussionmentioning

confidence: 99%

Double Lumbar Localization of Myelomeningocele: Case Report

Belfatmi¹,

Motawei²,

Rezkallah³

et al. 2023

Pediatr Neurosurg

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“…Therefore, the number of centers offering this fetal intervention for selected cases with isolated OSB is growing worldwide [6,7]. Double neural tube defects on the spine are rare, accounting for less than 1% of all defects [8], and are considered a severe condition associated with hydrocephalus often requiring postnatal ventriculoperitoneal shunting, severe motor and neurological impairment, and even infant death [9][10][11][12][13][14][15]. There are only a few cases of double neural tube defects reported in the literature, with a very small proportion of them having been diagnosed prenatally [15,16], and all of them receiving postnatal surgical repair [17][18][19].…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, the number of centers offering this fetal intervention for selected cases with isolated OSB is growing worldwide [6, 7]. Double neural tube defects on the spine are rare, accounting for less than 1% of all defects [8], and are considered a severe condition associated with hydrocephalus often requiring postnatal ventriculoperitoneal shunting, severe motor and neurological impairment, and even infant death [9‒15]. There are only a few cases of double neural tube defects reported in the literature, with a very small proportion of them having been diagnosed prenatally [15, 16], and all of them receiving postnatal surgical repair [17‒19].…”

Section: Discussionmentioning

confidence: 99%

Double Myelomeningocele Repair by Fetal Surgery with a Single Micro-Hysterotomy

Chavelas-Ochoa,

Bermúdez-Rojas,

Medina-Jiménez

et al. 2024

Pediatr Neurosurg

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Introduction: Open spina bifida (OSB) is the most common congenital anomaly of the central nervous system. It is associated with severe neurodevelopmental delay, motor impairment, hydrocephalus, and bowel and bladder dysfunction. In selected cases, intrauterine spina bifida repair has been shown to improve neonatal outcomes. Rarely, the spine can have a double defect compromising two different segments and there is a lack of evidence on the feasibility and benefits of intrauterine repair in these cases. Case Presentation: We present a case with both cervicothoracic and lumbosacral myelomeningocele, Arnold-Chiari malformation type II and bilateral ventriculomegaly, that was treated successfully at 25 weeks with open micro-neurosurgery. Double myelomeningocele was successfully treated through a single 2-cm micro-hysterotomy, by performing external versions to sequentially expose and repair both defects. Weekly postoperative follow-up showed no progression of ventriculomegaly or complications attributable to the procedure. Preterm rupture of membranes prompted a conventional cesarean delivery at 32 weeks of gestation. Neurodevelopmental outcome at 20 months was within normal ranges, having achieved ambulation without orthopedic support and with no need for ventriculoperitoneal shunting. Conclusion: This report demonstrates for the first time the feasibility of double OSB repair through a single 2-cm micro-hysterotomy, suggesting that selected isolated cases of double myelomeningocele could be candidates for fetal intervention. Further prospective studies should be carried out to assess the potential benefit of double OSB intrauterine open repair.

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Presentation of a double myelomeningocele in the upper thoracic and thoracolumbar spine: A case report

Abstract: Highlights Multiple neural tube defects is very uncommon. High morbidity in multiple neural tube defects compared to single defects. Association of other spinal cord and vertebral malformation with multiple neural tube defects.

Cited by 2 publications

References 10 publications

Double Lumbar Localization of Myelomeningocele: Case Report

Double Lumbar Localization of Myelomeningocele: Case Report

Double Myelomeningocele Repair by Fetal Surgery with a Single Micro-Hysterotomy

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