Double encephalocele: An unusual presentation

Yhoshu, Enono; Dash, Vedarth; Bawa, Monika

doi:10.4103/jpn.jpn_22_18

Cited by 3 publications

(1 citation statement)

References 8 publications

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“…In the present study, the rare craniofacial defect associated with ABS was double encephalocele, which is characterized by multiple neural tube defects of rare occurrence. [ 23 ] According to Van Allen et al . [ 21 ]and Nakatsu et al .,[ 15 ] there are several points of neural tube closure controlled by one or more genes, and the failure of one or more of them to close could lead to the formation of multiple neural tube defects.…”

Section: Discussionmentioning

confidence: 99%

Amniotic band syndrome with double encephalocele: A case report

Silva

Silva²,

Mariano³

2020

Surgical Neurology International

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Background: Amniotic band syndrome (ABS) is a rare condition of controversial etiology that is associated with varying degrees of anomalies. This study reports a case of a newborn with ABS associated with double encephalocele in the frontal region. Case Description: A 29-year-old primiparous woman with no history of prenatal infection or consanguineous marriage had a cesarean section at gestational week 38, giving birth to a newborn who was well but had limb anomalies (constriction rings, amputations, and syndactyly) and craniofacial anomalies, mainly double frontal encephalocele. The patient underwent surgical repair and subsequent placement of a ventriculoperitoneal shunt. Conclusion: Studies clarifying this uncommon association with double encephalocele are limited. ABS associated with double encephalocele is rare and even more complex when associated with other anomalies. Thus, the conditions in such children are severe and require multidisciplinary monitoring.

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Section: Discussionmentioning

confidence: 99%

Amniotic band syndrome with double encephalocele: A case report

Silva

Silva²,

Mariano³

2020

Surgical Neurology International

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Multiple-site neural tube defects: embryogenesis with complete review of existing literature

Deora¹,

Srinivas²,

Shukla³

et al. 2019

Neurosurgical Focus

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OBJECTIVEMultiple-site neural tube defects (MNTDs) are very uncommon, with the predominant number of cases being reported in developing countries. The classic theory of neural tube closure fails to explain the occurrence of these defects. Multisite closure theory, first proposed in 1995, explains most of the occurrences with a few modifications specific to a few defects. In this paper, the authors endeavor to explain all the defects, along with their genetic and embryological bases, and to review the available literature and discuss their own experience in the management of these complex cases.METHODSThe authors retrospectively reviewed the data of all the patients treated surgically for MNTDs over that past 14 years. All possible demographic data, clinical details, and radiological imaging data were reviewed. In addition, surgical parameters, complications, and status at follow-up of more than 12 months were evaluated. All previously reported cases of MNTD were analyzed, and comparisons with the present series were made.RESULTSA total of 3 major series (including the present one) on MNTDs have been from India. A total of 57 such cases (including those of the present series) have been reported in the available literature. While previous series reported a higher incidence of spinal defects, the present series had a higher rate of cephalic defects (55%). Among the reported cases, insertion of a ventriculoperitoneal shunt was necessary in 12 (26%), and only 4 patients were operated on in 2 stages. Neurological status at presentation dictated outcome.CONCLUSIONSMNTDs are extremely rare, and their embryogenesis is different from that of single neural tube defects. Simultaneous repair of 2 or even 3 defects is possible in a single-stage surgery. The requirement of a shunt is uncommon, and complications following surgery are rare. Folic acid supplementation may reduce the incidence of defects.

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