Seven Distinct Coexistent Cranial and Spinal Anomalies

Abstract

Existence of multiple cranial and spinal anomalies in spina bifida is well known; however, coexistence of seven different severe anomalies is extremely rare. The location of the anomalies, the patient’s age and presentation are other interesting aspects of the presented case. Case Report: A 1-year-old girl with an enlarged head and big, infected lumbosacral myelomeningocele (MMC) was admitted to the emergency department in a comatose state. Further investigations revealed hydrocephalus, Chiari malformation, syringohydromyelia, split cord malformation, dermal sinus tract, lumbosacral MMC, and tethered cord. Unfortunately, the patient died 3 days after admission because of meningitis and sepsis. Discussion: Coexistence of seven different craniospinal anomalies is extremely unusual. Further investigations with magnetic resonance imaging for associated anomalies, early shunt placement and MMC repair are required to prevent this life-threatening condition in spina bifida.

Myelomeningocele Associated With Split Cord Malformation Type I -Three Case Reports-

“…1,2,4,5,7,11,14,15) Other central nervous system anomalies, such as Chiari malformation, hydrocephalus, and syrinx, are also common in patients with SCM and MMC, so management of these disorders may be difficult. 3,9,10) Here we describe three difficult cases of SCM and MMC associated with various concomitant anomalies.…”

Do we need to scan the whole neuraxis for coexistent abnormalities in children with surgically treated occult spinal dysraphism?

“…SDs may be in simple and complex forms. Simple SD has single type of SDs without any associated abnormalities like syrinx, chiari malformation or hydrocephalus, while complex form includes multiple types of SDs, or associated abnormalities . Emmez et al .…”

“…Simple SD has single type of SDs without any associated abnormalities like syrinx, chiari malformation or hydrocephalus, while complex form includes multiple types of SDs, or associated abnormalities . Emmez et al . reported an extremely rare case with seven different pathologies including hydrocephalus, Chiari malformation, syringohydromyelia, SCM, dermal sinus tracts, myelomeningocele and tethered cord.…”

“…Spinal dysraphism (SD) is a group of congenital malformations associated with neural tube closure defects during early embryogenesis between 2 and 6 weeks of gestational age . Closed spinal dysraphism (spina bifida occulta, SBO) including lipomyelomeningocele, terminal myelocystocele, fatty thickened filum terminale, split cord malformations (SCMs), meningocele manqué, dermal sinus tract, neuroenteric and dermoid cysts is a result of secondary neurulation abnormalities (third to fourth week) …”

“…Spinal dysraphism (SD) is a group of congenital malformations associated with neural tube closure defects during early embryogenesis between 2 and 6 weeks of gestational age. [1][2][3] Closed spinal dysraphism (spina bifida occulta, SBO) including lipomyelomeningocele, terminal myelocystocele, fatty thickened filum terminale, split cord malformations (SCMs), meningocele manqué, dermal sinus tract, neuroenteric and dermoid cysts is a result of secondary neurulation abnormalities (third to fourth week). 1,3 Currently, it is well known that open SDs may coexist with various cranial abnormalities, nevertheless, associated cerebral malformations are less known for closed neural tube defects and these defects seem to be unrelated with cranial anomalies.…”