Sonographic images of fetal terminal myelocystocele: a rare form of closed spinal dysraphism

OBJECTIVE The objective of this study was to assess the prevalence and spectrum of spinal dysraphism in a cohort of children with cloacal exstrophy (CEX) using MRI. METHODS Children with CEX presenting between 1999 and 2019 with baseline spinal MRI were included. The images were reviewed in consensus to assess the type of dysraphism. The dysraphisms were initially reviewed and described based on their descriptive anatomy, and then classified according to anomalies of gastrulation, primary neurulation, or secondary neurulation. RESULTS Thirty-four children were included. Thirty-three of these children had closed spinal dysraphism, and 1 had a normal spine. Of the 33 cases of closed spinal dysraphism, the conus and/or filum terminale were involved in all cases. The most common malformations were spinal lipoma (n = 20) and terminal myelocystocele (n = 11). The lipomas were heterogeneous: 4 dorsal, 9 transitional, 4 chaotic, and 3 terminal. A large subgroup (10/20, 50%) within the lipomas had an unusual morphology of noncontiguous double lipomas, the proximal fat related to the conus and the distal fat within the filum. These were difficult to characterize using existing classifications. In 2 cases, only a thickened filum was noted. The majority of these malformations were compatible with a disorder of secondary neurulation. CONCLUSIONS Complex spinal dysraphisms are consistently associated with CEX. The unusual dysraphism patterns found in this group of patients highlight the limitations of current embryological classifications. Given the propensity for neurological deterioration in this group of patients, spinal MRI should be routinely performed. The type and distribution of malformations seen have implications for the wider understanding of the pathogenesis and classification of lumbosacral lipomas.

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A Rare Form of Heteropagus Twinning: Three-Armed Infant with Spinal Dysraphism

Case Reports in Pediatrics ◽

10.1155/2012/831649 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4 ◽

Cited By ~ 2

Author(s):

Aynur Solak ◽

Sonnaz Ergün ◽

İpek Polat ◽

Neslın Şahin ◽

Berhan Genç

Keyword(s):

Vertebral Column ◽

Case Reports ◽

Spinal Dysraphism ◽

Surgical Excision ◽

Dorsal Region ◽

Rare Form ◽

Fetus In Fetu ◽

Live Births ◽

First Case ◽

Probable Diagnosis

An ectopic or accessory limb attached to the back is an extremely rare and strange condition, and there are only a few documented cases in the worldwide literature. The first case was described by Jones and Larkin (1889). There are several theories regarding the origin of this condition. Asymmetric conjoined twinning which is located dorsally in the vertebral column (rachipagus) is the most probable diagnosis of our patient. Conjoined twinning is very rare and the incidence is 1 per 50 000 live births. Rachipagus is even rarer, with no more than 30 case reports so far (Chadha et al. (1993, 2006)). In this report, we present a patient who underwent successful surgical excision of a third arm attached to the back in the midline over the low-dorsal region. Differential diagnoses including teratoma and fetus in fetu are discussed.

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Refractory CSF leakage following untethering surgery performed 10 months after birth for enlarging terminal myelocystocele associated with OEIS complex

Surgical Neurology International ◽

10.25259/sni_995_2021 ◽

2021 ◽

Vol 12 ◽

pp. 628

Author(s):

Nobuya Murakami ◽

Ai Kurogi ◽

Yoshihisa Kawakami ◽

Yushi Noguchi ◽

Makoto Hayashida ◽

...

Keyword(s):

Subarachnoid Space ◽

Spinal Dysraphism ◽

Abdominal Pressure ◽

Caudal Part ◽

Muscle Flaps ◽

Dural Sac ◽

Gluteus Muscle ◽

Csf Leakage ◽

Untethering Surgery ◽

Terminal Myelocystocele

Background: Terminal myelocystocele (TMC) is an occult spinal dysraphism characterized by cystic dilatation of the terminal spinal cord in the shape of a trumpet (myelocystocele) filled with cerebrospinal fluid (CSF), which herniates into the extraspinal subcutaneous region. The extraspinal CSF-filled portion of the TMC, consisting of the myelocystocele and the surrounding subarachnoid space, may progressively enlarge, leading to neurological deterioration, and early untethering surgery is recommended. Case Description: We report a case of a patient with TMC associated with OEIS complex consisting of omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I), and spinal deformity (S). The untethering surgery for TMC had to be deferred until 10 months after birth because of the delayed healing of the giant omphalocele and the respiration instability due to hypoplastic thorax and increased intra-abdominal pressure. The TMC, predominantly the surrounding subarachnoid space, enlarged during the waiting period, resulting in the expansion of the caudal part of the dural sac. Although untethering surgery for the TMC was uneventfully performed with conventional duraplasty, postoperative CSF leakage occurred, and it took three surgical interventions to repair it. External CSF drainage, reduction of the size of the caudal part of the dural sac and use of gluteus muscle flaps and collagen matrix worked together for the CSF leakage. Conclusion: Preoperative enlargement of the TMC, together with the surrounding subarachnoid space, can cause the refractory CSF leakage after untethering surgery because the expanded dural sac possibly increases its own tensile strength and impedes healing of the duraplasty. Early untethering surgery is recommended after recovery from the life-threatening conditions associated with OEIS complex.

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"Giant" terminal myelocystocele: A rare variant of spinal dysraphism

Asian Journal of Neurosurgery ◽

10.4103/1793-5482.162730 ◽

2015 ◽

Vol 10 (4) ◽

pp. 350 ◽

Cited By ~ 3

Author(s):

Sumit Bansal ◽

AshokKumar Mahapatra

Keyword(s):

Rare Variant ◽

Spinal Dysraphism ◽

Terminal Myelocystocele

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The Mermaid Malformation: Cloacal Exstrophy and Occult Spinal Dysraphism

Neurosurgery ◽

10.1227/00006123-199106000-00008 ◽

1991 ◽

Vol 28 (6) ◽

pp. 834-843 ◽

Cited By ~ 48

Author(s):

Alan R. Cohen

Keyword(s):

Spina Bifida ◽

Spinal Dysraphism ◽

Radiographic Examination ◽

Developmental Defect ◽

Pathological Anatomy ◽

Occult Spinal Dysraphism ◽

Cloacal Exstrophy ◽

Neurological Evaluation ◽

Terminal Myelocystocele

Abstract Five infants with cloacal exstrophy underwent neurological evaluation and radiographic examination of the caudal spine shortly after birth. Each was found to have occult spinal dysraphism. Four had terminal myelocystoceles, and one had a lipomyelomeningocele. Pathological anatomy was confirmed during surgery for the release of the tethered spinal cords. The striking association between cloacal exstrophy and occult spinal dysraphism suggests a common developmental defect in the caudal pole of the embryo. A hypothesis is offered to explain this association. Terminal myelocystocele and lipomyelomeningocele appear to be part of a continuum of lesions associated with skin-covered spina bifida.

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A rare case of thoracic myelocystocele associated with type 1 split cord malformation with low lying tethered cord, dorsal syrinx and sacral agenesis: Pentad finding

Journal of Neurosciences in Rural Practice ◽

10.4103/0976-3147.143209 ◽

2015 ◽

Vol 6 (01) ◽

pp. 087-090 ◽

Cited By ~ 1

Author(s):

Dipanker Singh Mankotia ◽

Guru Dutta Satyarthee ◽

Bhawani Shankar Sharma

Keyword(s):

Spina Bifida ◽

Surgical Management ◽

Rare Case ◽

Clinical Presentation ◽

Spinal Dysraphism ◽

Tethered Cord ◽

Split Cord Malformation ◽

Rare Form ◽

Sacral Agenesis

ABSTRACTMyelocystocele is a rare form of spinal dysraphism. Thoracic myelocystocele is still rarer. The occurrence of thoracic myelocystocele associated with type-1 split cord malformation, low lying tethered cord, dorsal syrinx and spina bifida is extremely rare. Clinical presentation of such a rare case and an early surgical management is discussed briefly.

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Asymptomatic posterior cervical myelomeningocele with tethered cord in an adolescent: a rare form of spinal dysraphism with rare presentation

Romanian Neurosurgery ◽

10.1515/romneu-2016-0018 ◽

2016 ◽

Vol 30 (1) ◽

pp. 113-117

Author(s):

Gangesh Gunjan ◽

Sumit Sharma ◽

S.K. Jain ◽

S. Chopra

Keyword(s):

Spinal Dysraphism ◽

Tethered Cord ◽

Cervical Cord ◽

Rare Form ◽

Rare Presentation ◽

Lumbar Spinal

AbstractUnlike lumbar spinal dysraphism, cervical spinal dysraphism with or without tethered cord are rare lesions. These lesions are generally asymptomatic at birth, but with progression of time symptoms develop. These may be associated with various other anomalies. Not much have been reported in literature about cervical myelomeningocele (MMC) in adults or adolescents. We report a case of a 17 years old adolescent boy with cervical myelomeningocele with tethered cervical cord, who was completely intact neurologically even at this age and was without any associated anomaly. He came to us only for cosmetic reasons. The tethering band, which was evident on imaging was confirmed intra-operatively.

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