Dataset Information

Split Cord Malformation Type 2 with Double Dorsal Lipoma: A Sequela or a Chance.

ABSTRACT: An 11-month-old baby girl, with normal perinatal history, presented with a congenital lumbosacral swelling and a sacral dimple. Imaging revealed a split cord malformation (SCM) type 2 at L1-L3 with a lipomeningocoele extending intradurally and tethering both the hemicords, the conus was noted to be at L4. She underwent excision of the lipoma and detethering of the hemicords. Intraoperatively, the split cord was seen from L1 to L3 with an aberrant median vessel passing between the hemicords. Two lipomas were seen separately attached to each of the hemicords, the lipomas were dissected off the hemicords, and the hemicords were neurulated. The case helps revisit the unified theory proposed by Pang for SCM as well as the theory of premature disjunction in the pathogenesis of lipomeningocoele. Formation of the endomesenchymal tract splits the developing cord into two, whereas the premature detachment of neuroectoderm from the cutaneous ectoderm can lead to lipomeningocoeles. The present case is only the fourth case ever reported of an SCM type 2 with double lipoma. In the case report, we discuss the embryological basis of this condition and surgical nuances of management.

SUBMITTER: Jamaluddin MA

PROVIDER: S-EPMC7519753 | biostudies-literature | 2020 Apr-Jun

REPOSITORIES: biostudies-literature

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Split Cord Malformation Type 2 with Double Dorsal Lipoma: A Sequela or a Chance.

Jamaluddin Mohamed A MA Nair Prakash P Divakar Ganesh G Gohil Jaypalsinh A JA Abraham Mathew M

Journal of pediatric neurosciences 20200401 2

An 11-month-old baby girl, with normal perinatal history, presented with a congenital lumbosacral swelling and a sacral dimple. Imaging revealed a split cord malformation (SCM) type 2 at L1-L3 with a lipomeningocoele extending intradurally and tethering both the hemicords, the conus was noted to be at L4. She underwent excision of the lipoma and detethering of the hemicords. Intraoperatively, the split cord was seen from L1 to L3 with an aberrant median vessel passing between the hemicords. Two ...[more]

PMID: 33042248

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Project description:BackgroundSplit cord malformations (SCMs) are uncommon congenital anomalies. They can be divided into Type 1 (bony septum and two separate dural sheaths) and Type 2 (fibrous septum and a single dural sheath).[1,2,4] Rarely,SCM can be associated with orthopedic anomalies (mostly seen in older children), such as scoliosis, followed by kyphosis, and/or both.[1-3] Conversely, patients with congenital scoliosis have 40% risk of congenital underlying neural deformities, with 16.3% attributed to SCM.[1,4] Those with thoracic or lumbosacral SCM may have congenital tethering lesions, predominantly consisting of intradural lipomas.[3,4] Surgery is optimally performed in two stages: first, removal of the spinal septum and untethering of the cord, and second, correction of the kyphosis, scoliosis, and/or both.[1-5].Case descriptionIn this video, a 44-year-old female initially presented after having progressively developed kyphoscoliosis since childhood. Three months before presentation, she had developed increased left lower extremity pain with hypoesthesia followed 1 month later by the onset of the left lower extremity monoplegia (0/5). The computed tomography and magnetic resonance imaging studies revealed a SCM at the L3 level with bony and membranous septum, accompanied by an intradural lipoma, and tethered cord. She underwent a two- stage procedure; first, removal of the bony and membranous septum, resection of the intradural lipoma, and untethering of the cord; second, she had correction of the kyphoscoliosis. Postoperatively, although the pain and sensory deficits improved, the left lower extremity monoplegia remained.ConclusionSCM is a rare cause of spinal deformity. If left untreated, the associated neurological deficits may progress. Treatment should include a two-staged approach; first, the bony and membranous septum should be removed followed by lipoma resection and untethering the cord with adequate cord decompression, while second, a fusion may be performed to address attendant kyphoscoliosis.KeywordsIntradural lipoma, Kyphoscoliosis, Split cord malformation, Tethered cord.

Project description:BackgroundTraumatic spinal cord injury (SCI) is also a combat-related injury that is increasing in modern warfare. The aim of this work is to inform medical experts regarding the different course of bladder cancer in able-bodied patients compared with SCI patients based on the latest medical scientific knowledge, and to present decision-making aids for the assessment of bladder cancer as a late sequela of traumatic SCI.MethodsA study conducted between January 1998 and December 2019 in the BG Trauma Hospital Hamburg formed the basis for the decision-making aids. Urinary bladder cancer was diagnosed in 40 out of 7396 treated outpatient and inpatient SCI patients. General patient information, latency period, age at initial diagnosis, type of bladder management and survival of SCI patients with bladder cancer were collected and analysed. T category, grading and tumour entity in these patients were compared with those in the general population. Relevant bladder cancer risk factors in SCI patients were analysed. Furthermore, relevant published literature was taken into consideration.ResultsInitial diagnosis of urinary bladder cancer in SCI patients occurs at a mean age of 56.4 years (SD ± 10.7 years), i.e., approximately 20 years earlier as compared with the general population. These bladder cancers are significantly more frequently muscle invasive (i.e., T category ≥ T2) and present a higher grade at initial diagnosis. Furthermore, SCI patients show a significantly higher proportion of the more aggressive squamous cell carcinoma than that of the general population in areas not endemic for the tropical disease schistosomiasis. Consequently, the survival time is extremely unfavourable. A very important finding, for practical reasons is that, in the Hamburg study as well as in the literature, urinary bladder cancer is more frequently observed after 10 years or more of SCI. Based on these findings, a matrix was compiled where the various influencing factors, either for or against the recognition of an association between SCI and urinary bladder cancer, were weighted according to their relevance.ConclusionsThe results showed that urinary bladder cancer in SCI patients differs considerably from that in able-bodied patients. The presented algorithm is an important aid in everyday clinical practice for assessing the correlation between SCI and bladder cancer.

Dataset Information

Split Cord Malformation Type 2 with Double Dorsal Lipoma: A Sequela or a Chance.

Publications

Split Cord Malformation Type 2 with Double Dorsal Lipoma: A Sequela or a Chance.

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