scholarly journals 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

2015 ◽  
Vol 6 (01) ◽  
pp. 087-090 ◽  
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.

scholarly journals Spinal dysraphism with tripedus morphology: A case report

2021 ◽  
pp. 509-511
Author(s):  
Mohd Monis ◽  
Shagufta Wahab ◽  
Divyashree Koppal ◽  
Aiman Ibbrahim
Keyword(s):  
Case Report ◽  
Spina Bifida ◽  
Vertebral Column ◽  
Rare Case ◽  
Surgical Intervention ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Lower Back ◽  
Rare Case Report ◽  
Spinal Mri

This is a rare case report of a 5-month-old child with a complex spinal dysraphic state, and an accessory limb (tripedus morphology), accessory genitalia, and anal dimple. The child was brought to the hospital with an accessory limb arising from the back. On clinical examination, an accessory limb arising from the lower back with a partially developed foot with the presence of toes and nails was noted. Spinal MRI was advised which revealed dysraphic features including spina bifida with the low lying and posteriorly tethered cord with diastematomyelia along with a supernumerary appendage attached to the vertebral column having rudimentary bones resembling those of extremities. The presence of an accessory limb with spinal dysraphism is quite a rare anomaly. The condition can be treated by surgical intervention and involves excision of the accessory limb with adequate dural and paraspinal muscle cover.

Surgical Management of Right Aortic Arch Obstruction Associated With Rare Form of Vascular Ring

2020 ◽  
Vol 11 (2) ◽  
pp. 222-225
Author(s):  
Evgeny V. Krivoshchekov ◽  
Frank Cetta ◽  
Oleg A. Egunov ◽  
Evgenii A. Sviazov ◽  
Valeriy O. Kiselev ◽  
...  
Keyword(s):  
Aortic Arch ◽  
Surgical Management ◽  
Rare Case ◽  
Vascular Ring ◽  
Right Aortic Arch ◽  
Rare Form ◽  
Descending Aorta ◽  
Arch Obstruction ◽  
Right Descending Aorta

This clinical case demonstrated surgical management for a rare case of vascular ring associated with an elongated and kinked aortic arch and a right descending aorta in a ten-year-old male using an extra-anatomic bypass grafting method and dividing the vascular ring. Computer tomography performed at six-month follow-up showed a favorable surgical outcome.

scholarly journals Limited Dorsal Myeloschisis with and without Type I Split Cord Malformation: Report of 3 Cases and Surgical Nuances

Medicina ◽  
2019 ◽  
Vol 55 (2) ◽  
pp. 28
Author(s):  
Yusuf Izci ◽  
Cahit Kural
Keyword(s):  
Spinal Cord ◽  
Tethered Cord ◽  
Intraoperative Neuromonitoring ◽  
The Other ◽  
Split Cord Malformation ◽  
Type I ◽  
Cystic Lesions ◽  
Rare Form ◽  
Urological Complications ◽  
Spinal Malformations

Limited dorsal myeloschisis (LDM) is a rare form of spina bifida which is characterized by a fibroneural stalk between the inner part of the skin and the spinal cord. It may be associated with split cord malformation (SCM). Diagnosis and management of this complex malformation is challenging. We presented 3 different cases of LDM. Two of them were associated with Type I SCM and the other had no associated malformation. All of them were evaluated radiologically just after the birth and underwent surgical treatment under intraoperative neuromonitoring. They discharged without any complication. Newborns with spinal cystic lesions should be carefully evaluated for spinal malformations after the birth and treated surgically as soon as possible in order to prevent neurological and urological complications secondary to tethered cord syndrome. Surgical technique in LDM-SCM patients is quite different than the patients with solitary LDM.

scholarly journals Surgical Management of Spinal Dysraphism: Five -year Experience in a Central Hospital

2014 ◽  
Vol 34 (1) ◽  
pp. 34-38
Author(s):  
N Banskota ◽  
R Jha ◽  
N Khadka ◽  
GR Sharma ◽  
P Bista ◽  
...  
Keyword(s):  
Surgical Management ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Clinical Presentation ◽  
Spinal Dysraphism ◽  
Cell Mass ◽  
Sensory Loss ◽  
Fetal Life ◽  
Common Location ◽  
Lower Limb Paralysis

Introduction: Spinal dysraphism is a heterogeneous group of congenital spinal anomalies resulting from defective closure of the neural tube early in fetal life and anomalous development of the caudal cell mass. Meningomyelocele is common among Neural tube defects. Patients with myelomeningocele present with a spectrum of impairments, including primary functional deficits like are lower limb paralysis, sensory loss, bladderbowel dysfunction and cognitive dysfunction. Medical, surgical management and rehabilitation have helped patients with neural tube defects to participate and be productive in mainstream society. The aims of this study were to review the clinical presentation, surgical management and their outcome in the patient with spinal dysraphism. Materials and Methods: This is a retrospective study of Forty-one cases of spinal dysraphism managed during a period of five years from January 2008 to December 2012 in Department of Neurosurgery, National Academy of Medical Sciences (NAMS), Bir Hospital. Demographic profiles, clinical presentation of patients with spinal dysraphism, associated hydocephalus, surgical management and outcome were studied. Results: Out of total 41 cases studied, male patients outnumbered female with 58.5% to 41.5%. Age ranged from 5 days to 29 years and mean age was 2.71 years whereas. Lump (97%) and paraparesis (88%) were frequent mode of presentation. Lumbar lesion (65%) was commonest followed by lumbosacral (29%). Hydrocephalus was present in 51% of cases and in 24% cases developed hydrocephalus later after repair. Total 75.6% of cases were treated with VP shunt. Conclusion: Spinal dysraphism is debilitating entity and management is challenging. Lump on back and weakness of limb are major factor for children and their parents seek medical service. Lesion in low back (lumbar and lumbosacral) were most common location. Besides repair, majority of them needed CSF diversion surgery for hydocephalus. Aim of surgical management was to prevent further deterioration, control of hydrocephalus or leak. DOI: http://dx.doi.org/10.3126/jnps.v34i1.9378 J Nepal Paediatr Soc 2014;34(1):34-38

scholarly journals Occult spinal dysraphia - a case report

2004 ◽  
Vol 132 (suppl. 1) ◽  
pp. 111-114 ◽  
Author(s):  
Mirjana Raicevic ◽  
Dusan Abramovic ◽  
Miljan Mihajlovic ◽  
Ivana Petronic ◽  
Marko Vidosavljevic
Keyword(s):  
Diabetes Mellitus ◽  
Spina Bifida ◽  
Spinal Dysraphism ◽  
Occult Spinal Dysraphism ◽  
Skin Changes ◽  
Previous Pregnancy ◽  
X Ray ◽  
Female Children ◽  
Neurosurgical Treatment

One of the most complex and most difficult congenital anomalies is spina bifida. Peter Van Forest was the first one who noticed this anomaly in 1587, and Recklinghausen, in 1886, classified spina bifida to types and suggested surgical procedures for its management. Earlier name, spina bifida, is currently more and more replacing with a term ?defect of neural tube? (NTDs), or even more, ?spinal dysraphia?. Anomaly can appear at any level of spinal cord (cervical, thoracal, lumbar and sacral) and posterior localization is more often than the anterior one. Contrary to the open spinal dysraphism that can be perceived immediately, closed spinal dysraphism is very deceiving anomaly, and therefore, it must be treated properly as soon as it is diagnosed. Because of its seclusion, the term usually used is ?occult spinal dysraphism (OSD)?. The incidence of this anomaly is unknown, but it has been reported that it is more common among female children. Etiology of OSD is also unknown, but some of its risk factors are as follows: previous pregnancy with NTD, partner with NTD, type 1 diabetes mellitus, usage of anticonvulsives, a lack of folates in mother?s nutrition. Prenatal diagnose of OSD is practically impossible. Skin changes, orthopedic, urological and neurological problems, suggest considering this complex anomaly. X-ray, ECHO, MRI, as well as neuropsychological examination corroborate diagnosis. At the same time, the diagnosis (once it is confirmed) represents the indication for neurosurgical treatment.

Bilateral vertex extradural hematomas: A case report

2008 ◽  
Vol 05 (02) ◽  
pp. 109-111 ◽  
Author(s):  
SGS Datta
Keyword(s):  
Case Report ◽  
Head Injury ◽  
Clinical Features ◽  
Rare Case ◽  
Clinical Presentation ◽  
Rare Form ◽  
Excellent Outcome ◽  
Intra Cranial Pressure

AbstractBilateral vertex extra dural hematomas (EDH) are a rare form of extra dural hematomas with many unique features. We report one such case of bilateral vertex extra dural hematoma. A young house wife had a fall in the bathroom and sustained head injury. She showed clinical features of progressive raised intra cranial pressure. Neuroimaging revealed bilateral vertex EDH. The larger left sided hematoma was evacuated surgically while the smaller right sided hematoma was managed non operatively, with excellent outcome. This case report presents this rare case and briefly reviews the literature. Vertex EDH has a special uniqueness as to their etiopathology, clinical presentation, diagnosis & management principles.

scholarly journals History of the current understanding and management of tethered spinal cord

2016 ◽  
Vol 25 (1) ◽  
pp. 78-87 ◽  
Author(s):  
Sam Safavi-Abbasi ◽  
Timothy B. Mapstone ◽  
Jacob B. Archer ◽  
Christopher Wilson ◽  
Nicholas Theodore ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Spina Bifida ◽  
20Th Century ◽  
Surgical Management ◽  
Spinal Dysraphism ◽  
Current Understanding ◽  
Spina Bifida Occulta ◽  
Tethered Spinal Cord ◽  
Modern Management ◽  
Modern Imaging

An understanding of the underlying pathophysiology of tethered cord syndrome (TCS) and modern management strategies have only developed within the past few decades. Current understanding of this entity first began with the understanding and management of spina bifida; this later led to the gradual recognition of spina bifida occulta and the symptoms associated with tethering of the filum terminale. In the 17th century, Dutch anatomists provided the first descriptions and initiated surgical management efforts for spina bifida. In the 19th century, the term “spina bifida occulta” was coined and various presentations of spinal dysraphism were appreciated. The association of urinary, cutaneous, and skeletal abnormalities with spinal dysraphism was recognized in the 20th century. Early in the 20th century, some physicians began to suspect that traction on the conus medullaris caused myelodysplasia-related symptoms and that prophylactic surgical management could prevent the occurrence of clinical manifestations. It was not, however, until later in the 20th century that the term “tethered spinal cord” and the modern management of TCS were introduced. This gradual advancement in understanding at a time before the development of modern imaging modalities illustrates how, over the centuries, anatomists, pathologists, neurologists, and surgeons used clinical examination, a high level of suspicion, and interest in the subtle and overt clinical appearances of spinal dysraphism and TCS to advance understanding of pathophysiology, clinical appearance, and treatment of this entity. With the availability of modern imaging, spinal dysraphism can now be diagnosed and treated as early as the intrauterine stage.

scholarly journals Focal Fibroadipose Overgrowth of the Forehead: A Case Report

2020 ◽  
Vol 3 (3) ◽  
pp. 234-240
Author(s):  
Boon B ◽  
De Praeter M ◽  
Jentjens S ◽  
van Heerden J
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Surgical Management ◽  
Rare Case ◽  
Patient Management ◽  
Clinical Presentation ◽  
Future Perspective ◽  
Proteus Syndrome ◽  
Best Interest ◽  
Overgrowth Syndromes

This article describes a rare case of isolated focal fibroadipose overgrowth of the forehead in a 15-year-old patient. Various overgrowth syndromes were considered in the differential diagnosis, including Proteus syndrome, facial infiltrating lipomatosis, and macrodystrophia lipomatosa. The diagnosis is primarily based on clinical presentation and imaging modalities. However, for academic and supporting diagnostic purposes a biopsy is advised, yet may not be in the best interest of the patient. Management was conservative with future perspective for surgical management after cessation of growth.

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