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Diastematomyelia

Diastematomyelias


Presentation

  • A summary of the management of a 54-year-old woman with recent clinical symptomatology related to an undiagnosed split-cord malformation is presented with accompanying literature review.[ncbi.nlm.nih.gov]
  • The clinical pattern could be seen in the general presentation of 'tethered cord' and there are 2 types of presentation with a different treatment. In our case report the two dominant complaints were pain in the right foot and sphincter problems.[ncbi.nlm.nih.gov]
  • To the best of our knowledge, this is the earliest case presented in the literature.[ncbi.nlm.nih.gov]
  • Both patients presented complete recovery in the follow-up. From the review of 15 adults described in literature including the present cases, the age ranged between 11 and 87 proving an open rage of onset of symptoms.[ncbi.nlm.nih.gov]
  • The third case, detected at 22 weeks' gestation, presented with widening of the lumbar canal and scoliosis.[ncbi.nlm.nih.gov]
Turkish
  • This paper presents a 12-year-old Turkish boy with diastematomyelia who was suspected with a demonstrative dermatologic finding without any neurologic sign and diagnosed with magnetic resonance imaging (MRI).[ncbi.nlm.nih.gov]
  • The Turkish journal of pediatrics. 1988 Dec;31(1):89-94.[pakjr.com]
Hyperpigmentation
  • Both patients exhibited associated congenital abnormalities of the neuroectoderm in the form of hyperpigmentation, hypertrichosis, lipomatosis, and dermal sinus that were demonstrated with clarity by the neuroimaging modalities used.[ncbi.nlm.nih.gov]
  • In more than 70% of patients, a localized area of hyperpigmentation and/or hypertrichosis can be found in the skin area directly overlying the defect. The symptomatology varies from asymptomatic to significant neurologic and skeletal abnormalities.[accessanesthesiology.mhmedical.com]
Cutaneous Manifestation
  • Early recognition of these cutaneous manifestations by the plastic surgeon, evaluation with an MRI scan, and consultation with a pediatric neurosurgeon can prevent delayed diagnosis of this surgically correctable anomaly and irreversible neurologic sequelae[ncbi.nlm.nih.gov]
  • The diagnosis is dependent on presentation of orthopedic syndromes, cutaneous manifestations, neurological deficits, congenital scoliosis and spina bifida in X-ray examination, a detailed evaluation of the spinal cord by myelography, CT myelography and[ci.nii.ac.jp]
Neurogenic Claudication
  • The authors describe a 42-year-old man who presented with pain and distal left-leg weakness as well as neurogenic claudication for 1 month.[ncbi.nlm.nih.gov]
  • Kaminker, R, Fabry, J: Split cord malformation with diastematomyelia presenting as neurogenic claudication in an adult . Spine 25: 2269 – 2271 , 2000 . Google Scholar 15.[journals.sagepub.com]
Lower Limb Muscle Weakness
  • A 44-year old woman, presented with progressive lower limb muscle weakness, gait disturbances and dysesthesias in the trunk and lower extremity.[ncbi.nlm.nih.gov]
Denial
  • For a patient with a congenital spine deformity, a diastematomyelia, a tight filum terminale, and a congenital stenosis, denial of magnetic resonance imaging evaluation and appropriate surgery cannot be justified.[ncbi.nlm.nih.gov]
Meningism
  • However, another episode of meningitis occurred after surgery, and a subsequent temporal bone scan revealed the presence of left Mondini dysplasia.[ncbi.nlm.nih.gov]
  • Abstract Accessory human tails are uncommon malformations consisting of meningeal and neural structures and possibly occult spinal dysraphism (pseudotails). The thoracic location of a lipomeningocele constituting a pseudotail is very rare.[ncbi.nlm.nih.gov]
  • Each half of the neural axis was invested with its own meninges.[pediatrics.aappublications.org]
Hyperreflexia
  • Sensorimotor deficits, hyperreflexia, and a positive Babinski reflex in the right lower extremity were demonstrated on examination.[ncbi.nlm.nih.gov]
Dysuria
  • A 26-year-old primigravida at 14 weeks' gestation was presented to our outpatient department with a complaint of dysuria.[ncbi.nlm.nih.gov]

Workup

  • Her initial workup revealed a lumbosacral dermoid sinus associated with diastematomyelia and a tethered cord. Therefore, a surgical repair to correct the anomaly was performed.[ncbi.nlm.nih.gov]
  • An F-NaF bone PET done as part of the metastatic workup showed the characteristic sagittal bone spur, and MRI demonstrated a complete split of the cervical and upper thoracic spinal cord, previously unknown to the patient.[ncbi.nlm.nih.gov]
  • Although cases of diastematomyelia have been previously reported, fully documented approaches by both prenatal and postnatal diagnostic workup are rare in the literature.[ncbi.nlm.nih.gov]
  • This case demonstrates that a thorough preoperative workup of patients with complex spinal deformities is imperative.[ncbi.nlm.nih.gov]

Treatment

  • Surgical resection of the septum was one of the possibilities, but because of the limited clinical impact, a conservative treatment was followed.[ncbi.nlm.nih.gov]
  • The treatment should be dependent on local pain intensity (which is often not directly associated with the disorder) and on the degree of neurological dysfunction. 5. 1.[ncbi.nlm.nih.gov]
  • CONCLUSION: Surgical treatment is the necessary management for type I diastematomyelia causing progressive neurological deterioration or with tethered filum, whereas conservative treatment is recommended to asymptomatic type I diastematomyelia and all[ncbi.nlm.nih.gov]
  • The cases illustrate that widening of the posterior ossification centres can occur in the absence of overt spina bifida and the prenatal detection of diastematomyelia will allow for early postnatal investigation and treatment.[ncbi.nlm.nih.gov]
  • What Are Some Common Treatments? Treatment does not aim to "reattach" the halves of the split spinal cord. However, treatment varies depending on the symptoms and severity of the malformation.[nervous-system-diseases.com]

Prognosis

  • We present eight cases of diastematomyelia diagnosed prenatally in our institution as well as a review of the literature in order to determine the prognosis of isolated cases of this very unusual condition.[ncbi.nlm.nih.gov]
  • Prenatal diagnosis of this anomaly is possible in the early mid-trimester by sonography, thus allowing for early surgical intervention and a favorable prognosis.[ncbi.nlm.nih.gov]
  • Prenatal ultrasonography contributes to the diagnosis of diastematomyelia and provides a basis for prenatal counseling and prognosis.[ncbi.nlm.nih.gov]
  • The family was counseled regarding the prognosis for the fetus. Their decision to terminate the pregnancy was considered and approved by the ethical council.[ncbi.nlm.nih.gov]
  • Prenatal diagnosis of this anomaly is possible in the early midtrimester by sonography, thus allowing for early surgical intervention and a favorable prognosis.[ncbi.nlm.nih.gov]

Etiology

  • They are organised into groups, and further divided into clinical, etiological or histopathological sub-types.[orpha.net]
  • He also provides updated information about the etiology, evaluation, and treatment of this interesting group of disorders.[medlink.com]
  • The precise etiology is not known.[nature.com]

Epidemiology

  • Introduction A fibrous, cartilagenous, or osseous bar creating a longitudinal cleft in the spinal cord if the cord does not reunite distally to the spur, it is considered a diplomyelia (true duplication of the cord) Epidemiology demographics typically[orthobullets.com]
  • Neurology and Neurosurgery Czech and Slovak Ophthalmology Czech and Slovak Psychiatry Czech Geriatric Review Czech Gynaecology Czech Rheumatology Czech Dental Journal Czech Urology Czech-Slovak Dermatology Czecho-Slovak Pathology Czech-Slovak Pediatrics Epidemiology[prolekare.cz]
  • American Journal of Epidemiology 153: 961 – 968 , 2002 . Google Scholar 11. Hughes, L, McCarthy, R: Segmental spinal dysgenesis: A report of three cases. J Pediatr . Orthop 18: 227 – 232 , 1998 . Google Scholar 12.[journals.sagepub.com]
  • Epidemiological studies have demonstrated multifactorial influences underlying NTDs. Variations in incidence have been seen across geographical variation, ethnic groups, socioeconomic status, and genetic predisposition.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Various precipitating factors have been implicated in their pathophysiology including trauma, spinal canal compression, and strong stretching factors. All these factors contribute to a neurologic deficit in an already compromised neural canal.[ajronline.org]
  • Pathophysiology [ edit ] Diastematomyelia is a " dysraphic state " of unknown embryonic origin, but is probably initiated by an accessory neurenteric canal (an additional embryonic spinal canal.)[en.wikipedia.org]

Prevention

  • This technique may be useful to prevent ventral retethering in diastematomyelia.[ncbi.nlm.nih.gov]
  • Early recognition of these cutaneous manifestations by the plastic surgeon, evaluation with an MRI scan, and consultation with a pediatric neurosurgeon can prevent delayed diagnosis of this surgically correctable anomaly and irreversible neurologic sequelae[ncbi.nlm.nih.gov]
  • In such patients, it may be prudent to avoid central neuraxial block to prevent neural damage.[ncbi.nlm.nih.gov]
  • Closure of the myeloschisis and removal of the septum were successfully accomplished in one stage to prevent subsequent infection and neurological deterioration.[ncbi.nlm.nih.gov]
  • In that way a later onset of neurological signs, due to the "tethered cord syndrome", was prevented. Six months following surgery the child's condition was still unchanged.[ncbi.nlm.nih.gov]

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