Cases reported "Spinal Dysraphism"

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1/11. Infantile arachnoid cyst compressing the sacral nerve root associated with spina bifida and lipoma--case report.

    A 2-year-old boy presented with a rare sacral arachnoid cyst manifesting as gait disturbance. neuroimaging revealed an intradural cyst in the sacral nerve root sheath associated with spina bifida occulta and a lipoma at the same level. At surgery, the conus medullaris was situated at the L-1 level and not tethered. The highly pressurized arachnoid cyst had exposed the dural sheath of the left S-2 nerve root and compressed the adjacent nerves. An S-2 nerve root pierced through the cyst. There was no communication between the cyst and spinal arachnoid space. We thought the one-way valve mechanism had contributed to the cyst enlargement and the nerve compression. Radical resection of the cyst was not attempted. A cyst-subarachnoid shunt was placed to release the intracystic pressure. Postoperatively, his gait disturbance improved and no deterioration occurred during the 4-year follow up. Both tethered cord syndrome and sacral arachnoid cyst in the nerve root sheath should be considered in pediatric progressive gait disturbance. Cyst-subarachnoid shunt is an alternative method to cyst resection or fenestration to achieve neurological improvement.
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2/11. Unpredicted spontaneous extrusion of a renal calculus in an adult male with spina bifida and paraplegia: report of a misdiagnosis. Measures to be taken to reduce urological errors in spinal cord injury patients.

    BACKGROUND: A delay in diagnosis or a misdiagnosis may occur in patients with spinal cord injury (SCI) or spinal bifida as typical symptoms of a clinical condition may be absent because of their neurological impairment. CASE PRESENTATION: A 29-year old male, who was born with spina bifida and hydrocephalus, became unwell and developed a swelling and large red mark in his left loin eighteen months ago. pyonephrosis or perinephric abscess was suspected. X-ray of the abdomen showed left-sided staghorn calculus. Since ultrasound scan showed no features of pyonephrosis or perinephric abscess, he was prescribed a prolonged course of antibiotics for infection presumed to arise from the site of metal implant in spine. He developed a discharging sinus, following which the loin swelling and red mark subsided. About three months ago, he again developed a red mark and minimal swelling in the left loin. Ultrasound scan detected no abnormality in the renal or perinephric region. Therefore, the red mark and swelling were attributed to pressure from the backrest of his chair. Five weeks later, the swelling in the left loin burst open and a large stone was extruded spontaneously. An X-ray of the abdomen showed that he had extruded the central portion of the staghorn calculus from left kidney. With hindsight, the extruded renal calculus could be seen lying in the subcutaneous tissue of left loin lateral to the 10th rib in the X-ray of abdomen, which was taken when he presented with red mark and minimal swelling. CONCLUSION: This case illustrates how mistakes in diagnosis could occur in spinal cord injury patients, and highlights the need for corrective measures to reduce urological errors in these patients. Voluntary reporting of urological errors is recommended to facilitate learning from our mistakes. In the patients who have marked spinal curvature, ultrasonography of kidneys and perinephric region may not be entirely reliable. As clinical symptoms and signs may be non-specific in SCI patients, they require prompt, detailed and occasionally, repeated investigations. A joint team approach by health professionals belonging to various medical disciplines, which is strengthened by frequent, informal and honest discussions of a patient's clinical condition, is likely to reduce urological errors in SCI patients.
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3/11. Treatment of refractory intracranial hypertension in a spina bifida patient by a concurrent ventricular and cisterna magna-to-peritoneal shunt.

    CASE REPORT: A 20-year-old female born with a thoracic level myelomeningocele, Chiari II malformation, and hydrocephalus treated at birth developed clinical features of increased intracranial pressure (ICP) due to shunt malfunction. The patient became comatose. Her ICP remained high despite a functioning shunt and even after the ventricular catheter was exteriorized. diagnostic imaging consistently demonstrated slit-like ventricles, a Chiari II malformation, and a tethered spinal cord. We attributed her neurological condition either to brainstem compression or increased ICP related to venous outlet obstruction at the foramen magnum. OUTCOME: The patient improved rapidly after undergoing a Chiari II decompression and placement of a shunt from the cisterna magna and upper cervical subarachnoid space to the peritoneum connected by a "Y" connector to the ventricular catheter. CONCLUSION: The complex hydrocephalus was effectively treated by this concurrent ventricular and cisterna magna-to-peritoneum shunt.
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4/11. Non-invasive assessment of ventricular shunt function using tympanic membrane displacement measurement technique.

    A technique which was originally developed for measuring cochlear fluid pressure, has been under trial for the past two years as a means of assessing ventricular shunt function. The principle of the technique has been reported in previous papers (1, 2, 3) and has been shown to provide a reliable measure of intracranial pressure (ICP) in terms of induced displacement of the tympanic membrane. This study concentrates on the assessment of shunt blockage in spina bifida patients who subsequently underwent shunt revision. The tympanic displacement technique is shown to be of value to this patient group in three respects. Firstly as a research technique to study group-averaged ICP measurements where the ICP needs to be known but invasive measurements cannot be justified. Secondly for assessing shunt dysfunction in individual patients and, finally, in determining the success of shunt revision surgery. The technique was found to be extremely sensitive to relative changes in ICP with shunt revision or any subsequent blockage. The technique is more reliable diagnostically in instances where the patients act as their own controls and a comparison with baseline measurements can be made. Three case reports are illustrated which were selected from those patients tested to emphasise the importance of serial measurements of ICP over period of several days post-revision. The tympanic displacement technique is shown to provide a practical and acceptable method by which this can be undertaken.
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5/11. spinal dysraphism: a comprehensive diagnostic approach.

    Twelve patients with the clinical findings of spinal dysraphism form the basis for this report. In eight patients, physical findings, plain x-rays, and unenhanced spinal computed tomography allowed for precise diagnosis and subsequent therapy, without contrast myelography. All patients in the study had intravenous pyelography, and this revealed evidence of renal deterioration in two. The other 10 patients had radiographically normal upper urinary tracts. Urodynamic assessment was performed in seven; three were normal and four were abnormal. The pattern of the abnormal studies (three "flaccid type" with an adequate urethral pressure profile and one with detrusor-sphincter dyssynergia) allowed for appropriate therapy to be employed for bladder emptying and continence. Both spinal computed tomography and urodynamic testing serve as noninvasive studies that can be employed in the follow-up and management of patients with spinal dysraphism.
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6/11. Latex allergy anaphylaxis in a spina bifida patient with a pressure ulcer.

    At a Memphis, Tenn., children's hospital, latex-free surgery is mandated for patients with spina bifida or for those who have had multiple surgeries to correct congenital urinary anomalies. Even in the absence of a positive clinical history, both categories of patients are presumed to have a latex allergy or sensitivity. This case report illustrates the severe anaphylactic reaction that may occur with exposure to latex gloves or glove powder that transfers the antigen. The increasing incidence of latex rubber allergy for patients and health care workers is reviewed. Screening for patients at risk for allergy is outlined. These allergic patients can undergo safe operations if precautions are undertaken to avoid latex exposure. Precautions must also be initiated and enforced throughout the hospital for treatment of the patient in the pre- and post-operative period.
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7/11. When is a pressure ulcer not a pressure ulcer? ... external pressure versus internal pressure.

    This case study presents a nontraditional explanation for recurring and difficult to heal pressure ulcers: internal pressure rather than external pressure. The authors are hopeful that this discussion will assist colleagues in recognizing this alternative diagnosis.
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8/11. The "innocent" cough or sneeze: a harbinger of serious latex allergy in children during bladder stimulation and urodynamic testing.

    latex hypersensitivity is a well documented phenomenon most commonly reported in children with spina bifida during surgical and other procedures involving exposure to latex. IgE-mediated immediate hypersensitivity to the protein or polypeptide components of latex may be severe and manifest as generalized anaphylaxis or cardiovascular collapse. Of 17 children with spina bifida undergoing transurethral electrical bladder stimulation we identified 5 with latex allergy 3 to 9 years old. All 5 patients were noted to manifest sneezing or a cough several minutes before the development of a generalized hypersensitivity reaction, which in several patients progressed to bronchospasm. Subsequent investigations have shown that the inciting agent was the rectal pressure balloon made from a latex finger cot. Recognition of the earliest manifestations of latex hypersensitivity is an important clinical tool in the prevention of severe allergic reactions.
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9/11. Superglue sealant for persistent leakage of cerebrospinal fluid.

    A patient is presented in whom a persistent low-pressure CSF leak was stopped by using histoacryl tissue glue following initial local flap closure.
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10/11. Latex allergy in the operating room: case report and a brief review of the literature.

    A patient with a history of spina bifida and cerebral palsy was anesthetized for an ileal conduit. The procedure was uneventful until penetration of the abdominal cavity, immediately after which the patient suffered severe hypotension and her peak inspiratory pressure doubled. The patient's skin became flushed and the capnogram tracing was consistent with bronchospasm. The patient was treated with phenylephrine hydrochloride (Neo-synephrine), and then epinephrine. This was followed with an epinephrine infusion, intravenous (i.v.) methylprednisolone sodium succinate (Solu-Medrol), inhaled albuterol sulfate, and diphenhydramine hydrochloride (Benadryl) i.v.. The patient responded to these interventions and the procedure was completed without further incident. A diagnosis of latex allergy was made based on the patient's clinical status, underlying diseases, and positive postoperative radioallergosorbent test (RAST). Anesthesiologists must be able to diagnose the signs and symptoms of allergic reactions in patients under anesthesia. This article will review the types of hypersensitivity reactions involved, define the risk groups, and examine the treatment protocols available for latex allergy.
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