Cases reported "Meningomyelocele"

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1/14. Clinical features in patients requiring reoperation after failed endoscopic procedures for hydrocephalus.

    The aim of this study was to clarify the clinical features of patients at risk of secondary obstruction following endoscopic fenestration. Clinical notes and endoscopic findings for 15 patients treated with endoscopic procedures were retrospectively reviewed. Endoscopic third ventriculostomy (ETV) was performed as initial treatment in 4 patients with non-communicating hydrocephalus, including a neonate with myelomeningocele, and as an alternative to shunt revision in 4 patients. Two patients with non-communicating hydrocephalus caused by tumor or arachnoid cyst were also managed with third ventriculostomy. Four patients with loculated hydrocephalus underwent endoscopic septostomy. A child with an isolated fourth ventricle was treated with endoscopic aqueductoplasty. Of the 15 patients undergoing endoscopic procedure, 4 required reoperation. Of the 10 patients treated with ETV, only the neonate with myelomeningocele required a ventriculoperitoneal shunt because of failure of the initial procedure. Of the 4 patients treated with endoscopic septostomy, 2 children with loculated hydrocephalus following intraventricular hemorrhage (IVH) underwent a second septostomy. In a patient with an isolated fourth ventricle following posthemorrhagic hydrocephalus, recurrence was noted 8 months after the initial procedure. He underwent a second procedure using a stent implanted into the aqueduct to maintain CSF circulation. Sufficient stomal size or implantation of a stent may be required in the under-2-year age group with hydrocephalus accompanied by IVH and associated with myelomeningocele, in whom the risk of secondary obstruction may be high.
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2/14. Bilateral split latissimus dorsi V-Y flaps for closure of large thoracolumbar meningomyelocele defects.

    Closure of large meningomyelocele wounds and defects always requires durable and safe coverage of the dural repair. A new technical method for the reconstruction of large thoracolumbar meningomyelocele defects is described in which bilateral musculocutaneous flaps are advanced and transposed medially in a V-Y sliding manner, based on the thoracolumbar perforatiors of the latissimus dorsi. This procedure provides a reliable, well-vascularized soft tissue coverage over the neural repair with minimum donor-site morbidity. Additionally, this method is particularly appropriate to the thoracolumbar area, as it preserves the lateral adjacent regions of the defect, for later alternative and/or reconstructive options.
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3/14. Unusual findings during abdominal placement of a ventriculoperitoneal shunt: report of three cases.

    The authors present three cases of infants born with myelodysplasia. Each infant underwent closure of a myelomeningocele and within 2 to 4 days placement of a ventriculoperitoneal (VP) shunt. In each case, on opening the peritoneal cavity, the authors observed egress of a dark or creamy dark fluid. None of the patients had a history of abdominal birth trauma. The decision was made to continue the procedures and send samples of the unusual fluids to the laboratory for culture and analysis. The cultures proved to be nondiagnostic and the characteristics of the fluid samples were most consistent with those of blood-tinged chyle. The authors hypothesize that, occasionally, the mechanical tautness that is created with repair of myelomeningoceles is sufficient to rupture small lymphatic vessels and accompanying blood vessels of the abdomen. An alternative hypothesis is that abdominal compression due to closure of the myelomeningocele may temporarily compress the liver, leading to raised intraportal pressures and resulting in weeping of chyle from the gastrointestinal tract. This abnormal fluid accumulation did not lead to chronic ascites, VP shunt infection, or dysfunction at long-term follow-up examination and abdominal visceral function has not been an issue.
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4/14. Use of allogeneic skin graft for the closure of large meningomyeloceles: technical case report.

    OBJECTIVE AND IMPORTANCE: To describe an alternative approach to the operative management of large meningomyeloceles, in which primary closure is not possible. CLINICAL PRESENTATION: Two full-term infants presented with very large meningomyeloceles for closure after an uncomplicated delivery. TECHNIQUE: An allogeneic skin graft was used in both cases to approximate the skin defect that remained after removal of the large meningomyelocele sacs. The surgical site was treated with dressing changes only during the postoperative period. CONCLUSION: The independent use of allogeneic skin grafts can lead to a successful cosmetic result for the treatment of large meningomyeloceles without the use of complicated skin incisions or flap advancements.
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5/14. The manchester-fothergill procedure as a fertility sparing alternative for pelvic organ prolapse in young women.

    Although reproductive organ prolapse typically affects older, parous women, a certain population of children and adolescents, primarily adolescents with congenital spinal defects, are also at risk. The Manchester-Fothergill procedure was first performed in 1888 by Dr. Archibald Donald of Manchester, england. Although this is a well-described procedure in historic texts, the practical application is not often employed, and modern surgical texts rarely describe how to perform this surgery. It is crucial to educate younger physicians about the technical aspects of this procedure so as not to lose this operation as an alternative surgical approach. We present a case of recurrent uterine prolapse after a prior laparoscopic uterine suspension procedure. In order to address recurrent prolapse, which was accompanied by cervical elongation, this young woman elected to have additional surgical management with the Manchester-Fothergill procedure. We describe the procedure in detail, accompanied by photographs taken during the operation to fully illustrate the extent of this patient's condition and to document the surgical technique. The Manchester Fothergill procedure is an additional fertility-sparing surgical approach to treatment of pelvic organ prolapse.
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6/14. Gracilis muscle transposition for anal incontinence in children: long-term follow-up.

    We report a series of 7 patients, aged 6.5 to 19 years (mean, 12.9), who have been treated for uncontrollable fecal incontinence since 1976: 5 had imperforate anus and multiple subsequent operative procedures, 1 had a low myelomeningocele with bi-sphincteric incontinence, and 1 had a traumatic destruction of the sphincter apparatus. A modified Pickrell procedure was performed, with the gracilis muscle transposed subcutaneously, without constructing a pulley through the median raphe as originally described. All patients were evaluated by anorectal manometry preoperatively and post-operatively. They were followed-up for a period of 0.5 to 12.5 years (mean, 4.4). All patients were continent at follow-up, with a normal defecation pattern and no enemas required. None of the patients had evidence of fibrosis of the muscle or anal canal, and tension in the transposed muscle was maintained. Voluntary contractions remain efficient in all cases. Age was thought to be an important factor: personal motivation and compliance with physiotherapy, essential for a good outcome, is unlikely to be present in the younger child. We conclude that the gracilis sling procedure is an excellent long-term alternative for total fecal incontinence when time and other therapeutic measures have failed.
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7/14. Behavioral methods for teaching self-catheterization skills to anxious children with myelomeningocele.

    A behavior-based protocol was developed which outlined all necessary behaviors for successful self-catheterization to an anxious child with myelomeningocele. The subject had been wearing diapers daily since birth, and he expressed fear of pain and lacked confidence because of previous unsuccessful attempts. The present protocol included progressive muscle relaxation, guided visual imagery, and behavioral rehearsal that was implemented by his parents. Data collected showed daily successful intermittent self-catheterization skills two weeks and 6 months posttreatment. Thus, an alternative to traditional educational methods exists that can successfully enhance the acquisition of self-catheterization skills.
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8/14. meningomyelocele: a new or a vanishing disease?

    I believe meningomyelocele is not vanishing, but is a new and diminishing disorder. prenatal diagnosis will be accepted only by a portion of most communities if abortion is the only alternative to the delivery of an impaired child. I believe participation in neural tube screening programmes can be increased by offering improved pregnancy outcome with concomitant prelabor caesarean section in addition to termination. Regardless, the prevalence at birth of children with myelomeningocele will decrease both in total numbers and in the severity of the expressed lesion. The resultant rarity will require collaboration between centers to evaluate treatment. The developing International Myelodysplasia Study Group using a Patient Data Management System and computer-assisted analysis is a model of successful collaboration that allows better exploration of the multiple variables that contribute to the well-being of children with open neural tube defects of the spine.
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9/14. Fascial sling to correct male neurogenic sphincter incompetence: the McGuire/Raz approach.

    We report the surgical approach used in 4 male myelomeningocele patients suffering from severe urinary incontinence owing to poor bladder compliance and sphincter incompetence. While bladder compliance was corrected by enlargement cystoplasty the sphincteric incompetence was treated by an autologous fascial sling around the bladder neck with excellent results. We consider this operation as an alternative to the artificial urinary sphincter in cases of male neurogenic sphincter incontinence.
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10/14. Intermittent clean catheterization: an alternative to diversion in continent transplant recipients with lower urinary tract dysfunction.

    A total of 3 renal transplant recipients who were candidates for urinary diversion underwent successful transplantation using a planned program of intermittent clean catheterization. The urinary tract dysfunction was caused by a lower motor neuron neurogenic bladder, prune belly syndrome and myelodysplasia. The patients remain dry between catheterizations and maintain serum creatinine levels of 1.1, 0.8 and 0.5 mg. per cent, respectively, with a followup of 6 to 25 months. There has been only 1 urinary tract infection during 42 patient-months at risk while on self-catheterization. Pre-transplant urologic evaluation and patient education are mandatory. The ideal candidate for intermittent clean catheterization is a patient with a low pressure bladder that fails to empty and who is continent between catheterizations. Intermittent clean catheterization is a safe and effective alternative to diversion in continent transplant recipients with lower urinary tract dysfunction.
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