1/20. Segmental costovertebral malformations: association with neural tube defects. Report of 3 cases and review of the literature.patients with spondylocostal dysostosis (SCD) have vertebral abnormalities and numerical or structural rib anomalies that produce thoracic asymmetry. Rib anomalies and dysmorphism are the typical features that differentiate this syndrome from spondylothoracic dysostosis (STD). Jarcho-Levin syndrome is a severe form with involvement of the whole vertebral column. Other associated findings such as congenital heart defects, abdominal wall malformations, genitourinary malformations and upper limb anomalies may be found; in addition, neural tube defects (NTDs) have been associated with this malformation. SCD is transmitted both in a recessive form and as a dominant defect. We report on 3 children with SCD; 2 also had NTDs. All of them were studied with x-rays and spinal magnetic resonance (MR), and over the same period they underwent multidisciplinary clinical functional evaluation. One of our cases with NTD also presented polythelia, which has not previously been described in patients with SCD. The common association of segmental costovertebral malformations with NTDs could be related to an early gastrulation genomic defect, or one after gastrulation, when there are two independent somitic columns. The latter sometimes progresses and then involves primary and secondary neurulation. Also, the association of SCD with NTDs could be related to the interaction of different genes, resulting in this complex phenotype. Therefore, additional genetical and embryological studies are necessary to provide evidence of an etiological link between SCD and NTD.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
2/20. An unusual cause of incontinence.BACKGROUND: Incontinence is the most common urological symptom of tethered cord syndrome and may present as the earliest sign of this condition. The presence of unexplained incontinence with spinal anomalies, low back and leg symptoms, should raise the suspicion of tethered cord syndrome and lead to a lumbar MRI study, even though only subtle neurological abnormalities are found. OBJECTIVE: This case acts as a timely reminder of the need to be familiar with adult tethered cord syndrome so that an appropriate diagnosis is made promptly. DISCUSSION: The two groups of tethered cord syndrome, group 1 and group 2, are briefly defined; this paper discusses the presenting history of a patient in the first group.- - - - - - - - - - ranking = 338.78944469743keywords = incontinence (Clic here for more details about this article) |
3/20. Intramedullary neurenteric cyst with a false mural nodule: case report.OBJECTIVE AND IMPORTANCE: Spinal neurenteric cysts are rare congenital lesions that may occur either alone or in the context of a complex malformative disorder including typical vertebral and cutaneous abnormalities. The interest of the case of a spinal neurenteric cyst described here lies in its rare intramedullary location and in the false mural nodule image on the preoperative magnetic resonance imaging scan. A further distinctive feature is the association with a cleft spinal cord. CLINICAL PRESENTATION: A 28-year-old woman presented with a 2-year history of progressive paraparesis and urinary retention. A magnetic resonance imaging study disclosed a T8-T9 intramedullary cystic lesion with a mural nodulelike formation on the posterior face. INTERVENTION: A posterior midline myelotomy exposed a cystic lesion that had translucent walls and contained a milky fluid. No mural nodules were found. Once the cyst had been emptied, a collateral finding was a cleft that was clearly observed in the anterior spinal cord. The histological diagnosis was a neurenteric cyst. Retrospectively, the nodular lesion found on the preoperative scan was attributed to mucinous clots deposited at the bottom of the cyst. CONCLUSION: This case report demonstrates that neurenteric cysts can vary widely in radiological appearance, depending on the contents of the cyst. These differences become especially important if the associated stigmata are lacking and the preoperative diagnosis rests on magnetic resonance imaging scan appearance alone.- - - - - - - - - - ranking = 1keywords = urinary (Clic here for more details about this article) |
4/20. Evaluation of anorectal function in patients with tethered cord syndrome: saline enema test and fecoflowmetry.OBJECT: Disturbance in anorectal function is a major factor restricting the activities of daily living in patients with spinal cord disorders. To detect changes in anorectal motilities due to a tethered spinal cord, anorectal functions were evaluated using a saline enema test and fecoflowmetry before and after patients underwent untethering surgery. methods: The bowel functions in five patients with a tethered cord syndrome (TCS) were evaluated by performing a saline enema test and fecoflowmetry. The contractile activity of the rectum, the volume of infused saline tolerated in the rectum, anal canal pressure, and the ability to evacuate rectal content were examined. The characteristic findings in anorectal motility studies conducted in patients with TCS were a hyperactive rectum, diminished rectal saline-retention ability, and diminished maximal flow in saline evacuation. A hyperactive rectum was considered to be a major contributing factor to fecal incontinence. In one asymptomatic patient diminished anal squeezing pressure was exhibited and was incontinent to liquid preoperatively, but recovered after surgery. Two patients who underwent surgery for myeloschisis as infants complained of progressive fecal incontinence when they became adolescents. In one patient fecal incontinence improved but in another patient no improvement was observed after untethering surgery. CONCLUSIONS: Fecodynamic studies allow the detection of neurogenic disturbances of the anorectum in symptomatic and also in asymptomatic patients with TCS. More attention should be paid to the anorectal functions of patients with TCS.- - - - - - - - - - ranking = 203.27366681846keywords = incontinence (Clic here for more details about this article) |
5/20. Cervical meningocele in association with spinal abnormalities.CASE REPORT: This case report presents a newborn baby girl, who was diagnosed at birth with a mid-cervical meningocele. Further radiographic workup by MRI revealed co-existing thoracic diplomyelia and bilateral tethered cords. At birth the patient was found to be neurologically intact. Surgery was performed at 4 months of age, the patient undergoing simultaneous repair of the cervical meningocele, exploration of the diplomyelia, and release of the tethered cords bilaterally. Long-term follow-up revealed an ambulating patient with no bowel or bladder incontinence, who has developed well for her chronological age so far. review OF THE literature: A review of the literature relevant to this case is also presented.- - - - - - - - - - ranking = 67.757888939486keywords = incontinence (Clic here for more details about this article) |
6/20. muscle weakness and paresthesia associated with epidural analgesia in a patient with an intrathecal neurofibrolipoma as part of a tethered cord syndrome.We report a case of a 75-yr-old female patient in whom motor deficits and paresthesias occurred after lumbar epidural analgesia. These symptoms were eventually found to be due to a tethered cord syndrome. An epidural catheter was inserted for analgesia after colon surgery. The postoperative course was characterized by fluctuating sensory and motor symptoms. A magnetic resonance imaging scan showed an intraspinal mass, which was removed by laminectomy. The presented complication is of major interest because the intraspinal tumor, which must have been present for years, became acutely symptomatic. Tethered cord syndrome is caused by a limited longitudinal mobility of the cord. It is often seen as a part of spinal closure defects and is also associated with intrathecal tumors. Typically, adult patients complain of weak legs, paresthesias of the legs, and urinary incontinence. However, our patient had denied any muscular or neurological problems or urinary incontinence during the preoperative interview. Postoperative electromyogram and electroneurography ascertained chronic neurogenic lesions of multiple lumbar and sacral nerve roots. Three months after the operation, the patient was able to walk 100 m with a crutch.- - - - - - - - - - ranking = 756.19148798475keywords = urinary incontinence, incontinence, urinary (Clic here for more details about this article) |
7/20. Split cord malformation as a cause of tethered cord syndrome in a 78-Year-old female.A 78-year-old woman presented for evaluation of back pain, urinary dysfunction, leg weakness and progressive equinovarus foot deformity. She reported that shortly after her birth in 1924, she underwent resection of a subcutaneous 'cyst' in the lower lumbar area. Seven years prior to evaluation at our institution, she had undergone bilateral total knee arthroplasty for osteoarthritis. After the procedure, she began to experience severe low back pain that radiated into her legs. Weakness of the foot inverters, urinary dysfunction and worsening bilateral equinovarus foot deformity developed in the years following the surgery. MRI revealed a split cord malformation with a tethered spinal cord. Because of the patient's age and poor medical condition, her symptoms were managed conservatively. This case demonstrates symptomatic deterioration in an elderly patient with a tethered spinal cord after many years of clinical stability.- - - - - - - - - - ranking = 2keywords = urinary (Clic here for more details about this article) |
8/20. Midline defects in FG syndrome: does tethered spinal cord contribute to the phenotype?OBJECTIVES: FG syndrome is an X-linked recessive mental retardation syndrome with ano-rectal anomalies, constipation, and occasional urinary incontinence. Because tethered spinal cord syndrome (TCS) has similar symptoms, we evaluated imaging for TCS in patients with FG syndrome. STUDY DESIGN: patients were recruited from the International FG syndrome Support Group, and an FG syndrome consensus Group reviewed clinical histories, medical records, and photographs of each responding patient. Results of cranial and spinal imaging studies were available for 12 patients. RESULTS: Of 12 boys with FG syndrome, 6 had hypoplasia of the corpus callosum, and 3 of these had TCS (all with constipation and urinary symptoms). The other 9 did not have urinary symptoms. After surgical untethering, bowel and bladder symptoms improved. CONCLUSIONS: Tethered spinal cord syndrome occurred in 25% of patients with FG syndrome associated with hypoplasia of the corpus callosum and causing bowel and bladder incontinence. A high index of suspicion is necessary for early diagnosis, and timely intervention results in significant improvement in symptomatology.- - - - - - - - - - ranking = 447.85363293186keywords = urinary incontinence, incontinence, urinary (Clic here for more details about this article) |
9/20. Diffuse back pain and urological symptoms: recognizing tethered cord syndrome early.Tethered cord syndrome (TCS) is a rare entity in adults, with only about 100 cases being reported in the literature. It is a syndrome in which patients have neurologic, urologic, and orthopedic signs and symptoms. We present a case of a 40-year-old man with back pain and urinary incontinence who was diagnosed with tethered cord syndrome. After the cord de-tethering in the operating room, his pain improved. TCS is an important condition to consider in the differential diagnosis of an adult who presents with back pain and urological symptoms. Imaging studies should be done, and operative treatment may be required to alleviate the symptoms. TCS should be in the differential diagnosis of any patient who presents with back and lower extremity complaints with superimposed urological problems. If TCS is confirmed radiologically by MRI, surgery can be performed with reasonable results provided that the disease process has not progressed.- - - - - - - - - - ranking = 378.09574399238keywords = urinary incontinence, incontinence, urinary (Clic here for more details about this article) |
10/20. Transient ventriculoperitoneal shunt dysfunction in children with myelodysplasia and urinary bladder infection. Report of three cases.The authors present three children born with myelomeningocele and hydrocephalus. Each presented with symptoms/signs of ventriculoperitoneal shunt malfunction. All patients at the time of presentation exhibited significant urinary bladder infections and were appropriately treated for their infection. No patient was found to have an underlying shunt infection. All patients without medically threatening symptoms were carefully observed and noted to have resolution of their shunt dysfunction symptoms/signs following treatment of their urinary bladder infections and thus did not undergo a shunt operation. Based on the courses of these patients, we believe that significant urinary bladder infection in patients with myelodysplasia in whom a shunt has been placed may often be enough to bring a subclinical shunt malfunction to clinical attention or even to be the cause of temporary distal peritoneal shunt malabsorption. Although the exact mechanism for this dysfunction is unclear, treatment of the bladder infection may address the symptomatic shunt dysfunction in some patients so as to avoid operative intervention. We emphasize, however, that careful observation of these patients should be performed during hospitalization because they often rely on adequate cerebrospinal fluid diversion. Only patients with mild symptomatology should be observed first as the initial line of treatment.- - - - - - - - - - ranking = 7keywords = urinary (Clic here for more details about this article) |
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