scholarly journals Refractory CSF leakage following untethering surgery performed 10 months after birth for enlarging terminal myelocystocele associated with OEIS complex

2021 ◽  
Vol 12 ◽  
pp. 628
Author(s):  
Nobuya Murakami ◽  
Ai Kurogi ◽  
Yoshihisa Kawakami ◽  
Yushi Noguchi ◽  
Makoto Hayashida ◽  
...  

Background: Terminal myelocystocele (TMC) is an occult spinal dysraphism characterized by cystic dilatation of the terminal spinal cord in the shape of a trumpet (myelocystocele) filled with cerebrospinal fluid (CSF), which herniates into the extraspinal subcutaneous region. The extraspinal CSF-filled portion of the TMC, consisting of the myelocystocele and the surrounding subarachnoid space, may progressively enlarge, leading to neurological deterioration, and early untethering surgery is recommended. Case Description: We report a case of a patient with TMC associated with OEIS complex consisting of omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I), and spinal deformity (S). The untethering surgery for TMC had to be deferred until 10 months after birth because of the delayed healing of the giant omphalocele and the respiration instability due to hypoplastic thorax and increased intra-abdominal pressure. The TMC, predominantly the surrounding subarachnoid space, enlarged during the waiting period, resulting in the expansion of the caudal part of the dural sac. Although untethering surgery for the TMC was uneventfully performed with conventional duraplasty, postoperative CSF leakage occurred, and it took three surgical interventions to repair it. External CSF drainage, reduction of the size of the caudal part of the dural sac and use of gluteus muscle flaps and collagen matrix worked together for the CSF leakage. Conclusion: Preoperative enlargement of the TMC, together with the surrounding subarachnoid space, can cause the refractory CSF leakage after untethering surgery because the expanded dural sac possibly increases its own tensile strength and impedes healing of the duraplasty. Early untethering surgery is recommended after recovery from the life-threatening conditions associated with OEIS complex.

Author(s):  
Neetu Kumar ◽  
Chinky Chatur ◽  
Ankit Balani ◽  
May Bisharat ◽  
Zubair Tahir ◽  
...  

OBJECTIVE The objective of this study was to assess the prevalence and spectrum of spinal dysraphism in a cohort of children with cloacal exstrophy (CEX) using MRI. METHODS Children with CEX presenting between 1999 and 2019 with baseline spinal MRI were included. The images were reviewed in consensus to assess the type of dysraphism. The dysraphisms were initially reviewed and described based on their descriptive anatomy, and then classified according to anomalies of gastrulation, primary neurulation, or secondary neurulation. RESULTS Thirty-four children were included. Thirty-three of these children had closed spinal dysraphism, and 1 had a normal spine. Of the 33 cases of closed spinal dysraphism, the conus and/or filum terminale were involved in all cases. The most common malformations were spinal lipoma (n = 20) and terminal myelocystocele (n = 11). The lipomas were heterogeneous: 4 dorsal, 9 transitional, 4 chaotic, and 3 terminal. A large subgroup (10/20, 50%) within the lipomas had an unusual morphology of noncontiguous double lipomas, the proximal fat related to the conus and the distal fat within the filum. These were difficult to characterize using existing classifications. In 2 cases, only a thickened filum was noted. The majority of these malformations were compatible with a disorder of secondary neurulation. CONCLUSIONS Complex spinal dysraphisms are consistently associated with CEX. The unusual dysraphism patterns found in this group of patients highlight the limitations of current embryological classifications. Given the propensity for neurological deterioration in this group of patients, spinal MRI should be routinely performed. The type and distribution of malformations seen have implications for the wider understanding of the pathogenesis and classification of lumbosacral lipomas.


2010 ◽  
Vol 13 (5) ◽  
pp. 659-661 ◽  
Author(s):  
Nicola Alberio ◽  
Luigi Pentimalli ◽  
Raffaele Alessandrello ◽  
Rita Lipani ◽  
Marco Maiello ◽  
...  

The authors describe the case of a 47-year-old woman with a wide (14 × 12–cm) ulcerated lumbosacral myelomeningocele. The patient had sought medical attention for a sudden copious CSF leak from the lumbosacral sac followed by clinical signs of CSF leakage. After admission, neuroradiological assessment (spinal MR and 3D CT imaging) revealed the uncommon finding of a complex malformation characterized by a complete spine duplication originating at the L2–3 level, both hemicords having a separate dural sac. The myelomeningocele sac originated medially at the L-2 level. Surgical repair of the lumbosacral myelomeningocele was performed. The placement of a ventriculoperitoneal shunt became necessary to treat secondary hydrocephalus. After reviewing accredited classifications on spinal cord malformations, the authors believe that, to date, complete duplication and separation of the spine and dural sac seems exceptional, and its report in adulthood appears exceedingly rare.


2020 ◽  
Author(s):  
denglu yan ◽  
Zaiheng Zhang ◽  
Zhi Zhang

Abstract Background There are many factors that affect the C5 palsy in the treatment of cervical spondylosis myelopathy (CSM) with ossification of the posterior longitudinal ligament (OPLL), but the spinal compression degrees may be an important factor in affection on it. Objective The purpose of this study was to evaluate the effect of spinal cord compression degrees on the clinical outcomes of OPLL patients which been treated by anterior and posterior surgery. Methods From May 2010 to October 2017, 145 patients cervical spondylosis myelopathy with OPLL (spinal canal narrowing by the OPLL exceeded 30%) were enrolled in this study; 76 patients received anterior cervical corpectomy and fusion surgery (ACCF), and 69 patients had posterior cervical decompression and laminoplasty procedures (PCDL). The clinical outcomes of pain relief (visual analog scale, VAS), functional disability (neck disability index, NDI) were recorded at baseline and at the final follow-up. Results There were five patients with C5 palsy in this series cases (two in ACCF and three in PCDL), and the rate is 4.1% (3.2% in ACCF and 5.2% in PCDL, P > 0.05). Five C5 palsy cases had more than 50% compressed degrees of dural sac and flattened spinal cord, and the compressed dural sac and flattened spinal cord had totally expansion after operation. There were no patients resented with CSF leakage in PCDL group, and six patients presented with CSF leakage in ACCF. The mean postoperative canal diameter were significant better than preoperative in PDL patients (P < 0.01). The average spinal sagittal and axial compressive ratio were significantly decreased from preoperative in ACR and PDL groups (P < 0.01). The mean cervical ROM values were decreased in ACR and PDL groups (P< 0.05). Conclusions Based on the results of this study, anterior and posterior surgery was effective and safe in the treatment of the OPLL patients. The complication of C5 palsy were no related in the surgery methods, but may be related the degrees of spinal cord compressed.


2021 ◽  
Author(s):  
Isabela Turino Lougon ◽  
Taciana Maria Rolim Sampaio Oliveira ◽  
Yan Ribeiro Francisco

Introduction: Lipomyelomeningocele is a congenital malformation in which the neural plate lies outside the spinal canal. Patients younger than 6 months of age are neurologically asymptomatic. However, if not diagnosed, the disease evolves with deterioration of bowel and bladder functions and, later, with motor deficits (paralysis) and loss of sensation. Accordingly, a literature review was developed about the therapeutic methods that enable a better quality of life and prevent or control disease progression. Objectives: This literature review aims to elucidate leading updates in the literature regarding the therapy for lipomyelomeningocele, a rare form of spinal dysraphism. Methods: Searches were performed in the LILACS e PubMed database and in the health descriptors (DeCs/MeSH), to establish the descriptors. Of the 6,292 articles found, 20 were selected, since only they presented relevant information about this topic. Results: From reading the articles, it was concluded that surgery is the recommended treatment for cases of lipomyelomeningocele. The main goal of surgery is to prevent future damage or further neurological deterioration and also to preserve or improve current neurological function. The widespread use of advanced prenatal ultrasonography can show neural development and suggest congenital malformations and progressive neurological deterioration. Conclusions: Although surgery is the best treatment, it may also be accompanied by outcomes such as meningitis, CSF leakage, neurological deterioration as a result of tethered cord or secondary to nerve injury, and incomplete healing or wound breakdown. Prenatal ultrasound is necessary to identify the malformation and support early diagnosis of lipomyelomeningocele and may open doors for future care and treatment.


2009 ◽  
Vol 11 (6) ◽  
pp. 764-767 ◽  
Author(s):  
Besnik Nurboja ◽  
Kia Rezajooi ◽  
Mary C. Newton ◽  
Adrian T. H. Casey

Patients undergoing epidural injection for labor pains occasionally sustain iatrogenic inadvertent puncture of the dura with or without damage to the underlying neurological structures. This may be associated with CSF leakage, headache, neurological deficit, and infection. Rarely, the headache persists for years. To the authors' knowledge, chronic headache due to acquired spinal meningocele featuring as a duplicated dural sac, as a sequela of traumatic inadvertent dural puncture, has not been previously reported. The authors report a case of a 20-year-old woman with persistent headaches following an epidural injection. Five years later, the persistent headache was found to be due to a large acquired spinal meningocele. The operative removal of the meningocele led to resolution of headaches. This report highlights the importance of considering a spinal condition as a culprit for chronic headache and postulates a mechanism for the formation of the acquired spinal meningocele appearing as a duplicated dural sac. The authors recommend early MR imaging of the spine for any persisting headache that has a history of attempted spinal access. If an acquired spinal meningocele collection is found, exploration with a view to complete removal of the sac should be considered. To the authors' knowledge, this is the first case report depicting a rare, treatable cause of chronic spinal hypotension resulting in headaches.


2018 ◽  
Vol 35 (2) ◽  
pp. 227-231 ◽  
Author(s):  
Giorgia Totonelli ◽  
Raffaella Messina ◽  
Francesco Morini ◽  
Giovanni Mosiello ◽  
Chiara Iacusso ◽  
...  

1978 ◽  
Vol 48 (2) ◽  
pp. 252-258 ◽  
Author(s):  
Joseph A. Epstein ◽  
Bernard S. Epstein ◽  
Leroy S. Lavine ◽  
Alan D. Rosenthal ◽  
Robert E. Decker ◽  
...  

✓ The authors report five patients with spinal stenosis who had a total myelographic block at the level of the obliterated subarachnoid space. Arachnoiditis had not been considered as a primary diagnosis until laminectomy revealed a non-pulsating, thickened dural sac that conformed to the internal configuration of the involved spinal canal. Two patients had stenosis complicated by spondyloarthrosis over multiple lumbar levels, one had a previous spinal fusion, another had degenerative spondylolisthesis, and the fifth had a large midline extruded disc at L2–3 that completely blocked the spinal canal. The dura was opened in two patients, confirming the lesion. Despite obliteration of the subarachnoid space, significant relief for approximately 1 year followed decompressive laminectomy, foraminotomy, and discectomy, with disappearance of neurogenic claudication in three patients. Postoperative erect films showed no caudad passage of contrast. While further observations are required, an awareness of this complication of spinal stenosis is important in the diagnosis and management of such patients and in evaluating their ultimate prognosis.


Neurosurgery ◽  
1991 ◽  
Vol 28 (6) ◽  
pp. 834-843 ◽  
Author(s):  
Alan R. Cohen

Abstract Five infants with cloacal exstrophy underwent neurological evaluation and radiographic examination of the caudal spine shortly after birth. Each was found to have occult spinal dysraphism. Four had terminal myelocystoceles, and one had a lipomyelomeningocele. Pathological anatomy was confirmed during surgery for the release of the tethered spinal cords. The striking association between cloacal exstrophy and occult spinal dysraphism suggests a common developmental defect in the caudal pole of the embryo. A hypothesis is offered to explain this association. Terminal myelocystocele and lipomyelomeningocele appear to be part of a continuum of lesions associated with skin-covered spina bifida.


2017 ◽  
Vol 16 (3) ◽  
pp. 213-219
Author(s):  
ALEXEY KASHCHEEV ◽  
SERGEY ARESTOV ◽  
ARTEM GUSHCHA

ABSTRACT Objetive: Thecaloscopy is a less invasive method of exploration of the spinal subarachnoid space, using an ultra-thin, flexible endoscope and endoscopic fenestration of scars and adhesions. Thecalopscopy was used in Russian neurosurgery for the first time. Methods: Since 2009, we have operated on 32 patients with the following diagnoses: 17 - spinal adhesive arachnoiditis (8 - local forms, 9 - diffuse forms), 12 - spinal arachnoid cysts (7 - post-traumatic cysts, 5 - idiopathic cysts), and 3 - extramedullary tumors (thecaloscopic videoassistance and biopsy). In all cases, we performed exploration of subarachnoid space and pathologic lesion with endoscopic perforation of the cyst or dissection of adhesions using special instrumentation. The mean follow-up time in our group was 11.4 months. Results: Neurological improvement (mean 1.4 by the modified Frankel scale, 1.8 by the Ashworth spasticity scale) was seen in 87% of patients operated for spinal arachnopathies. Temporary neurological deterioration (mild disturbances of deep sensitivity) was seen in 9% of patients and managed successfully with conservative treatment. One patient (3.1%) was operated three times due to relapse of adhesions. There were no serious intraoperative complications (e.g. severe bleeding or dura perforation). Postoperative complications included one CSF leakage and one case of postoperative neuralgic pain. The mean hospitalization time was 7.6 days. Conclusion: According to our data, we conclude that thecaloscopy is efficient and safe method, and should be widely used for spinal arachnopathies, adhesive arachnoiditis and arachnoid cysts. Taking into account that adhesive spinal arachnoiditis is a systemic process, and that spinal arachnoid cysts may also be extended, thecaloscopy may be regarded as the most radical and less-invasive form of surgical treatment that currently exists in neurosurgery.


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