Repair of giant lumbosacral pseudomeningocele with fast-resorbing polymer mesh in a pediatric patient operated for posterior dysraphism.

Lumbar pseudomeningocele is a common complication of spinal surgery. It is caused by dural defect, and it consists of extradural collection of cerebrospinal fluid communicating with subarachnoid space. Usually it is small, asymptomatic, and self-limiting. In case of persistence, common treatments include bedrest, puncture and compressive dressing, subcutaneous or subarachnoid lumbar drainage, surgical re-exploration for durotomy closure, and blood patch. Fast-resorbing polymer mesh has been recently used for closure osseous gap in adult patients who developed giant pseudomeningocele following laminectomy. We report the case of a 16-year-old girl, affected by spinal dysraphism, who developed a persistent giant lumbar pseudomeningocele after repeated surgery for detethering of the spinal cord and removing of spinal lipomyelomeningocele. She was finally treated with fast-resorbing polymer mesh, to fill the gap of the posterior osseous defect and to provide support for the thecal sac. For our best knowledge, this is the first pediatric case reported in literature on this kind of reconstruction. In complex cases of pseudomeningocele, implant of fast-resorbing polymer mesh may represent an option also in pediatric patients.

Surgical management of middle cranial fossa bone defects: meningoencephalic herniation and cerebrospinal fluid leaks.

Along the medical practice of an Otologist he/she will face middle cranial fossa (MCF) bone defects. The purpose of this study is to contribute to the understanding of this possible life threatening condition, and to share and discuss our management approach. A literature review is also presented. STUDY DESIGN: Retrospective case series at García-Ibáñez Otology and Skull base private center referral. METHODS: This study is based on the analysis of data collected from 19 cases of temporal bone meningoencephalic herniations surgically treated from 2006 to 2018. The follow-up ranged from 18 to 162 months with a mean average of 44.5 months. MAIN FINDINGS: Meningoencephalic herniations were divided into four etiologic groups: spontaneous (24.8%), secondary to chronic otitis media (21.8%), iatrogenic (45.9%), and posttraumatic (7.5%). Different surgical techniques were used as treatment: transmastoid (TM) approach (27.8%), MCF approach (27.8%), combined technique (transmastoid plus minicraniotomy, 3%), and middle ear obliteration with blind sac closure of the external auditory canal (41.4%). CONCLUSIONS: Variables like bilateral hearing level, size and location of the bone defect and existence of CSF leak should be analyzed to select the safest and most effective closing surgical approach.

Comparing the results of duraplasty using amniotic membrane versus pericranium as dural graft; concerning CSF leakage and pseudomeningocele.

Purpose: In many brain surgeries, dura cannot be primarily repaired and it is necessary to use a graft. Appropriate repair of dura plays a major role in preventing CSF leakage and meningitis. In the cases where sufficient Pericranium is not available for recovery, we need to use graft from other resources. The present study compares the results of amniotic membrane dural graft and pericranium dural graft in terms of CSF leakage and Pseudomeningocele. Materials and methods: This is a semi-empirical research. Thirty cases underwent dural graft with amnion membrane because of inadequate local pericranium for repair. There were 30 other historical cases that had undergone auto-graft dural graft with Pericranium in the same center.Results: The average age of the participants in Pericranium graft group (P, n = 30) and those in amniotic graft group (A, n = 30) was 39.1 ± 15 and 43.9 ± 19 years old, respectively. As for the P and A groups, there were 14 and 17 males, respectively. Two cases of CSF leakage (7%) were observed in P group, while none was reported in A group. There were 3 cases (10%) of meningitis in Pericranium group and 2 cases (7%) were reported in amnion group. Out of 2 cases of meningitis in group P, 2 cases were associated with CSF leak. 5 cases of Hydrocephalus (17%) were observed in group P and 9 cases (30%) were reported in group A. No significant difference was observed between the two groups in any complication. The frequency of hydrocephalus was significantly higher in posterior fossa craniotomy.Conclusion: Amniotic membrane may provide a good source for cases with a large dural defect and can be utilized for all ages. Of course, its application in cases of posterior fossa craniotomy needs to be carefully studied so that the best results may be obtained.

Descriptive and risk factor analysis of nonsyndromic sacral agenesis: National Birth Defects Prevention Study, 1997-2011.

Sacral agenesis is a rare birth defect characterized by partial or complete absence of the sacrum. We sought to (a) describe case characteristics, (b) estimate birth prevalence, and (c) identify risk factors for nonsyndromic sacral agenesis using data from the National Birth Defects Prevention Study (NBDPS). The NBDPS was a population-based, case-control study involving pregnancies with estimated dates of delivery from October 1997 through December 2011. We estimated birth prevalence using all NBDPS eligible cases. Using self-reported maternal exposure information, we conducted multivariable logistic regression analysis to identify potential risk factors overall and among women without diabetes. The birth prevalence of sacral agenesis was 2.6/100,000 live births. In the multivariable analysis, multifetal pregnancy, pre-existing Type 1 diabetes, and pre-existing Type 2 diabetes were positively and significantly associated with sacral agenesis, albeit estimates were imprecise. Preexisting Type 1 diabetes was the strongest risk factor (adjusted odds ratio = 96.6, 95% confidence interval = 43.5-214.7). Among women without diabetes, periconceptional smoking was positively and significantly associated with sacral agenesis. Our findings underscore the importance of smoking cessation programs among women planning pregnancy and the importance of better understanding the role of glycemic control before and during pregnancy when designing interventions for primary prevention of sacral agenesis.

Resistant Pseudomeningocele following Craniotomy for Non-Traumatic Acute Intracranial Haematoma in Very Young Children: Report of Four Cases.

BACKGROUND: The incidence of non-traumatic intracranial haemorrhage among neonates is less than among adults. However, the outcomes are generally poor, with high rates of morbidity and mortality. Non-traumatic intracranial haemorrhage can cause a sudden increase in intracranial pressure, requiring emergency craniotomy for evacuation. METHODS: We report 4 cases following craniotomy for non-traumatic acute intracranial haematoma in very young children. The surgical technique and postoperative course are discussed, with emphasis on dural repair to avoid resistant pseudomeningocele. RESULTS: Despite successful management of the acutely raised intracranial pressure, all patients afterwards developed tense pseudomeningoceles and required formal dural repair. This led to a change in institutional practice and modifications to the surgical technique. CONCLUSION: The authors highlight the postoperative occurrence of resistant pseudomeningoceles in young children after clot evacuations and leaving the dura open. They describe the surgical procedures to avoid these pseudomeningoceles.

Analysis of petrous apex meningocele associated with meningioma: is there any relation with chronic intracranial hypertension?

PURPOSE: Petrous apex meningocele (PAM) is an uncommon cystic lesion involving the petrous apex. The underlying cause of PAM may be related to chronic elevated intracranial pressure. The aim of the study was to explore the relationship between PAM and meningioma and between PAM and other intracranial hypertension findings. METHODS: Two hundred seventy-eight consecutive patients with meningiomas were retrospectively studied. Fifty age- and gender-matched controls were also enrolled in this study. The incidence of PAM, empty sella, tortuosity of the optic nerve, and hydrops of optic nerve sheath was evaluated. The maximum width, area, volume of each PAM, or Meckel's cave and volume of meningioma were measured in controls and patients, separately. RESULTS: One hundred fifty-nine (57.19%) patients were detected with coexistent PAMs. One hundred twenty-five patients had bilateral PAMs, 34 had unilateral lesions, and the remaining 119 did not have PAM. Two subjects (4/50) had unilateral PAMs in normal controls. The maximum width, area, volume of PAM, or Meckel's cave were significantly larger in the patients with bilateral PAM group than those in the unilateral PAM group, in the group without PAM, and those in control group (p = 0.000). The volume of meningioma was positively correlated with the PAM volume (r = 0.48). There was a positive correlation for the incidence between PAM and (1) empty sella (r = 0.901) and (2) tortuosity of the optic nerves and hydrops of the optic sheath (r = 0.825). CONCLUSION: Coexistence of PAMs with meningiomas is not rare in incidence, and it suggests a potential role for chronically elevated intracranial pressure and disturbance of CSF circulation in their pathophysiology.

Penetrating orbital trauma leading to trans-orbital brain herniation.

INTRODUCTION: Development of a posttraumatic herniation of brain parenchyma through the orbit is a rare complication of orbital roof fracture. Mostly, the injury is due to a direct impact to the frontal region resulting in orbital roof fracture with dural defect and herniation of cerebrospinal fluid or brain parenchyma. These patients present with acute or gradually progressive proptosis with impending risk of loss of vision and mandate surgical decompression of optic nerve with watertight closure of the dural defect. Bony reconstruction may be required to achieve normal contour of the orbit. MATERIALS AND METHODS: We encountered an interesting pediatric patient who presented to us with altered sensorium and progressive proptosis 3 days after a penetrating trauma to his left orbit by falling on the handle of a bicycle. The plain computerized tomography of the head (NCCT) showed a left orbital roof defect with herniation of brain matter into the orbit displacing the globe inferolaterally. A craniotomy was performed and watertight closure of the dural rent was achieved with use of autologous bone to cover the defect. CONCLUSIONS: The child improved gradually, and at 6 months follow-up, he had good cosmetic outcome with improvement of vision.

Craniocervical Pseudomeningocele Following Cerebellar Meningioma Resection: Demonstration of Neck of Pseudomeningocele With Three-Dimensional Isotropic T2-Weighted SPACE Sequence at 3 Tesla (3T) Magnetic Resonance Imaging.

Postoperative pseudomeningocele is an uncommon complication of craniospinal surgery. Diagnosis is reached on a postoperative computed tomography and magnetic resonance (MR) imaging. Demonstration of the location and dimension of the dural defect before surgical therapy is a very important. T1- and T2-weighted MR images revealed a significant pseudomeningocele extending from left cervicooccipital region. Magnitude and phase-contrast-MR images showed a cerebrospinal fluid (CSF) flow into pseudomeningocele, but they no revealed dural defect. Three-dimensional isotropic T2-weighted SPACE sequence revealed a signal void indicating CSF flow into pseudomeningocele and location and exact size of dural tear. Three-dimensional isotropic T2-weighted SPACE sequence is certainly the noninvasive and optimal method for demonstrating postoperative pseudomeningocele sacs. It demonstrates a pseudomeningocele regardless of an existing communication with the dural membrane.

Intradiploic pseudomeningocele and ossified occipitocervical pseudomeningocele after decompressive surgery for Chiari I malformation: report of two cases and literature review.

Intradiploic cerebrospinal fluid (CSF) collections are rare findings. The authors describe two pediatric patients with iatrogenically induced occipital CSF collections after decompressive surgery for Chiari I malformation. The first patient presents a large occipital intradiploic pseudomeningocele and the second patient an intradiploic pseudomeningocele merging with an ossified occipitocervical pseudomeningocele. Though being rarities after decompression for Chiari I malformation, intradiploic fluid collection and ossified pseudomeningocele should be considered if patients represent with aggravating presurgical or new symptoms.

Posttraumatic Intradiploic Pseudomeningocele of the Occipito-Cervical Region.

Pseudomeningocele is a collection of the cerebrospinal fluid in the extradural space due to a defect in the dura-arachnoid layer of the meninge, and manifests as a fibrous capsule in the space of subcutaneous tissues. Classically, growing skull fracture caused by the pseudomeningocele is not uncommon in the pediatric age group. However, a posttraumatic intradiploic pseudomeningoceles is extremely rare, and only a few patients have been described. Herein, the authors present a 6-year-old girl who developed an intradiploic pseudomeningocele of the occipito-cervical region after a severe head trauma.

Low-pressure headaches following foramen magnum decompression secondary to absorption of cerebrospinal fluid into the venous system of the diploic space.

INTRODUCTION: We describe a case of a patient who suffered low-pressure headaches secondary to absorption of cerebrospinal fluid into the venous system of the diploic space following a foramen magnum decompression. This case is important as it describes a physiologically plausible but previously undescribed complication of a common surgical intervention. CASE: A nine-year-old boy underwent a foramen magnum decompression for a Chiari malformation (Type I). Five years after his initial decompression, his original symptoms returned and he underwent further decompression with short-term relief of symptoms. He then began to describe low-pressure headaches. Comprehensive investigations revealed a small posterior pseudomeningocoele that had extended into the diploic space at the site of the previous surgery to the occipital bone. It was postulated that the diploic space may be acting as an additional site for reabsorption of his CSF, and as a result of this, he was experiencing consistently low-pressure symptoms. His symptoms have completely resolved following surgical intervention to seal the site of communication with the diploic space. DISCUSSION: We review recent literature that supports this theory through an understanding of the anatomy of the diploic venous system and also its physiological behaviour as demonstrated in recent cadaveric and porcine studies.

Ethmoidal meningoencephalocele and CSF leak after posthaemorrhagic ventricular dilatation in a newborn child.

BACKGROUND: Rhinoliquorrhoea suggests a communication between the subarachnoid space and the sinonasal tract. Clinical presentation includes clear nasal discharge, headache, pneumocephalus, meningitis or brain abscess. Cerebrospinal fluid (CSF) leaks are mostly of traumatic origin (skull base fractures), iatrogenic (secondary to endoscopic endonasal surgery) or associated with tumour aetiology. Occasionally, hydrocephalus has been the cause of rhinoliquorrhoea in adults, presumably secondary to the chronically raised intracranial pressure with skull base erosion and meningocele. To our knowledge, the association of hydrocephalus and ethmoid meningoencephalocele/CSF leak has not been previously reported in a newborn child. CASE PRESENTATION: We present the case of a 9-month-old girl who was referred for rhinorrhoea. She had a history of posthaemorrhagic ventricular dilatation. Brain computed tomography (CT) and magnetic resonance imaging (MRI) showed a left ethmoidal meningoencephalocele and small ventricular size. The meningoencephalocele was surgically repaired using an intradural subfrontal approach. During the postoperative period, after the transient lumbar drain was withdrawn, she developed symptomatic hydrocephalus. Ventriculoperitoneal shunting was required. CONCLUSION: Progressive ventricular dilatation may arise from a meningoencephalocele/CSF leak in paediatric patients. Early identification and repair of the meningoencephalocele are critical to avoid development of complications.

Occipital post-traumatic intradiploic arachnoid cyst converted to pseudomeningocele after re-trauma: a rare complication of rare pathology.

BACKGROUND: Growing skull fracture (GSF)/pseudomeningocele is a rare complication of head injury in children. Post-traumatic intradiploic arachnoid cyst (PTIAC) is a variant but it is extremely rare. PTIACs are usually asymptomatic or with mild symptoms like headache. The rupture of PTIAC due to re-trauma leading to pseudomeningocele formation has not been seen or reported before. PATIENT AND METHOD: We present a case of occipital PTIAC where pseudomeningocele developed after re-trauma, and discuss the pathogenesis and management. RESULT: En masse removal of the ruptured PTIAC was performed along with dural-defect repair. The post-operative recovery was good. CONCLUSION: PTIAC is an extremely rare occurrence as a variant of GSF. It is usually asymptomatic, but it may rupture with trivial trauma due to a thinned-out outer table and then converts into pseudomengocele. En masse excision with dural-defect repair gives good results in cases of ruptured PTIAC due to re-trauma.

[Caudal regression sequence: clinical-radiological case]. / Secuencia de regresión caudal: caso clínico-radiológico.

Caudal regression syndrome is an uncommon congenital malformation that includes a wide spectrum of clinical presentations. Characterised by caudal musculoskeletal compromise, it can be associated to neurological, gastrointestinal, renal and genitourinary defects. Although the specific aetiology has not been clarified, it has been associated with the presence of maternal diabetes and mutations in homeobox gene HBLX9. Its diagnosis is based on a good prenatal ultrasound detection, detailed physical examination, and post-natal imaging study using radiography and magnetic resonance. Caudal regression syndrome requires multidisciplinary management, and it seems that good metabolic control of gestational diabetes constitutes the best preventive measure available. We present the clinical case and images of a male term newborn, born to a pregestational diabetic mother with poor metabolic control and a prenatal ultrasound diagnosis of lumbar spine, iliac bones and lower limbs malformation. Born in good conditions, the diagnosis was confirmed using X-rays and magnetic resonance.

[Pseudomeningocele with spinal cord compression following removal of meningioma at the Th3-Th4 level: a case report].

Pseudomeningocele is an abnormal extradural collection of the cerebrospinal fluid in soft tissues, communicating with the arachnoid space through a dural defect. Postoperative pseudomenigocele of the thoracic spine presenting with myelopathy is a rare phenomenon; we found only two such cases in the literature. A clinical case of a female patient operated on for meningioma at the Th3-Th4 level with postoperative pseudomenigocele and spinal cord compression is presented.

Surgical management of chronic traumatic pseudomeningocele of the craniocervical junction: case report.

PURPOSE: Chronic traumatic pseudomeningocele (PM) is a rare complication of gunshot injuries of the craniocervical junction in pediatric patients. Impairment of the CSF dynamics may cause severe symptoms and should be treated. METHODS: We report the case of a 6-year-old girl who was accidentally shot in the neck during tribal clashes. On being admitted, she was neurologically intact with cerebrospinal fluid (CSF) leakage through the wounds. She underwent primary closure of the wounds in a rural medical facility. After two episodes of meningitis, CSF leakage resolved spontaneously. Nine months later, the patient was presented with a disfiguring mass growing in the posterior neck, severe headaches, and constitutional symptoms such as loss of appetite and a failure to thrive. RESULTS: Neurosurgical intervention was performed with the patient in the prone position. Occipital pericranium graft was used to repair the defect, and the cavity of the PM was obliterated with muscle layers. The patient's symptoms improved at 1 year follow-up without PM recurrence. CONCLUSION: This is a rare presentation of gunshot injuries in an environment with limited neurosurgical resources. Restoring the normal pattern of CSF circulation should be the aim of any neurosurgical intervention.

Lateral meningocele syndrome: additional report and further evidence supporting a connective tissue basis.

Lateral meningocele syndrome is a rare disorder of unknown etiology, first described in 1977 and subsequently reported in nine other patients. These patients present distinctive craniofacial features and skeletal abnormalities in addition to multiple lateral meningoceles, suggesting a connective tissue disorder. Autosomal dominant inheritance is clearly suggested in one family and could explain familiar aggregation in another. We describe a simplex case of lateral meningocele syndrome with bicuspid aortic valve, supporting the hypothesis of a connective tissue basis for this disorder and further expanding the phenotype.