-
Journal of Personalized Medicine Apr 2024Spontaneous orbital cephaloceles are a rare condition. The purpose of this study is to provide a description of a clinical case and to carry out a systematic literature... (Review)
Review
BACKGROUND
Spontaneous orbital cephaloceles are a rare condition. The purpose of this study is to provide a description of a clinical case and to carry out a systematic literature review.
METHODS
A systematic review of the English literature published on the Pubmed, Scopus, and Web of Science databases was conducted, according to the PRISMA recommendations.
RESULTS
A 6-year-old patient was admitted for right otomastoiditis and thrombosis of the sigmoid and transverse sinuses, as well as the proximal portion of the internal jugular vein. Radiological examinations revealed a left orbital mass (22 × 14 mm) compatible with asymptomatic orbital meningocele (MC) herniated from the superior orbital fissure (SOF). The child underwent a right mastoidectomy. After the development of symptoms and signs of intracranial hypertension (ICH), endovascular thrombectomy and transverse sinus stenting were performed, with improvement of the clinical conditions and reduction of the orbital MC. The systematic literature review encompassed 29 publications on 43 patients with spontaneous orbital MC. In the majority of cases, surgery was the preferred treatment.
CONCLUSIONS
The present case report and systematic review highlight the importance of ICH investigation and a pathophysiological-oriented treatment approach. The experiences described in the literature are limited, making the collection of additional data paramount.
PubMed: 38793047
DOI: 10.3390/jpm14050465 -
Otolaryngology--head and Neck Surgery :... Jul 2024To review surgical techniques used in the endoscopic transnasal repair of pediatric basal meningoencephaloceles and compare perioperative outcomes in children <2 and... (Review)
Review
OBJECTIVE
To review surgical techniques used in the endoscopic transnasal repair of pediatric basal meningoencephaloceles and compare perioperative outcomes in children <2 and ≥2 years old.
DATA SOURCES
MEDLINE, EMBASE, and CENTRAL.
REVIEW METHODS
Data sources were searched from inception to August 22, 2022, using search terms relevant to endoscopic transnasal meningoencephalocele repair in children. Reviews and Meta-analyses were excluded. Primary outcomes were the incidence of intraoperative and postoperative complications, including cerebrospinal fluid leak, recurrence, and reintervention. Quality assessments were performed using Newcastle-Ottawa Scale, ROBIN-I, and NIH.
RESULTS
Overall, 217 patients across 61 studies were identified. The median age at surgery was 4 years (0-18 years). Fifty percent were female; 31% were <2 years. Most defects were meningoencephaloceles (56%), located transethmoidal (80%), and of congenital origin (83%). Seventy-five percent of repairs were multilayered. Children ≥2 years underwent multilayer repairs more frequently than those <2 years (P = 0.004). Children <2 years more frequently experienced postoperative cerebrospinal fluid leaks (P = 0.02), meningoencephalocele recurrence (P < 0.0001), and surgical reintervention (P = 0.005). Following multilayer repair, children <2 years were more likely to experience recurrence (P = 0.0001) and reintervention (P = 0.006).
CONCLUSION
Younger children with basal meningoencephaloceles appear to be at greater risk of postoperative complications following endoscopic endonasal repair, although the quality of available evidence is weakened by incomplete reporting. In the absence of preoperative cerebrospinal fluid leak or meningitis, it may be preferable to delay surgery as access is more conducive to successful repair in older children.
Topics: Adolescent; Child; Child, Preschool; Female; Humans; Infant; Encephalocele; Endoscopy; Meningocele; Natural Orifice Endoscopic Surgery; Nose; Postoperative Complications; Male; Infant, Newborn
PubMed: 38494838
DOI: 10.1002/ohn.711 -
Child's Nervous System : ChNS :... Jul 2024Meckel-Gruber Syndrome (MKS) is an autosomal recessive genetic disorder, notable for its triad of occipital encephalocele, polycystic renal dysplasia, and postaxial... (Review)
Review
INTRODUCTION
Meckel-Gruber Syndrome (MKS) is an autosomal recessive genetic disorder, notable for its triad of occipital encephalocele, polycystic renal dysplasia, and postaxial polydactyly. Identified by Johann Friederich Meckel in 1822, MKS is categorized as a ciliopathy due to gene mutations. Diagnosis is confirmed by the presence of at least two key features. The condition is incompatible with life, leading to death in the womb or shortly after birth. Recent studies have largely focused on the genetic aspects of MKS, with limited information regarding the impact of neurosurgical approaches, particularly in treating encephaloceles.
METHODS
A systematic review was performed according to the PRISMA statement. The PubMed, Embase, and Web of Science databases were consulted for data screening and extraction, which was conducted by two independent reviewers. The search strategy aimed to encompass studies documenting cases of MKS with published reports of encephalocele excisions, and the search strings for all databases were: Meckel-Gruber syndrome OR Meckel Gruber syndrome OR Meckel-gruber OR Meckel Gruber.
RESULTS
The study included 10 newborns with MKS associated with occipital encephalocele or meningocele, all of whom underwent surgical repair of the occipital sac. The mean gestational age at birth was 36 (± 2) weeks. The mean of birth weight was 3.14 (± 0.85) kilograms. The average head circumference at birth was 33.82 cm (± 2.17). The mean diameter of the encephalocele/meningocele was 5.91 (± 1.02) cm. Other common central nervous system abnormalities included hydrocephalus, Dandy-Walker malformation, and agenesis of the corpus callosum. 40% required shunting for hydrocephalus. Surgery to remove the occipital sac occurred at a median age of 2.5 days (1.5-6.5). The most common post-surgical complication was the need for mechanical ventilation. The most common cause of death was pneumonia and the median age at death was 6.66 (0.03-18) months.
CONCLUSION
Our findings suggest that neurosurgical intervention, especially for managing encephaloceles, may offer some improvement in survival, albeit within a context of generally poor prognosis. However, these results should be interpreted with caution.
Topics: Humans; Encephalocele; Retinitis Pigmentosa; Neurosurgical Procedures; Ciliary Motility Disorders; Polycystic Kidney Diseases; Eye Abnormalities; Infant, Newborn
PubMed: 38459147
DOI: 10.1007/s00381-024-06346-3 -
The Laryngoscope May 2024We undertook a systematic review of the literature with meta-analysis to identify the role of obesity (BMI ≥30) in the patient characteristics presenting with... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
We undertook a systematic review of the literature with meta-analysis to identify the role of obesity (BMI ≥30) in the patient characteristics presenting with spontaneous cerebrospinal fluid (sCSF) leaks of the lateral skull base and the outcomes of their repair.
DATA SOURCES
A Systematic Review of English Articles using MEDLINE, EMBASE, and Cochrane Library.
REVIEW METHODS
The research algorithm included the following keywords: "spontaneous CSF leak," "lateral skull base," "temporal bone," "meningocele," "encephalocele," and "otorrhea." We also manually searched the references of included studies, to identify possible studies missed during our literature search.
RESULTS
More than two-thirds of the patients were female (69.2%) and often were obese (mean BMI 36.5 kg/m) with a mean age of 57. Most common presenting symptoms were otorrhea and hearing loss. Most authors did not report a routine use of a post-operative lumbar drain. Most patients had a single skull base defect and encephaloceles prolapsing through, across obese and non-obese groups. Median length of stay in hospital was 3.2 days, and the majority of patients did not have any recurrence during their follow-up (89.6%), which was not affected by obesity.
CONCLUSION
Obesity does not affect length of hospital stay or recurrence rate following surgical repair of lateral skull base sCSF leaks. Surgical repair is a safe and viable approach in the management of obese patients with sCSF leaks in the temporal bone.
LEVEL OF EVIDENCE
NA Laryngoscope, 134:2012-2018, 2024.
Topics: Humans; Female; Middle Aged; Male; Retrospective Studies; Treatment Outcome; Skull Base; Cerebrospinal Fluid Leak; Obesity; Temporal Bone; Encephalocele
PubMed: 38400793
DOI: 10.1002/lary.31349 -
Child's Nervous System : ChNS :... Jan 2024Autosomal dominantly inherited neurofibromatosis type I (NF1) is a systemic disorder caused by a mutation of a gene on chromosome 17q11.2 and characterized by multiple... (Review)
Review
Autosomal dominantly inherited neurofibromatosis type I (NF1) is a systemic disorder caused by a mutation of a gene on chromosome 17q11.2 and characterized by multiple café-au-lait spots, lentiginous macules, Lisch nodules of the iris, and tumors of the nervous system. Bony manifestations such as scoliosis, dysplasia of the greater sphenoidal wing, tibial pseudoarthrosis, short stature, and macrocephaly have been reported in approximately 50% of patients. However, calvarial bone defects are rare. After screening 324 articles, 23 cases (12 adult and 11 pediatric patients) of occipital bone defects in NF1 patients were selected. All patients had a single/multiple bone defect over the lambdoid suture. Adjacent benign plexiform neurofibromas were observed in 14 patients (60.8%, 7 adults and 7 children); one adult patient was diagnosed with neurofibrosarcoma. Meningoencephalocele over the occipital defect was noted in 8 cases (34.78%, all adults). Cranioplasty was performed in only 17.39% of patients. Histologic examination was performed in 7 of the 15 patients with associated neurofibromas/neurofibrosarcomas. Biopsy of the bone margins surrounding the defect was performed in only one case. Pathologic examination of the herniated parieto-occipital or cerebellar tissue was not performed in any of the patients studied. We report the case of a 9-year-old girl with NF1 and a significant occipital bone defect and performed a systematic review of the relevant literature to highlight the challenges in treating this condition and to investigate the underlying mechanisms contributing to bone defects or dysplasia in NF1.
Topics: Adult; Female; Humans; Child; Neurofibromatosis 1; Cafe-au-Lait Spots; Mutation; Encephalocele; Occipital Bone
PubMed: 37993698
DOI: 10.1007/s00381-023-06232-4 -
Acta Otorhinolaryngologica Italica :... Apr 2023
Review
PubMed: 37698096
DOI: 10.14639/0392-100X-suppl.1-43-2023-02 -
Neurology India 2022The culprit of trigeminal neuralgia (TGN) may occur at any point between the nerve's root entry zone (REZ) and Meckel's cave. Meckel's cave meningoencephaloceles are... (Review)
Review
BACKGROUND
The culprit of trigeminal neuralgia (TGN) may occur at any point between the nerve's root entry zone (REZ) and Meckel's cave. Meckel's cave meningoencephaloceles are rare middle cranial fossa defects that usually remain asymptomatic but may contain prolapsed trigeminal nerve rootlets and result in TGN. Their management and surgical outcomes remain poorly understood.
OBJECTIVES
To perform a systematic review of clinical presentation and surgical outcomes of middle fossa defects presenting with trigeminal nerve-related symptoms.
MATERIALS AND METHODS
A systematic review was conducted in accordance with the PRISMA guidelines for all reports of middle cranial fossa defects causing trigeminal nerve-related symptoms. The pathophysiology, presentation, surgical management, and outcomes are discussed and illustrated with a case.
RESULTS
Initial search from inception to March 2021 identified 33 articles for screening. After applying inclusion and exclusion criteria, 6 articles were included representing a total of 8 cases in addition to our case (n = 9). All 9 patients were females and 33.3% (n = 3) presented with classic trigeminal neuralgia. "Empty sella" syndrome and radiologic signs of intracranial hypertension were present in 40%-62%. No patient presented with cerebrospinal fluid leak. The preferred treatment modality was surgical with subtemporal extradural repairs using combinations of autologous fat and muscle grafts and synthetic dura. Postoperative outcomes were only available in 55.5% (n = 5) of the cases, and nearly all reported complete symptom resolution, except for one case in which the meningoencephalocele wall was incised, along with trigeminal rootlets adhered to it. Our patient had immediate and durable symptom relief after a 4-year follow-up.
CONCLUSIONS
MEC containing prolapsed trigeminal nerve rootlets can cause typical trigeminal neuralgia from chronic pulsatile stress. This supports the hypothesis that the compressive or demyelinating culprit can locate more ventrally on the course of the trigeminal nerve. Subtemporal extradural surgical repairs can be safe, effective, and durable. Incising the MEC wall should be avoided as it may have trigeminal rootlets adhered to it.
Topics: Cranial Fossa, Middle; Dura Mater; Encephalocele; Female; Humans; Male; Meningocele; Trigeminal Nerve; Trigeminal Neuralgia
PubMed: 35864609
DOI: 10.4103/0028-3886.349629 -
Journal of Neurosurgery. Pediatrics Apr 2022Improper embryological development of the clivus, a bony structure that comprises part of the skull base, can lead to a clival canal defect. Previously thought to be a... (Review)
Review
OBJECTIVE
Improper embryological development of the clivus, a bony structure that comprises part of the skull base, can lead to a clival canal defect. Previously thought to be a benign condition, clival canals have been reported to be associated with meningitis and meningoceles. In this review, the authors sought to present an unpublished case of a patient with a clival canal defect associated with meningitis and to evaluate all other reported cases.
METHODS
In October 2020, a search of PubMed, Web of Science, and Scopus was conducted to identify all cases of clival canals reported from January 1, 1980, through October 31, 2020.
RESULTS
Including the case presented herein, 13 cases of clival canals, 11 in children (84.6%) and 2 in adults (15.4%), have been identified. Of the pediatric patients, 5 (45.5%) had an associated meningocele, and 8 (72.7%) had meningitis. Nine of the 13 patients (69.2%) had defects that were treated surgically, 5 (38.5%) by a transnasal approach and 4 (30.8%) by a transoral approach. Two patients (15.4%) were treated with drainage and antibiotics, 1 patient (7.7%) was treated solely with antibiotics, and 1 patient (7.7%) was not treated. In the literature review, 8 reports of clival canals were found to be associated with meningitis, further contributing to the notion that the clival canal may be an overlooked source of recurrent infection. In several of these cases, surgical repair of the lesion was curative, thus preventing continued episodes of meningitis.
CONCLUSIONS
When a patient has recurrent meningitis with no clear cause, taking a closer look at clival anatomy is recommended. In addition, if a clival canal defect has been identified, surgical repair should be considered a safe and effective primary treatment option.
Topics: Adult; Child; Cranial Fossa, Posterior; Humans; Meningitis; Meningocele; Skull Base; Treatment Outcome
PubMed: 35171832
DOI: 10.3171/2021.11.PEDS21388 -
Journal of Plastic, Reconstructive &... Apr 2022Complex back wounds with cerebrospinal fluid (CSF) leak constitute a challenge for surgeons in clinical practice. While repair of complex back wounds with various flaps... (Review)
Review
Complex back wounds with cerebrospinal fluid (CSF) leak constitute a challenge for surgeons in clinical practice. While repair of complex back wounds with various flaps is well described in the literature, there is a paucity of reviews and articles regarding optimal treatment for refractory CSF leaks. The aim of this systematic review was to present the different flap techniques proposed in the literature for CSF leaks fistulas and pseudo-meningoceles. A systematic review of the literature was conducted using the PubMed, Medline, Embase, and Cochrane databases. Studies tackling different flap techniques used for spinal or skull base CSF leak were included in the studies. The main outcomes were complications and recurrences after previous failed treatments. Fifteen studies were included in the study, constituting a cohort of 42 patients (mean age 45 years; F/M ratio 1.15:1). A previous unsuccessful non-flap procedure was proposed in 88.1% of patients for their CSF leak. Only 16.7% had a recurrence of their CSF leak after the flap procedure. Free radial forearm flap and regional muscle flaps were more frequently used in the skull base region. Pedicled omental flaps and reverse turnover latissimus dorsi were the most commonly used flaps in the thoracolumbar region. No statistically significant differences were found between these flaps regarding rates of complications and recurrence. Flaps should be considered as a safe and effective treatment option for complex back wounds with CSF leaks. All proposed flaps in the literature seem to provide an equivalent cure rate for recurrent and refractory leaks.
Topics: Cerebrospinal Fluid Leak; Humans; Middle Aged; Plastic Surgery Procedures; Retrospective Studies; Skull Base; Skull Base Neoplasms; Surgical Flaps
PubMed: 35168923
DOI: 10.1016/j.bjps.2022.01.022 -
Clinical Neurology and Neurosurgery Nov 2021Thoracic meningoceles and dural ectasia are less commonly recognized manifestations of neurofibromatosis 1 (NF1). Rarely, large thoracic meningoceles may become...
Thoracic meningoceles and dural ectasia are less commonly recognized manifestations of neurofibromatosis 1 (NF1). Rarely, large thoracic meningoceles may become compressive and lead to respiratory compromise secondary to lung compression. Surgical goals aim to increase lung aeration through decreasing the size of the meningocele through shunting, excision or repair of the meningocele, and varying degrees of dural tube reconstruction. There is no agreement on the best approach for large, symptomatic meningoceles. Here, we discuss the case of a 41-year-old woman with NF1 who presented with dyspnea and enlargement of a large, 19 cm thoracic meningocele. A multidisciplinary team of thoracic, plastic, and neurological surgery participated in the operation to excise the meningocele and reconstruct the dural tube without the need for subsequent shunting of spinal fluid. We also systematically review the literature on thoracic meningoceles in NF1 to understand the optimal treatment of this pathology.
Topics: Adult; Female; Humans; Meningocele; Neurosurgical Procedures; Thoracic Vertebrae; Thoracotomy
PubMed: 34739882
DOI: 10.1016/j.clineuro.2021.106996