-
Journal of Neurosurgery. Case Lessons Sep 2022Multiple-site open neural tube defects (MNTDs) and multiple-site split cord malformations (MSCMs) are extremely rare congenital anomalies that are defined by the...
Multiple-site neural tube defects complicated by multiple-site split cord malformations and thickened filum terminale: experience at a pediatric neurosurgical teaching hospital in Ethiopia. Illustrative case.
BACKGROUND
Multiple-site open neural tube defects (MNTDs) and multiple-site split cord malformations (MSCMs) are extremely rare congenital anomalies that are defined by the simultaneous noncontiguous occurrence of more than one neural tube defect (NTD) and split cord malformation (SCM), respectively, in a single case with normal neural tissue in between. This work shows the cooccurrence of MNTDs and MSCMs, which has never been reported in the literature.
OBSERVATIONS
A single-stage repair for a 13-day-old female neonate with a preoperative diagnosis of MNTDs (thoracic meningocele and thoracolumbar myelomeningocele) plus an additional intraoperative diagnosis of MSCMs (type 3c) of thoracic and thoracolumbar spine, and thickened filum terminale was done with a favorable smooth postoperative course.
LESSONS
The use of intraoperative meticulous surgical technique along with preoperative skin stigmata helped for anticipation, detection, and treatment of associated complex spinal MNTDs, especially in resource-limited settings, where preoperative magnetic resonance imaging is not routinely used. Whether to repair the MNTDs as a single- versus multiple-stage procedure is mainly a function of the patient's tolerance to the duration of anesthesia and the anticipated blood loss for the patient's age. The overall developmental biology and long-term clinical outcome of MNTDs compared to single NTD/SCM is poorly understood and needs further study.
PubMed: 36593675
DOI: 10.3171/CASE22220 -
Current Opinion in Neurology Feb 2023Idiopathic intracranial hypertension (IIH) is a disorder of raised intracranial pressure (ICP). Although the majority of patients with IIH present classically with... (Review)
Review
PURPOSE OF REVIEW
Idiopathic intracranial hypertension (IIH) is a disorder of raised intracranial pressure (ICP). Although the majority of patients with IIH present classically with headache and papilledema, some patients may have unusual presentations or manifestations. Recent advancements in neuroimaging have facilitated the identification of other presentations associated with IIH. This review provides an overview of the expanding clinical spectrum of IIH.
RECENT FINDINGS
Presentations of IIH that are considered unusual include highly asymmetric or unilateral papilledema, IIH without papilledema, and IIH associated with cranial nerve involvement. These presentations likely reflect differences in the way cerebrospinal fluid (CSF) pressure is transmitted intracranially. Radiological signs of intracranial hypertension are increasingly recognized in patients with IIH and provide further insights into the effects of raised ICP on intracranial structures. Osseous changes in the skull base leading to formation of meningoceles and encephaloceles have been identified in patients with IIH, spontaneous skull base CSF leak, and drug-resistant temporal lobe epilepsy, suggesting a possible association.
SUMMARY
Clinicians should be familiar with the expanding clinical spectrum of IIH and the implications for the management of these presentations.
Topics: Humans; Pseudotumor Cerebri; Papilledema; Intracranial Hypertension; Cerebrospinal Fluid Leak; Neuroimaging
PubMed: 36444979
DOI: 10.1097/WCO.0000000000001131 -
The Pan African Medical Journal 2022
Topics: Humans; Meningocele; Meningomyelocele; Spinal Dysraphism
PubMed: 36405669
DOI: 10.11604/pamj.2022.42.288.36209 -
Medicine Nov 2022Neurofibromatosis type 1 (NF-1) can manifest with various neurological symptoms. However, sensory ataxia has not been reported.
RATIONALE
Neurofibromatosis type 1 (NF-1) can manifest with various neurological symptoms. However, sensory ataxia has not been reported.
PATIENT CONCERNS
A 44-year-old man with NF-1 presented with several weeks of unsteady gait. He was diagnosed with gastric neuroendocrine tumor with multiple hepatic metastases 6 years ago and received palliative chemotherapy. Neurological examination revealed ataxia veering to the right side with no motor weakness.
DIAGNOSES
Clinical manifestations and electrodiagnostic studies suggested the dysfunction of the thoracic dorsal column (DC). Initial magnetic resonance imaging showed a lateral thoracic meningocele (LTM) located in the right paravertebral area at the T3-T4 vertebral level, but the spinal cord was unremarkable. Gait disturbance worsened after 9 months, and follow-up magnetic resonance imaging showed high signal intensity involving the right DC at the level adjacent to the LTM and spinal cord atrophy distal to the DC lesion. Tests for well-characterized paraneoplastic antibodies were negative. Ultimately, the patient was assumed to have sensory neuronopathy due to compressive damage to the dorsal root ganglia within the intervertebral foramina by LTM.
INTERVENTIONS
Empirical treatment with vitamin B12 supplementation and corticosteroids failed to improve his condition. The patient underwent decompressive laminectomy and excision of the meningocele with dura repair.
OUTCOMES
The patient temporarily improved to walk with assistance postoperatively. However, he developed dyspnea and hypotension 5 weeks later. Carcinoid heart disease confined the patient to the bed. The patient died of pneumonia 3 months after the operation.
LESSONS
This case with NF-1 shows asymmetric sensory ataxia of subacute progression. LTM may contribute to the development of sensory neuronopathy by damaging sensory neurons of the dorsal root ganglia. The comorbidities of the patient, including gastric neuroendocrine tumor and LTM, made it challenging to investigate the pathomechanism.
Topics: Male; Humans; Adult; Neurofibromatosis 1; Meningocele; Spinal Cord; Ataxia; Neuroendocrine Tumors
PubMed: 36397418
DOI: 10.1097/MD.0000000000031718 -
BMC Neurology Nov 2022Meningoencephalocele is a rare malformation caused by congenital and acquired lesions. The association between recurrent bacterial meningitis and meningoencephaloceles...
BACKGROUND
Meningoencephalocele is a rare malformation caused by congenital and acquired lesions. The association between recurrent bacterial meningitis and meningoencephaloceles with cerebrospinal fluid (CSF) leak is reported in the literature. We report a rare case of meningoencephalocele secondary to chronic idiopathic intracranial hypertension as a result of hospitalization repeatedly for meningitis due to the lack of CSF leak.
CASE PRESENTATION
This study presents a case of a patient with a decade of recurrent meningitis. With clinical symptoms and imaging examination with chronic idiopathic intracranial hypertension, this patient was diagnosed with meningoencephalocele. With the treatment of acetazolamide to decrease CSF product, the patient had no recurrence of meningitis over the 6-months follow-up period.
CONCLUSION
In patients with recurrent intracranial infections but no history of immunodeficiency, cranial trauma, or neurosurgery, the possibility of meningitis should be considered appropriately, even in the absence of CSF otorrhea or rhinorrhea.
Topics: Humans; Pseudotumor Cerebri; Cerebrospinal Fluid Rhinorrhea; Meningocele; Encephalocele; Cerebrospinal Fluid Leak; Central Nervous System Infections; Meningitis, Bacterial; Intracranial Hypertension
PubMed: 36368955
DOI: 10.1186/s12883-022-02959-w -
Neurology India 2022
Topics: Child; Humans; Meningocele; Developmental Disabilities; Sacrum; Spinal Diseases; Meningomyelocele; Magnetic Resonance Imaging
PubMed: 36352675
DOI: 10.4103/0028-3886.359169 -
Cureus Nov 2022Each vertebra is formed by combining the distal portion of one somite and the cranial half of its proximate part. genes regulate the patterning of the shapes of the...
BACKGROUND
Each vertebra is formed by combining the distal portion of one somite and the cranial half of its proximate part. genes regulate the patterning of the shapes of the non-identical spinal column. In the sacral area, anatomical dissimilarity is on account of the distinct shape of the sacral hiatus and the outright non-subsistence of the posticous embankment of the sacral neural tube, which is a consequence of the non-fulfillment of bonding of lamina of all sacral vertebrae. After that, the meninges and the spinal nerve are unprotected and undiagnosable without an X-ray examination. Therefore, it is difficult to detect the reasons for caudal block failure, low back pain, etc. The current research aimed to improve the proficiency of anatomical and developmental errors of the dorsal wall of the sacrum.
METHODS
This study was conducted on 60 dried adult sacra of unknown sexes from the stock wing of Anatomy, Sylhet MAG Osmani Medical College, Bangladesh, from 2017 to 2018. The undefined gender of the sacrum was identified.
RESULTS
Out of 60 sacra, 30 (50.0%) were found to be that of males and 30 (50.0%) of females. Among the study samples, only three (5%) samples presented a complete absence of the sacrum's dorsal wall and and incidence among males was higher than females.
CONCLUSION
This type of sacral aberration has paramount clinical importance. Thereby avoiding caudal epidural block-connected sufferings and backbone operative procedures. The expertise regarding the anatomical variation of sacral hiatus is necessary to reduce the failure rate during caudal epidural anesthesia, helps orthopedic surgeons diagnose the cause of low back pain or in surgical situations, and helps pediatricians deal with congenital anomalies such as meningocele and myelomeningocele.
PubMed: 36349077
DOI: 10.7759/cureus.31163 -
Cureus Oct 2022Intrathoracic meningoceles (IM) are quite rare; they are commonly associated with neurofibromatosis type 1 (NF-1). We report a case of a 55-year-old lady who was...
Intrathoracic meningoceles (IM) are quite rare; they are commonly associated with neurofibromatosis type 1 (NF-1). We report a case of a 55-year-old lady who was admitted to our emergency department with a sore throat, mild fever, cough, and right-sided chest pain, and tested positive for coronavirus disease 2019 (COVID-19). Ιmaging revealed a meningocele in the right upper pulmonary area, attributed to her NF-1. Clinicians should be aware that patients with NF-1 can develop IM, and they should be included in the differential diagnosis of patients with an intrathoracic mass.
PubMed: 36348911
DOI: 10.7759/cureus.29872 -
The Pan African Medical Journal 2022
Topics: Humans; Spinal Dysraphism; Meningomyelocele
PubMed: 36338552
DOI: 10.11604/pamj.2022.42.258.35894 -
The Pan African Medical Journal 2022Anterior dural tears complicated by cerebrospinal fluid (CSF) leakage with anterior meningocele are rare. Indeed, in the literature, cases are described during anterior...
Anterior dural tears complicated by cerebrospinal fluid (CSF) leakage with anterior meningocele are rare. Indeed, in the literature, cases are described during anterior arthrodesis, but no cases of post-cervical arthroplasty are described. The management of this type of complication is poorly described and not consensual. We present a case of a patient who underwent cervical arthroplasty complicated by an anterior meningocele at 1 month after the first surgery. Imaging revealed a compressive anterior meningocele in relation to the clinically progressive worsening. Revision surgery consisted of a combination of closure of the gap with a fatty patch covered with a TachoSil patch, followed by reinsertion of a new cervical prosthesis. At the last follow-up at 1 year, the patient showed no residual effects of the complication, and the mobility of the disc prosthesis was not impaired by it. Clinical results of the arthroplasty are also very satisfactory. Although these types of complications are rare, it is important to have a consensus on the management of anterior meningocele. In our experience, TachoSil appears to be a satisfactory option for the management of these complications.
Topics: Humans; Meningocele; Cervical Vertebrae; Treatment Outcome; Diskectomy; Arthroplasty; Postoperative Complications
PubMed: 36338550
DOI: 10.11604/pamj.2022.42.257.35812