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BACKGROUND
Bilateral cerebellopontine angle (CPA) lipomas are extremely rare. Herein the authors present a case of bilateral CPA lipomas in an infant along with a literature review of bilateral CPA lipomas.
OBSERVATIONS
A newborn girl was incidentally found to have bilateral CPA lipomas during the workup for an occipital encephalocele. The encephalocele was repaired primarily on day 2 after birth. The patient demonstrated no symptoms associated with the bilateral CPA lipomas. Eight cases of bilateral CPA lipomas were identified in the literature review and are summarized. Conservative management is the consensus strategy, given minimum growth of the tumor and the high risk of surgical intervention.
LESSONS
This is the first reported case of bilateral CPA lipomas in an infant as well as the first with a coexisting intracranial malformation. Intracranial lipomas share an extremely low growth rate and typically do not cause severe symptoms. The management of asymptomatic or mildly symptomatic bilateral CPA lipomas is usually conservative.
BACKGROUND
Brain arteriovenous malformations (AVMs) usually manifest as hemorrhages or seizures. They rarely present with ischemic symptoms, especially in young patients. We present a case of an epileptogenic AVM that led to cerebral infarction due to paradoxical embolic occlusion of the middle cerebral artery (MCA) involving the main feeder of the lesion.
OBSERVATIONS
A 35-year-old male had been suffering from AVM-associated epilepsy for 10 years and was scheduled for surgery. He suddenly developed right-sided hemiconvulsions followed by hemiparalysis and impaired consciousness. Computed tomography revealed no intracerebral hemorrhage, and symptoms were initially thought to indicate epilepsy and Todd’s palsy. Because of his prolonged symptoms, he underwent magnetic resonance imaging, which revealed a large cerebral infarction due to occlusion of the MCA involving the main feeder of the AVM. The patient underwent AVM resection, and the partially thrombosed nidus was completely removed. Histopathological investigation revealed a fresh thrombus in totally occluded nonarteriosclerotic feeders. He had no atrial fibrillation; however, subsequent transesophageal echocardiography revealed a patent foramen ovale, suggesting a paradoxical embolism.
LESSONS
This case serves as a reminder that AVMs can present with considerable variability. Acute cerebral infarction should be considered a possible mechanism of seizures, even in patients with epileptogenic AVM.
BACKGROUND
The occurrence of hyperostotic bilateral spheno-orbital meningiomas (BSOMs) is very rare. Patients present with bilateral symptoms and require bilateral treatment. This series describes 6 patients presenting to 2 UK neurosurgical units and includes a literature review. To the best of the authors’ knowledge, this is the largest series documented.
OBSERVATIONS
This is a retrospective review of patients with BSOMs presenting between 2006 and 2023. Six females, whose mean age was 43 (range: 36–64) years, presented with features of visual disturbance. Bilateral sphen-oorbital meningiomas were identified. All patients underwent bilateral staged resections. The patients had an initial improvement in their symptoms. Extensive genetic testing was performed in 4 patients, with no variants in the NF2, LZTR1, SMARCB1, SMARCE1, and SMARCA4 genes or other variants detected. The mean follow-up was 100.3 (range: 64–186) months. Sixty-seven percent of patients had good long-term visual acuity. The progression rate was 75% and was particularly aggressive in 1 patient. Four patients required radiation therapy, and 2 needed further surgery.
LESSONS
Hyperostotic BSOMs are extensive, challenging tumors causing significant disability. They can recur, with significant patient impact. Multidisciplinary management and indefinite long-term follow-up are essential. The biology of these tumors remains unclear. As molecular testing expands, the understanding of BSOM oncogenesis and potential therapeutic targets is likely to improve.
BACKGROUND
This article describes a rare case of cervical spinal arteriovenous malformation (AVM) mimicking a neurogenic spinal tumor.
OBSERVATIONS
A 22-year-old female presenting with a C6–7 AVM with a calcification nodule experienced new-onset acute right upper radiculopathy associated with extradural compression of the spinal cord. Note that spinal AVMs with a calcified nodule are rare. Endovascular embolization is generally used to relieve the symptoms of AVM; however, this procedure cannot relieve cord compression, particularly in cases complicated by calcified nodules. This article discusses treatment options.
LESSONS
Decompression surgery is preferable to endovascular embolization because it alleviates symptoms while preventing cord compression and minimizing the risk of recurrence.
BACKGROUND
Underprivileged and underserved patients from developing countries often present late with advanced, untreated spinal deformities. We report a three-stage all-posterior approach using limited skeletal traction with Gardner-Wells tongs (GWTs) for the management of severe idiopathic scoliosis during a humanitarian surgical mission trip.
OBSERVATIONS
A 17-year-old high-school female was previously diagnosed with juvenile idiopathic scoliosis (diagnosed at age 8) and progressed to a severe 135° kyphoscoliosis. Procedural stage 1 involved spinal instrumentation and posterior releases via posterior column osteotomies from T3 to L4. She then underwent 7 days of skeletal traction with GWTs in the intensive care unit as stage 2. In stage 3, rod engagement, posterior spinal fusion, and partial T10 vertebral column resection were performed. There were no changes in intraoperative neuromonitoring during either surgery and she woke up neurologically intact after both stages of the surgical procedure.
LESSONS
Skeletal traction with GWTs is a viable alternative to traditional halo-gravity traction in settings with limited resources. Three-stage spinal deformity correction using limited skeletal traction is a feasible and effective approach for managing severe scoliosis during humanitarian surgical mission trips.
BACKGROUND
Intraorbital arteriovenous fistula (IOAVF) is a rare type of intracranial fistula that presents with ocular signs similar to those of cavernous sinus dural arteriovenous fistula. The treatment of IOAVF is based on the vascular architecture of each case due to its infrequent occurrence. The authors report the case of an IOAVF that was successfully treated with embolization via the facial vein, with good outcomes.
OBSERVATIONS
A 78-year-old woman presented with left eyelid swelling, pulsatile ocular protrusion, and left ocular conjunctival hyperemia. Ophthalmological evaluation revealed elevated intraocular pressure; time-of-flight magnetic resonance angiography revealed a dilated left superior ophthalmic vein. Digital subtraction angiography showed an arteriovenous shunt in the left superior orbital fissure, which was treated using transvenous coil embolization. The patient experienced immediate improvement in left ocular protrusion and conjunctival hyperemia. Ophthalmological evaluation 1 month after treatment showed normal intraocular pressure in the left eye. No neurological symptoms were observed, and there was no recurrence of the arteriovenous shunt 3 months postoperatively.
LESSONS
The authors report a rare case of IOAVF treated with embolization via the facial vein with a good outcome. A thorough understanding of the vascular architecture using three-dimensional images is useful for determining endovascular access and procedures.
BACKGROUND
Intramedullary schwannomas account for 1.1% of all spinal schwannomas. Preoperative diagnosis is best accomplished by thoroughly evaluating clinical and radiological characteristics, accompanied by a high index of suspicion. The authors report a case of C2–3 intramedullary schwannoma in a young male who presented with neck pain and vertigo. The current literature is also reviewed.
OBSERVATIONS
The authors reviewed the data of a young male with a 2-month history of neck pain and vertigo. Magnetic resonance imaging of the brain and cervical spine showed an intramedullary mass at C2–3 with a syrinx extending into the cervicomedullary junction. Laminectomy, myelotomy, and microsurgical excision of the mass under intraoperative neurological monitoring (IONM) were done. Postoperative pathology reported the specimen as a schwannoma.
LESSONS
Gross-total resection of a schwannoma using IONM is the treatment of choice because of the lesion’s benign nature, a better prognosis, and defined cleavage plane. Schwannomas should be included in the differential diagnosis of intramedullary spinal tumors. Because of its progressive nature, early surgery is recommended in symptomatic patients.
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