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Open access

Approaches to ventriculoperitoneal shunt scalp erosion: countersinking into the calvarium. Illustrative case

Denise Brunozzi, Melissa A LoPresti, Jennifer L McGrath, and Tord D Alden

BACKGROUND

Ventriculoperitoneal shunting (VPS) is a standard procedure for the treatment of hydrocephalus, and the management of its complications is common in the practice of pediatric neurosurgery. Shunt exposure, though a rare complication, can occur because of thin, fragile skin, a young patient age, protuberant hardware, poor scalp perfusion, and a multitude of other patient factors.

OBSERVATIONS

The authors report a complex case of VPS erosion through the scalp in a young female with Pfeiffer syndrome treated with external ventricular drainage, empirical antibiotics, and reinternalization with countersinking of replaced shunt hardware into the calvarium to prevent internal skin pressure points, reduce wound tension, and allow wound healing.

LESSONS

Recessing the shunt hardware, or countersinking the implant, into the calvarium is a simple technique often used in functional neurosurgical implantation surgeries, providing a safe surgical strategy to optimize wound healing in select cases in which the skin flap is unfavorable.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 22 (Nov 2023)

Open access

Cranial vault suspension for basilar invagination in patients with open cranial sutures: technique and long-term follow-up. Illustrative case

Christopher B Cutler, Daphne Li, and John R Ruge

BACKGROUND

Hajdu-Cheney syndrome (HCS) is an extremely rare genetic disorder characterized by severe osteoporosis, scoliosis, and persistent open cranial sutures (POCSs). Neurological complications include hydrocephalus, Chiari I malformations, and basilar invagination (BI). Surgical intervention in HCS is challenging due to severe osteoporosis, ligamentous laxity, POCSs, and extreme skeletal deformities. Herein, the authors present a case of BI repair in a patient with HCS and POCSs, requiring a novel technique of cranial vault suspension, with long-term follow-up.

OBSERVATIONS

A 20-year-old female with HCS and progressive symptomatic BI, initially managed with posterior fossa decompression and occipital to cervical fusion, subsequently required cranial vault expansion due to symptomatic shifting of her cranium secondary to POCS. This custom construct provided long-term stabilization and neurological improvement over a follow-up duration of 9.5 years. A literature review performed revealed three other cases of surgical intervention for BI in patients with HCS and clinicopathological characteristics of each case was compared to the present illustrative case.

LESSONS

POCSs in patients with BI complicate traditional surgical approaches, necessitating more invasive techniques to secure all mobile cranial parts for optimal outcomes. Using this cranial vault suspension and fusion technique results in lasting neurological improvement and construct stability.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 22 (Nov 2023)

Open access

Symptomatic spinal arachnoid cyst with spastic diplegia secondary to cerebral palsy: illustrative case

Andrew Guillotte, Abdul-Rahman Alkiswani, Kathryn A Keeler, and Michael D Partington

BACKGROUND

Selective dorsal rhizotomy (SDR) can improve the spastic gait of carefully selected patients with cerebral palsy. Spinal arachnoid cysts are a rare pathology that can also cause spastic gait secondary to spinal cord compression.

OBSERVATIONS

The authors present an interesting case of a child with cerebral palsy and spastic diplegia. He was evaluated by a multidisciplinary team and determined to be a good candidate for SDR. Preoperative evaluation included magnetic resonance imaging (MRI) of the spine, which identified an arachnoid cyst causing spinal cord compression. The cyst was surgically fenestrated, which provided some gait improvement. After recovering from cyst fenestration surgery, the patient underwent SDR providing further gait improvement.

LESSONS

SDR can be beneficial for some patients with spastic diplegia. Most guidelines do not include spinal MRI in the preoperative evaluation for SDR. However, spinal MRI can be beneficial for surgical planning by localizing the level of the conus. It may also identify additional spinal pathology that is contributing to the patient’s spasticity. In rare cases, such as this one, patients may benefit from staged surgery to address structural causes of spastic gait prior to proceeding with SDR.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 20 (Nov 2023)

Open access

Microsurgical resection of a ruptured intraventricular arteriovenous malformation in a neonate: considerations in management. Illustrative case

Lauren Stone, Reid Colliander, Melissa A LoPresti, Ali Shaibani, and Sandi Lam

BACKGROUND

Arteriovenous malformations (AVMs) are the most common cause of intracranial hemorrhage in children, although they are rarer in neonates. Age, location, lesion architecture, and rupture status define treatment options. Sparse literature exists to guide the management of clinically symptomatic intraventricular AVM rupture in neonates. We highlight the case of a neonate with a ruptured intraventricular AVM to showcase considerations in treatment, discuss surgical technique, and help guide management.

OBSERVATIONS

An 18-day-old female presented with lethargy in extremis and was found to have new intraventricular hemorrhage. Angiogram revealed a Spetzler-Martin grade 2 AVM with a right posterior choroidal feeder and deep venous drainage within the ventricle. Her age limited radiosurgical and endovascular interventions. She underwent an interhemispheric, transcollosal, intraventricular approach for complete AVM resection. Perioperative care was managed by a multidisciplinary team, successfully mitigating the patient’s high risk of hemovascular collapse.

LESSONS

Stereotactic radiosurgery, endovascular embolization, and microsurgery are options for AVM obliteration, and multimodal therapy must be tailored to the lesion and patient. Conservative management can also be considered. Each intervention carries risks and varying likelihoods of success. Balancing these outcomes is challenging without definitive, high-quality, evidence-based guidance. The best treatment maximizes the chance of AVM obliteration while minimizing morbidity.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 15 (Oct 2023)

Open access

Spontaneous pseudoaneurysm of the superficial temporal artery in neurofibromatosis type 1: illustrative case

Fang Shen, Shi-ze Li, Yuan-yuan Shan, Xiao Ji, and Han-song Sheng

BACKGROUND

A pseudoaneurysm of the superficial temporal artery is an uncommon clinical entity that has largely been linked with direct traumatic causes. Neurofibromatosis type 1 (NF1)-related vasculopathy is a rare cause of idiopathic arterial bleeding in the craniofacial region.

OBSERVATIONS

A 46-year-old male with clinical features of NF1 presented to the hospital with an enlarging and tender right temporal mass without a history of trauma. Computed tomography angiography suggested the development of a pseudoaneurysm, and surgery was performed to resect the mass. Histopathological examinations showed focal interruption of the epithelium layer and elastic lamina, well-demarcated thickening of the smooth muscle layers of the arterial wall, supporting the diagnosis of pseudoaneurysm.

LESSONS

NF1-associated vasculopathy is likely the predisposing factor for the development of a superficial temporal artery pseudoaneurysm.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 14 (Oct 2023)

Open access

Bilateral cerebellopontine angle lipomas in an infant with encephalocele: illustrative case

Xiaochun Zhao, Kiana Y Prather, Sherwin A Tavakol, and Joanna E Gernsback

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.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 13 (Sep 2023)

Open access

Spinal arteriovenous malformation with a calcified nodule: illustrative case

Ping-Chuan Liu, Chung-Chia Huang, and Ching-Lin Chen

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.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 13 (Sep 2023)

Open access

Neglected cervical meningocele in an adult: illustrative case

Abolfazl Rahimizadeh, Seyed Ali Ahmadi, Ali Mohammadi Moghadam, Shaghayegh Rahimizadeh, Walter Williamson, Mahan Amirzadeh, and Sam Hajaliloo Sami

BACKGROUND

Posterior cervical meningoceles are rare in adults because most are surgically excised early in life. Such meningoceles in adults are mostly presented as a cystic mass and their presentation as a solid mass is very rare.

OBSERVATIONS

An asymptomatic adult with cervical meningocele presented as a congenital midline skin covered solid mass in the posterior aspect of the neck is presented. Neuroradiological surveys showed attachment of the mass to intradural spinal cord. With diagnosis of a cervical meningocele, after excision of the solid sac, the stalk extending from the core of the mass to the dura was isolated. This was followed by intradural spinal cord detethering. The mass was compatible with rudimentary meningocele in pathology.

LESSONS

Neglected cervical meningocele is quite rare in adults. Surgical removal of the mass in adults is usually for cosmetic reasons rather than neurological impairment. However, surgical removal of the mass without intradural cord detethering is not sufficient. In such cases, late onset quadriparesis may be appear due to the spinal cord tethering scenario.

In Journal of Neurosurgery: Case Lessons Volume 5 (2023): Issue 23 (Jun 2023)

Open access

Vascular anomaly, lipoma, and polymicrogyria associated with schizencephaly: developmental and diagnostic insights. Illustrative case

Kevin K. Kumar, Angus Toland, Nancy Fischbein, Martha Morrell, Jeremy J. Heit, Donald E. Born, and Gary K. Steinberg

BACKGROUND

Schizencephaly is an uncommon central nervous system malformation. Intracranial lipomas are also rare, accounting for approximately 0.1% of brain “tumors.” They are believed to be derived from a persistent meninx primitiva, a neural crest–derived mesenchyme that develops into the dura and leptomeninges.

OBSERVATIONS

The authors present a case of heterotopic adipose tissue and a nonshunting arterial vascular malformation arising within a schizencephalic cleft in a 22-year-old male. Imaging showed right frontal gray matter abnormality and an associated suspected arteriovenous malformation with evidence of hemorrhage. Brain magnetic resonance imaging revealed right frontal polymicrogyria lining an open-lip schizencephaly, periventricular heterotopic gray matter, fat within the schizencephalic cleft, and gradient echo hypointensity concerning for prior hemorrhage. Histological assessment demonstrated mature adipose tissue with large-bore, thick-walled, irregular arteries. Mural calcifications and subendothelial cushions suggesting nonlaminar blood flow were observed. There were no arterialized veins or direct transitions from the arteries to veins. Hemosiderin deposition was scant, and hemorrhage was not present. The final diagnosis was consistent with ectopic mature adipose tissue and arteries with meningocerebral cicatrix.

LESSONS

This example of a complex maldevelopment of derivatives of the meninx primitiva in association with cortical maldevelopment highlights the unique challenges from both a radiological and histological perspective during diagnostic workup.

In Journal of Neurosurgery: Case Lessons Volume 5 (2023): Issue 21 (May 2023)

Open access

Limited dorsal myeloschisis associated with intramedullary infantile hemangioma in the conus medullaris: illustrative case

Yoko Nakanishi, Noritsugu Kunihiro, Ryoko Umaba, Yasuhiro Matsusaka, Takeshi Inoue, and Hiroaki Sakamoto

BACKGROUND

Limited dorsal myeloschisis (LDM) and intramedullary infantile hemangioma rarely coexist in the spinal cord.

OBSERVATIONS

The authors describe the case of a 3-month-old girl who, despite lacking neurological symptoms or signs, had a cigarette burn-like mark at the lumbosacral area and skin dimpling in the gluteal area. Magnetic resonance imaging showed a low-set conus due to a thickened filum and an abnormal subcutaneous stalk connected to the conus medullaris. In combination with the skin lesions, these findings strongly implied nonsaccular-type LDM. An intramedullary mass in the conus medullaris was also shown on magnetic resonance imaging and was homogenously enhanced with isointensity on T1- and T2-weighted images. We prophylactically untethered the spinal cord and partially removed the intramedullary mass, which had no clear borders, for a safe surgical dissection. Histologically, the intramedullary mass was an infantile hemangioma, and the subcutaneous stalk was a lesion associated with LDM. The patient remained neurologically intact after surgery, and then 2 years later, there was spontaneous regression of the residual tumor.

LESSONS

Although rare, nonsaccular type LDM may appear concurrently with intramedullary infantile hemangioma at the conus medullaris. The authors present a possible mechanism behind this concurrent presentation in the same area.

In Journal of Neurosurgery: Case Lessons Volume 5 (2023): Issue 17 (Apr 2023)