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Open access

Successful surgical management of a superior cerebellar artery aneurysm in a patient with Marfan syndrome: illustrative case

Fangjun Liu, Mengqing Hu, Daling Ruan, Xiaoling Ruan, Ting Lei, and Xiang’en Shi

BACKGROUND

Marfan syndrome, a connective tissue disorder, poses unique challenges in neurosurgery, given the fragility of vascular structures. Superior cerebellar artery (SCA) aneurysms in patients with the syndrome are rare and present distinct surgical difficulties, necessitating innovative approaches.

OBSERVATIONS

A 29-year-old male with Marfan syndrome presented with a subarachnoid hemorrhage from a ruptured SCA aneurysm. Given the lack of a defined aneurysm neck and the small diameter of the SCA, standard clipping and endovascular therapies were unsuitable. A microsurgical approach using microsutures was successfully employed, effectively managing the aneurysm while preserving the parent artery.

LESSONS

This case underscores the efficacy of the microsuture technique in complex neurosurgical scenarios, particularly in patients with connective tissue disorders such as Marfan syndrome. The adaptability of surgical strategies, as demonstrated in this case, is crucial for achieving successful outcomes in patients with unique anatomical challenges.

Open access

Treatment of an anterior cervicothoracic myelomeningocele together with spine deformity correction in a child: illustrative case

Hudin N Jackson, Nealen Laxpati, and David F Bauer

BACKGROUND

Anterior cervicothoracic myelomeningoceles are a rare pathology. In reported cases, treatment has included shunting, isolated resection and repair without deformity correction, or isolated deformity correction without meningocele repair. The authors describe a pediatric patient with an anterior cervicothoracic myelomeningocele presenting with progressive neurological decline, who underwent simultaneous treatment of the myelomeningocele to detether the spinal cord and achieve major correction of the scoliotic deformity.

OBSERVATIONS

A 15-year-old girl was born with C7-T1-T2 hemivertebrae and anterior cervical myelomeningocele at C7–T1. She developed progressive cervical thoracic scoliosis, left hemiparesis initially, and additional right hemiparesis eventually. She underwent surgical repair via C7, T1, and T2 corpectomies with intradural detethering of the spinal cord. The scoliosis was treated with C7–T2 Ponte osteotomies and C2–T5 posterior fixation, followed by anterior reconstruction with a titanium cage and anterior plate from C6 to T3. The myelomeningocele was adequately treated with good correction of the patient’s deformity. The patient had postoperative improvement in her strength and solid arthrodesis on postoperative imaging.

LESSONS

The authors describe the successful treatment of an anterior cervicothoracic myelomeningocele and associated scoliosis in a child. This is a unique report of a combined strategy to achieve both deformity correction and detethering of the spinal cord.

Open access

Laser interstitial thermal therapy as a radiation-sparing approach for central nervous system tumors in children with cancer predisposition syndromes: report of a child with Li-Fraumeni syndrome. Illustrative case

Sergio W Guadix, Abhinav Pandey, Carson Gundlach, Michael Walsh, Nelson S Moss, and Mark M Souweidane

BACKGROUND

Ionizing radiation and alkylating chemotherapies increase secondary malignancy risk in patients with cancer predisposition syndromes (CPSs), such as Li-Fraumeni syndrome. Laser interstitial thermal therapy (LITT) is a minimally invasive ablation technique that has not been associated with mutagenic risks. We describe the case of a child with LFS and a history of treated choroid plexus carcinoma (CPC) who developed a second primary glial tumor that was safely treated with magnetic resonance imaging (MRI)–guided LITT.

OBSERVATIONS

A 4-year-old male with left parietal World Health Organization grade III CPC associated with a TP53 germline mutation was evaluated. The patient underwent neoadjuvant platinum-based chemotherapy before near-total resection, followed by 131I-8H9 immunotherapy and 30 fractions of 54-Gy proton radiotherapy. He remained without evidence of disease for 2 years before developing a slow-growing mass adjacent to the left frontal ventricular horn. Stereotactic biopsy revealed a glial neoplasm. Given the nonsuperficial location and focality of the lesion, MRI-guided LITT was performed for ablative therapy. There were no complications, and 2 years of surveillance revealed continued retraction of the ablated tumor focus and no subsequent disease.

LESSONS

Alternatives to mutagenic therapies for brain tumors should be explored for patients with CPS. LITT paired with imaging surveillance is a logical strategy to ensure durable outcomes and mitigate treatment-related secondary neoplasms.

Open access

The complex treatment paradigms for concomitant tethered cord and scoliosis: illustrative case

Rose Fluss, Riana Lo Bu, Andrew J Kobets, and Jaime A Gomez

BACKGROUND

Scoliosis associated with tethered cord syndrome is one of the most challenging spinal deformities to manage. Multiple surgical approaches have been developed, including traditional staged and concomitant procedures, spine-shortening osteotomies, and individual vertebral column resections.

OBSERVATIONS

A 10-year-old female presented with congenital kyphoscoliosis with worsening curve progression, tethered spinal cord, and a history of enuresis. The scoliosis had progressed to a 26° coronal curve and 55° thoracolumbar kyphosis. Preoperative magnetic resonance imaging of the spine revealed a tethered cord between the levels of L3–4 and a large kyphotic deformity at L1. The patient underwent laminectomy, during which intraoperative motor signals were lost. A planned hemivertebrectomy at L1 was performed prior to an L4 laminectomy, untethering of the filum terminale, and posterior spinal fusion from T11 to L2. After surgery, the patient experienced transient lower-extremity weakness, with her neurological function improving from baseline over the next 2 months. Ultimately, the goal of this surgery was to halt the progressive decline in motor function, which was successfully achieved.

LESSONS

Much remains to be learned about the treatment of this complicated disease, especially in the setting of concomitant scoliosis. This case serves to exemplify the complex treatment paradigms that exist when attempting to manage this clinical syndrome and that more remains to be learned.

Open access

Nontraumatic symptomatic de novo arachnoid cyst in an adolescent patient treated with cystoperitoneal shunting: illustrative case

Angelica M Fuentes, Jonathan J Yun, and John A Jane Jr.

BACKGROUND

Arachnoid cysts are often congenital, asymptomatic lesions detected in the pediatric population. When seen in adults, they usually occur following trauma. De novo formation of arachnoid cysts is uncommon, with only a few instances cited in the literature and most of which occurred in the pediatric population. Treatment options for these lesions include observation, craniotomy for cyst resection, microsurgical/endoscopic fenestration, or shunting.

OBSERVATIONS

In this report, the authors describe a female patient with a de novo, symptomatic, enlarging middle cranial fossa arachnoid cyst detected at age 16 years. She was treated with the placement of a cystoperitoneal shunt. After surgery, she experienced clinical and radiological improvement.

LESSONS

We illustrate successful shunting of a de novo arachnoid cyst in a symptomatic teen patient. Although arachnoid cysts in certain intracranial locations are more likely to produce symptoms, those in the middle cranial fossa tend to be asymptomatic. To our knowledge, this is the first reported case of a symptomatic de novo arachnoid cyst located in the middle cranial fossa in a postpubertal patient. Although the current presentation is rare, the authors demonstrate an effective surgical treatment of a symptomatic, large, de novo arachnoid cyst in a postpubertal pediatric patient.

Open access

Techniques for restoring optimal spinal biomechanics to alleviate symptoms in Bertolotti syndrome: illustrative case

Nolan J Brown, Zach Pennington, Hania Shahin, Oanh T Nguyen, and Martin H Pham

BACKGROUND

Lumbosacral transitional vertebrae (LSTVs) are congenital anomalies that occur in the spinal segments of L5–S1. These vertebrae result from sacralization of the lowermost lumbar segment or lumbarization of the uppermost sacral segment. When the lowest lumbar vertebra fuses or forms a false joint with the sacrum (pseudoarticulation), it can cause pain and manifest clinically as Bertolotti syndrome.

OBSERVATIONS

A 36-year-old female presented with severe right-sided low-back pain. Computed tomography was unremarkable except for a right-sided Castellvi type IIA LSTV. The pain proved refractory to physical therapy and lumbar epidural spinal injections, but targeted steroid and bupivacaine injection of the pseudoarticulation led to 2 weeks of complete pain relief. She subsequently underwent minimally invasive resection of the pseudoarticulation, with immediate improvement in her low-back pain. The patient continued to be pain free at the 3-year follow-up.

LESSONS

LSTVs alter the biomechanics of the lumbosacral spine, which can lead to medically refractory mechanical pain requiring surgical intervention. Select patients with Bertolotti syndrome can benefit from operative management, including resection, fusion, or decompression of the pathologic joint.

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.

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.

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.

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.