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Open access

Endoscopic endonasal optic nerve decompression in children younger than 2 years old with congenital optic canal stenosis: illustrative cases

Peter H Yang, John S Schneider, Michael R Chicoine, Albert H Kim, and David D Limbrick Jr

BACKGROUND

Congenital optic canal stenosis causing compressive optic neuropathy is a rare disorder that presents unique diagnostic and treatment challenges. Endoscopic endonasal optic nerve decompression (EOND) has been described for optic nerve compression in adults and adolescents but has never been reported for young children without pneumatized sphenoid sinuses. The authors describe preoperative and intraoperative considerations for three patients younger than 2 years of age with congenital optic canal stenosis due to genetically confirmed osteopetrosis or chondrodysplasia.

OBSERVATIONS

Serial ophthalmological examinations, with a particular focus on object tracking ability, fundoscopic examination, and visual evoked potential trends in preverbal children, are important for detecting progressive optic neuropathy. The lack of pneumatization of the sphenoid sinus presents unique challenges and requires the surgical creation of a sphenoid sinus with the use of neuronavigation to determine the limits of bony exposure given the lack of easily identifiable anatomical landmarks such as the opticocarotid recess. There were no perioperative complications.

LESSONS

EOND for congenital optic canal stenosis is safe and technically feasible even given the lack of pneumatization of the sphenoid sinus in young patients. The key operative step is surgically creating the sphenoid sinus through careful bony removal with the aid of neuronavigation.

https://thejns.org/doi/10.3171/CASE23559

Open access

Surgical treatment of torticollis secondary to the presence of a congenital paracondylar process: illustrative case

Grace Hey, Otavio De Toledo, Salvador Gutierrez-Aguirre, Steven Andreoli, Pushpak Patel, Alyssa Tamasi, Asmaa Hatem, and Philipp Aldana

BACKGROUND

A paracondylar process is an abnormal bony exostosis arising from the skull base lateral to the occipital condyle and extending inferiorly toward the transverse process of the atlas. Paracondylar processes are typically identified incidentally and vary in size from a small protuberance to an elongated process.

OBSERVATIONS

The authors present the first pediatric case of torticollis secondary to a congenital paracondylar process successfully treated with resection of the paracondylar process and sternocleidomastoid release.

LESSONS

Cadaveric dissection, three-dimensional models, intraoperative imaging guidance, and multidisciplinary collaboration were paramount to a successful surgical outcome.

https://thejns.org/doi/10.3171/CASE2447

Open access

A child with unilateral abducens nerve palsy and neurovascular compression in Chiari malformation type 1 resolved with posterior fossa decompression: illustrative case

Olivia A Kozel, Belinda Shao, Cody A Doberstein, Natalie Amaral-Nieves, Matthew N Anderson, Gita V Harappanahally, Michael A Langue, and Konstantina A Svokos

BACKGROUND

Unilateral cranial nerve (CN) VI, or abducens nerve, palsy is rare in children and has not been reported in association with Chiari malformation type 1 (CM1) in the absence of other classic CM1 symptoms.

OBSERVATIONS

A 3-year-old male presented with acute incomitant esotropia consistent with a unilateral, left CN VI palsy and no additional neurological symptoms. Imaging demonstrated CM1 without hydrocephalus or papilledema, as well as an anterior inferior cerebellar artery (AICA) vessel loop in the immediate vicinity of the left abducens nerve. Given the high risk of a skull base approach for direct microvascular decompression of the abducens nerve and the absence of other classic Chiari symptoms, the patient was initially observed. However, as his palsy progressed, he underwent posterior fossa decompression with duraplasty (PFDD), with the aim of restoring global cerebrospinal fluid dynamics and decreasing possible AICA compression of the left abducens nerve. Postoperatively, his symptoms completely resolved.

LESSONS

In this first reported case of CM1 presenting as a unilateral abducens palsy in a young child, possibly caused by neurovascular compression, the patient’s symptoms resolved after indirect surgical decompression via PFDD.

Open access

Epidural lipomatosis with foci of hemorrhage and acute compression of the spinal cord in a child with CLOVES syndrome: illustrative case

Dmytro Ishchenko, Iryna Benzar, and Andrii Holoborodko

BACKGROUND

Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies, and/or scoliosis (CLOVES) syndrome is the most recently described combined vascular anomaly characterized by congenital excessive growth of adipose tissue, vascular malformations, epidermal nevi, and skeletal deformities. This condition exhibits a significant variability in clinical manifestations and a tendency for rapid progression and affects extensive anatomical regions. Information regarding the association of epidural lipomatosis with low-flow venous lymphatic malformations is rare, with few reports in the literature.

OBSERVATIONS

The authors present a case of a 6-year-old girl who was admitted to the emergency department complaining of rapidly progressing weakness in her lower extremities and partial loss of sensation in the inguinal area. Radiologically, an extradural mass was identified at the T2–6 level, causing acute spinal cord compression. Urgent decompression and partial resection of the mass were performed. Despite satisfactory intraoperative hemo- and lymphostasis, postoperative lymphorrhea/seroma leakage was encountered as a delayed complication and was managed conservatively.

LESSONS

CLOVES syndrome is characterized by the combination of various clinical symptoms, not all of which are included in the abbreviation, as well as a progressively deteriorating course, the emergence of new symptoms, and complications throughout the patient’s life. This necessitates ongoing monitoring of such patients.

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.