Isolated calvarial aneurysmal bone cyst in a pediatric patient: illustrative case.

BACKGROUND: Aneurysmal bone cysts (ABCs) are benign, osteolytic lesions that can occur in long bones, vertebrae, or rarely, the skull. Here the authors present the case of a 15-year-old male with a primary ABC of the left frontoparietal skull along with a review of the literature to provide insight into the nature of this rare disease. OBSERVATIONS: An otherwise healthy 15-year-old male presented with a tense, painful lesion of the left frontoparietal scalp. He could not identify any inciting trauma, but first noted the lesion less than 2 weeks prior to presentation with progressive enlargement. Cranial imaging revealed a lytic skull lesion with fluid-fluid levels suggestive of ABC. Curative therapy was provided via wide excision of the lesion and calvarial reconstruction of the resultant skull defect. This was performed without complication, and histopathological evaluation confirmed the diagnosis of primary ABC. LESSONS: ABCs of the skull are rare entities and most often arise in the skull base versus the calvaria. Typically, these lesions are associated with an underlying bone pathology (secondary ABCs) but can be rarely seen as isolated lesions (primary ABCs). Clinical management consists of excision and adjuvant therapy for underlying pathology where appropriate.

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

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.

Spinal epidural lipomatosis in a pediatric patient with a malignant brain tumor: illustrative case.

BACKGROUND: Spinal epidural lipomatosis (SEL) in pediatric patients with concomitant malignant brain neoplasms is rare and can present with rapid deterioration in neurological function. OBSERVATIONS: A 4-year-old boy with SEL became paraplegic 4 months after completion of chemoradiation for his previously resected, intracranial atypical teratoid rhabdoid tumor. The patient presented with rapid deterioration in lower extremity sensory and motor function, which, given his oncological history, was concerning for disease progression. Of note, 8 months prior, the patient was started on corticosteroid therapy for respiratory dysfunction. Magnetic resonance imaging revealed significant progression of lumbosacral SEL requiring surgical decompression with subsequent neurological improvement. LESSONS: When evaluating pediatric patients with primary or metastatic brain tumors with new or worsening myelopathy and motor or sensory deficits, it is important to consider SEL.

Quality of life in a 61-year-old paraplegic patient with myelomeningocele: illustrative case.

BACKGROUND: Limited data exist on survival of patients with myelomeningocele (MM) into late adulthood. Patients born prior to 1975 received less surgical treatment and demonstrated poorer survival. Patients born after 1975 show lower mortality rates because of advancements in prenatal care, diagnostics, technology, and treatment. The increasing number of adult patients with MM demonstrates these successes but also highlights the need for a better understanding of both medical management and quality-of-life issues in this population. OBSERVATIONS: A 61-year-old woman presented to the hospital with a functional level T12, vertebral level low lumbar MM that had developed a tissue defect with a cerebrospinal fluid leak. The patient had her MM closed at birth and since then had received no additional surgical treatment or repair of her MM defect. After successful treatment, the patient continues to follow up for additional decompression procedures and admits to a good quality of life. LESSONS: This case adds to the limited literature describing management of patients with MM over the age of 60. The patient's self-described good quality of life and longevity align with reports of importance of independence, level of education, bowel management, ability to provide self-care, strong interpersonal relationships, and absence of shunted hydrocephalus.

Postinfectious coronavirus disease 2019 hemorrhagic cerebellitis: illustrative case.

BACKGROUND: Conditions that can mimic posterior fossa tumors are rare. Their identification is crucial to avoid unnecessary surgical intervention, especially when prompt initiation of medical therapy is critical. OBSERVATIONS: The authors presented a case of pseudotumoral hemorrhagic cerebellitis in a 3-year-old boy who presented initially with headache, persistent vomiting, and decreased level of consciousness 9 weeks after severe acute respiratory syndrome coronavirus 2 infection. Magnetic resonance imaging showed a left cerebellar hemorrhagic mass-like lesion with edema and mild hydrocephalus. The patient responded to high-dose steroids and was discharged 2 weeks later with complete recovery. LESSONS: When evaluating patients with possible tumor syndromes, it is important to also consider rarer inflammatory syndromes that can masquerade as neoplasms. Postinfectious hemorrhagic cerebellitis is one such syndrome.

The use of 5-aminolevulinic acid in resection of pediatric brain tumors: a critical review.

The compound, 5-aminolevulinic acid (5-ALA) is approved for fluorescence-guided resections of malignant gliomas in Europe and other countries for use in adults, but not for children. The application of 5-ALA in children remains an off-label use. Several case reports on fluorescence-guided surgery use in children have been published, yet no prospective study has been conducted. Here we systematically review the reported studies and discuss the usefulness, application, and safety of 5-ALA use in resection of pediatric brain tumors.

Parasitic rachipagus conjoined twin: case report.

A parasitic twin represents a rare developmental anomaly in which an asymmetrical, nonviable conjoined twin is attached to the host body at the time of birth. Rachipagus is among the rarest of conjoined twin subtypes and typically features a parasitic twin mass attached at the spine. Herein, the authors review the literature and describe the case of a 9-month-old girl presenting with a rachipagus parasitic twin consisting of a fully developed set of lower extremities originating from the midline upper back. After a complete workup to delineate parasite and autosite anatomy, the parasitic twin mass was successfully excised by a multidisciplinary team of surgeons, and the resulting defect was closed in a single stage.

Considerations in the management of congenital cranial dermoid cysts.

OBJECTIVE Congenital dermoid cysts (CDCs) develop from the entrapment of the surface ectoderm along the lines of embryonic fusion and have a capacity to grow. Given this capacity for continual expansion, the timing of removal and anticipation of possible epidural extension is important. METHODS The authors retrospectively reviewed records of patients with the diagnosis of dermoid cyst presenting over a period of 10 years. Baseline characteristics, histological reports, and surgical records were collected and analyzed. Only those patients with histological confirmation of dermoid cyst were included in the study. RESULTS One hundred fifty-nine (64 male and 95 female) patients were studied. The average age at the time of surgery ranged from 1 month to 63 years (mean 3.55 ± 9.58 years, median 11 months). Eighteen (11.3%) CDCs were in the midline anterior fontanelle, 52 (32.7%) were frontozygomatic, 14 (8.8%) occurred along the coronal sutures, and 75 (47.2%) occurred along the lambdoid sutures. In 74 (46.5%) cases the cysts were found to insignificantly erode the cranium, 30 (18.9%) eroded through a partial thickness of the cranium, 47 (29.6%) eroded through the full thickness of the cranium, and 8 (5.0%) eroded through the full thickness of the cranium and demonstrated epidural extension. The study population showed female predominance (n = 95, 59.7%). Although cyst location and patient sex were not found to vary significantly with cranial involvement (p = 0.196 and p = 0.066, respectively), delay in time to surgery did vary significantly (p < 0.00001). CONCLUSIONS Congenital cranial dermoid cysts found in infants and children are best removed early.

Use of 5-aminolevulinic acid for visualization and resection of a benign pediatric brain tumor.

5-Aminolevulinic acid has been used successfully to facilitate resection of adult malignant gliomas. The authors report their experience in the treatment of a 9-year-old girl who underwent fluorescence-guided resection of a right temporal lobe pleomorphic xanthoastrocytoma using 5-aminolevulinic acid. Intraoperatively, the residual tumor fluoresced, allowing for better visualization. No serious side effects or complications occurred.

Intraoperative magnetic resonance imaging in pituitary macroadenoma surgery: an assessment of visual outcome.

Pituitary macroadenomas most frequently present with visual loss. Although transsphenoidal surgery remains the treatment of choice for patients with neurological manifestations, there have been several advances in its implementation over the last 5 years. Intraoperative magnetic resonance (MR) imaging has emerged as a novel quality control measure, with the potential to guide the surgeon to tumor remnants concealed from the operating microscope. Investigators have reported enhanced resections when using intraoperative MR imaging, leading to complete tumor removal in a larger proportion of cases. Further debulking of unresectable lesions may also prove beneficial in delaying symptom recurrence and facilitating radiotherapy, where distance between the tumor and optic chiasm is an important predictor of visual outcome. However, confirmation of such advantages is complicated by the fact that most macroadenomas are both indolent and hormonally silent, necessitating years of follow-up. Experienced pituitary surgeons will operate as safely with intraoperative MR imaging as without it, perhaps due to a balance between more elaborate resections and better visualization. Intraoperative MR imaging represents a new technique applied to an old problem in tumor surgery: complete, safe resection.