Case of pediatric cerebellar, hippocampal, and basal nuclei transient edema with restricted diffusion (CHANTER) syndrome in a 2-year-old girl.

Cerebellar, hippocampal, and basal nuclei transient edema with restricted diffusion (CHANTER) syndrome is a recently described entity that refers to a specific pattern of cerebellar edema with restricted diffusion and crowding of the fourth ventricle among other findings. The syndrome is commonly associated with toxic opioid exposure. While most commonly seen in adults, we present a case of a 2-year-old girl who survived characteristic history and imaging findings of CHANTER syndrome.

Clinical Outcomes in Neurologically Intact Children With Small Intracranial Bleeds and Simple Skull Fractures.

Introduction Children with minor intracranial hemorrhage (ICH) and/or simple skull fractures are often hospitalized for monitoring; however, the majority do not require any medical, surgical, or critical care interventions. Our purpose was to determine the rate of significant clinical sequela (SCS) and identify associated risk factors in neurologically intact children with close head trauma. Methods This is a retrospective observational study. Children (≤ 3 years of age) admitted with closed head trauma, documented head injuries (ICH ≤ 5mm and/or simple skull fracture), and a Glasgow Coma Scale (GCS) score of ≥14, between January 2015 and January 2020, were included. We collected demographics, resource utilization, and patient outcomes variables. SCS was defined as any radiologic progression, and/or clinically important medical or neurological deterioration. Results A total of 205 patients were enrolled in the study (65.4% male, mean age 7.7 months). Repeat neuroimaging was obtained in 41/205 patients (20%) with radiologic progression noted in 5/205 (2.4%). Thirteen out of 205 patients (6.3%) experienced SCS. Patients with SCS were more likely to be males (92.3% vs 63.5% in females, P=0.035) to have had a report filed with child protective services due to a concern for abuse/neglect (92.3% vs 61.5% in females, P=0.025), and to have had a non-linear skull fracture (P<0.001). No other factors were shown to be predictive of SCS with enough statistical significance. Conclusion Neurologically intact children with traumatic closed head injury are at low risk for developing SCS. This study suggests that most of these children may not need ICU monitoring. This study also showed that a certain subset might be at an increased risk of developing SCS.

To cut or not to cut? A case report on pediatric intervertebral disc calcification.

BACKGROUND: Intervertebral disc calcification (IVDC) is a rare cause of acute spinal pain in pediatric patients. The most common symptom is back or neck pain, but muscle spasm, muscle weakness, and sensory loss also occur. Many patients have an alarming presentation and radiological findings concerning for spinal cord compression. CASE DESCRIPTION: A 10-year-old female presented with 2 weeks of worsening back pain and restricted neck flexion with no history of preceding trauma. Magnetic resonance imaging (MRI) showed T4/5 and T5/6 vertebral disc calcification and posterior herniation causing thoracic spinal cord compression. Despite concerning imaging findings, we decided to manage this patient conservatively with nonsteroidal anti-inflammatory drugs, leading to the improvement of symptoms within 9 days, and resolution of all pain within 1 month after hospital discharge. At 6 months follow-up, MRI showed complete resolution of calcification within the spinal canal. CONCLUSION: This case report emphasizes IVDC as an important differential diagnosis of pediatric disc disease that does not require surgical intervention. X-ray imaging with PA and lateral views is an adequate screening for these patients. Majority of cases resolve within 6 months with conservative therapy.

Recurrence of cavernous malformations after surgery in childhood.

OBJECTIVE: Cavernous malformations (CMs) are commonly treated cerebrovascular anomalies in the pediatric population; however, the data on radiographic recurrence of pediatric CMs after surgery are limited. The authors aimed to study the clinical presentation, outcomes, and recurrence rate following surgery for a large cohort of CMs in children. METHODS: Pediatric patients (≤ 18 years old) who had a CM resected at a single institution were identified and retrospectively reviewed. Fisher's exact test of independence was used to assess differences in categorical variables. Survival curves were evaluated using the Mantel-Cox method. RESULTS: Fifty-three patients aged 3 months to 18 years underwent resection of 74 symptomatic CMs between 1996 and 2018 at a single institution. The median length of follow-up was 5.65 years. Patients most commonly presented with seizures (45.3%, n = 24) and the majority of CMs were cortical (58.0%, n = 43). Acute radiographic hemorrhage was common at presentation (64.2%, n = 34). Forty-two percent (n = 22) of patients presented with multiple CMs, and they were more likely to develop de novo lesions (71%) compared to patients presenting with a single CM (3.4%). Both radiographic hemorrhage and multiple CMs were independently prognostic for a higher risk of the patient requiring subsequent surgery. Fifty percent (n = 6) of the 12 patients with both risk factors required additional surgery within 2.5 years of initial surgery compared to none of the patients with neither risk factor (n = 9). CONCLUSIONS: Patients with either acute radiographic hemorrhage or multiple CMs are at higher risk for subsequent surgery and require long-term MRI surveillance. In contrast, patients with a single CM are unlikely to require additional surgery and may require less frequent routine imaging.

Primary solitary retro-clival amyloidoma.

BACKGROUND: Amyloidosis encompasses a group of disorders sharing the common feature of intercellular deposition of amyloid protein by several different pathogenetic mechanisms. Primary solitary amyloidosis, or amyloidoma, is a rare subset of amyloidosis in which amyloid deposition is focal and not secondary to a systemic process or plasma cell dyscrasia. CASE DESCRIPTION: This 84-year-old female presented with history of multiple syncopal episodes, dysphagia, and ataxia. Motor strength was 3+/5 in the right upper extremity. Rheumatoid factor, cyclic citrullinated peptide (CCP), and anti-nuclear antibody (ANA) were normal. Serum and urine immune-electrophoresis detected no abnormal bands. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated a non-enhancing soft-tissue mass extending from the retro-clivus to C2 posteriorly, eccentric to the right with severe mass effect on the upper cervical medullary junction. Endoscopic trans-nasal debulking of the retro-clival mass was performed with occiput to C5 posterior instrumentation for spinal stabilization. CONCLUSIONS: Primary solitary amyloidosis, unlike other forms of amyloidosis, has an excellent prognosis with local resection. Diagnosis requires special stains and a degree of suspicion for the disease. This is the first report to document an endoscopic trans-nasal approach for removal of a primary solitary amyloidosis of the retro-clivus. Management of vertebral amyloidoma involves aggressive local resection of the tumor when feasible and spine stabilization as the degree of tumor involvement mandates. Complete evaluation for the diagnosis of systemic amyloidosis is essential for the management and prognostication. Surgeons encountering such lesions must maintain high suspicion for this rare disease and advise pathologists accordingly to establish the correct diagnosis.

Diagnosis and treatment of pediatric frontotemporal pits: report of 2 cases.

In contrast to more common nasal and cervical lesions, the frontotemporal pit is a rarely encountered lesion that is often associated with a dermoid and may track intracranially. Due to delays in diagnosis, the propensity to spread intracranially, and the risk of infection, awareness of these lesions and appropriate diagnosis and management are important. The authors present 2 cases of frontotemporal pits from a single institution. Epidemiology, presentation, and management recommendations are discussed.

Supratentorial Neurenteric Cysts: Case Series and Review of Pathology, Imaging, and Clinical Management.

BACKGROUND: Neurenteric cysts are rare congenital lesions along the neuroaxis, typically found in the spine, and rarely intracranially. Here, we present 3 patients who presented to our institution during a 6-year period with supratentorial intracranial neurenteric cysts and conduct a comprehensive review of the literature to describe the salient pathology, radiologic features, and clinical issues regarding these lesions. CASE REPORTS: Three patients were treated surgically for supratentorial neurenteric cysts. One patient presented in extremis, whereas the others were treated electively. Each patient presented with significantly different signs and symptoms and unique radiologic findings. All patients were neurologically intact after surgery. CONCLUSIONS: Neurenteric cysts present with a variety of signs and symptoms. Given the increased use of neuroimaging, supratentorial neurenteric cysts may be encountered more frequently and are important to include on the differential diagnosis and managed accordingly. Postoperative seizures occur in more than 20%, even in patients who had no preoperative seizures. Surgery can be performed safely with good neurologic outcomes.

Iatrogenic dural arteriovenous fistula and aneurysmal subarachnoid hemorrhage.

The authors present the case of a patient who presented acutely with aneurysmal subarachnoid hemorrhage (SAH) and a contralateral iatrogenic dural arteriovenous fistula (DAVF). Diagnostic angiography was performed, revealing a right-sided middle cerebral artery (MCA) aneurysm and a left-sided DAVF immediately adjacent to the entry of the ventriculostomy and bur hole site. A craniotomy was performed for clipping of the ruptured MCA aneurysm, and the patient subsequently underwent endovascular obliteration of the DAVF 3 days later. The authors present their treatment of an iatrogenic DAVF in a patient with an aneurysmal SAH, considerations in management options, and a literature review on the development of iatrogenic DAVFs.