Risk Factors for Pediatric Intracranial Neoplasms in the Kids' Inpatient Database.

INTRODUCTION: In children and adolescents, brain and central nervous system (CNS) tumors are the leading types of cancers. Past studies have found differing rates of intracranial cancers among races and identified additional cancer risk factors. This study aimed to see if these differences can be substantiated with further investigation of the latest version (2019) of the Kids' Inpatient Database (KID). METHODS: A total of 7,818 pediatric patients <21 years old in KID with ICD-10 codes consisting of malignant neoplasms of the brain, brainstem, and cerebral meninges (C700, C709-C719) were queried. Modifiable risk factors evaluated include: hospital region, insurance type, hospital city size, the average income of patient zip code, and location/teaching status of a hospital. Non-modifiable risk factors were race and sex at birth. Dependent variables were tested in Excel and GraphPad Prism 9 using a χ2 test with Yates' continuity correction and Tukey's one-way and two-way ANOVAs. RESULTS: Mortality rates of females (2.88%) compared to males (1.99%) were significant (p < 0.05). Mortality was (4.17%) in black patients compared to (1.68%) for white (p < 0.0001), Hispanic mortality (2.95%) compared to white (p < 0.01), and mortality of Asian/Pacific Islander (3.86%) compared to white (p < 0.01). Black patients had significantly higher mortality than white, Hispanic, Asian/Pacific Islander, Native American, and other races overall (p < 0.01). There was no significant difference in the mortality rates between children's hospitals and large hospitals for any race. After accounting for patient race, mortality was still not significantly different for patients with Medicaid insurance compared to non-Medicaid insurance types. Of the children treated at children's hospitals, the most transferred in from outside hospitals were Native American (20.00%) followed by Asian/Pacific Islander (15.09%) then Hispanic patients (13.67%). A significant difference between races was also seen regarding length of stay (p < 0.001) and number of charges (p < 0.001). CONCLUSION: These findings confirm prior studies suggesting gender and race are significant factors in mortality rates for children with intracranial neoplasms. However, the findings do not identify the root causes of these discrepancies but may serve as an impetus for clinicians, healthcare administrators, and governmental leaders to improve national resource allocation to better care for pediatric patients with intracranial neoplasms.

Third ventricular choroid plexus papilloma: a pure neuroendoscopic approach.

Pure endoscopic technique in resection of intraventricular tumors is an emerging technology. This case demonstrates resection of a multicentric choroid plexus papilloma in a 2-month-old child. This child had two district tumors: one located in the left atrium and another in the third ventricle. Initial microsurgery was performed to resect the left atrial tumor. With the tumor noted to be not very vascular at initial surgery, the third ventricle tumor was resected with a GAAB neuroendoscope and NICO Myriad. A gross-total resection was achieved. At 3 years' follow-up, the child remains tumor free and developing without any functional deficits. The video can be found here: https://stream.cadmore.media/r10.3171/2023.1.FOCVID22145.

Solitary Metastasis of Colon Adenocarcinoma Mimicking Colloid Cyst of the Third Ventricle.

Metastatic lesions to the choroid plexus, although far less common than colloid cysts, can present very similarly both symptomatically and radiographically. Choroid plexus metastases are most common in the lateral ventricles, however, when they occur in the third and fourth ventricles they may cause obstructive hydrocephalus typical of a colloid cyst lesion. Renal cell carcinoma is the most common primary cancer, but many rare primaries have been reported. When patients are presenting with symptoms typical of colloid cysts it is important to consider past oncological history and if past medical history is significant for cancer using MR spectroscopy may be valuable in distinguishing between cystic and metastatic lesions.

Ultra-early synostectomy and cranial remodeling orthoses in the management of craniosynostoses.

OBJECTIVE: The advent of endoscopic synostectomy has enabled early surgery for infants with craniosynostosis. Even though diagnosis is often made at birth, endoscopic synostectomy has traditionally been delayed until the infant is 3 months of age. There have been very few published reports of this procedure being performed in the early neonatal period. The authors discuss their experience with ultra-early endoscopic synostectomy, defined as an operation for infants aged 8 weeks or younger. METHODS: A retrospective analysis of infants who underwent operations at or before 8 weeks of age between 2011 and 2020 was done. RESULTS: Twenty-five infants underwent operations: 11 were 2 weeks of age or younger, 8 were between 3 and 4 weeks of age, and 6 were between 5 and 8 weeks of age. The infants weighed between 2.25 and 4.8 kg. Eighteen had single-suture synostosis, and 7 had multiple sutures involved. Of these 7, 4 had syndromic craniosynostosis. The average operative time was 35 minutes, and it was less than 40 minutes in 19 cases. The estimated operative blood loss was 25 ml or less in 19 cases; 5 infants required an intraoperative blood transfusion. In 1 child with syndromic multisuture craniosynostosis, the surgery was staged due to blood loss. Two children experienced complications related to the procedure: one had an incidental durotomy with skin infection, and the other had postoperative kernicterus. All infants were fitted for cranial remodeling orthoses following surgery. Three of the 25 infants required reoperations, with 2 patients with syndromic craniosynostosis needing repeat surgery for cranial volume expansion and cosmetic appearance. Another child with syndromic craniosynostosis is awaiting cranial expansion surgery. Follow-up varied between 6 months and 8 years. CONCLUSIONS: The data show that ultra-early synostectomy is safe and not associated with increased complications compared with surgery performed between 3 and 6 months of age. Infants with multisuture synostosis had increased operative time, required blood transfusion, and were more likely to require a second operation.

Bilateral Transverse Sinus Thrombosis following Craniotomy and Cranial Reconstruction for Sagittal Craniosynostosis.

INTRODUCTION: Craniotomy and cranial reconstruction is the most common procedure for children older than 6 months with craniosynostosis. Dural sinus thrombosis after this surgery has not been well reported in the literature. CASE PRESENTATION: This 2-year-old child underwent a bilateral craniotomy and cranial reconstruction for sagittal craniosynostosis. He had a partial thickness tear of the wall of the right transverse sinus which was uneventfully managed. Postoperative imaging showed evidence of bilateral thrombosis of the transverse sinus with a small occipital hemorrhage. He was started on low-molecular-weight heparin. Follow-up imaging showed nonprogression of the thrombosis. Four days later, he developed pulmonary hemorrhage, had an extended period of low oxygenation and hypotension with acute respiratory distress syndrome, and had to be ventilated for a prolonged period. Follow-up MRI showed evidence of extensive bilateral cortical hypointensities possibly due to hypoxemia. At the last follow-up, he continued to be grossly neurologically impaired. CONCLUSION: Thrombosis of the dural sinuses is a very rare occurrence after an extensive craniotomy and cranial reconstruction. However, it should be considered during the postoperative period and, if diagnosed, it should be treated with anticoagulants. Avoiding a direct sinus injury during reflection of the craniotomy flap and covering the exposed sinus with moist cottonoids during the surgery is advocated to prevent sinus thrombosis.

Reversible Progressive Multiple Cranial Nerve Paresis in the Isolated Fourth Ventricle following Placement of Fourth Ventricle Shunt: Case Report and Review of the Literature.

BACKGROUND/AIMS: Multiple lower cranial nerve paresis occurring after placement of a fourth ventricle shunt for an isolated fourth ventricle is an uncommon complication in the postoperative period. Of the various etiologies, direct brain stem injury by the catheter and rapid decompression of the fourth ventricle by the shunt causing traction on the cranial nerves have been reported in the literature. METHODS: We report the case of a 9-year-old girl with an isolated fourth ventricle who developed bilateral facial and multiple lower cranial nerve paresis with bilateral internuclear ophthalmoplegia a month after placement of a ventriculoperitoneal shunt. The postprocedure MRI showed a well-decompressed fourth ventricle with catheter tip located along the long axis of the fourth ventricle. RESULTS: She was managed non-operatively. She improved gradually in her cranial nerve paresis over the next 3 months and completely recovered at 9 months. CONCLUSION: We believe the reversible multiple cranial nerve neuropathies resulted from acute decompression of the fourth ventricle following the shunt insertion. A gradual decompression of the dilated fourth ventricle by an aqueductal stent or a high-pressure shunting system could prevent this potential complication.

Isolated Fourth Ventricle: To Shunt or Stent.

BACKGROUND: Of the various management options for isolated fourth ventricle (IFV), fourth ventriculoperitoneal shunts (FVPS) and aqueductal stents (AST) have been the most favored. Though effective, FVPS are often difficult to place and have higher complication rates than conventional ventricular shunts. OBJECTIVE: To assess the efficacy of AST in IFV and compare the outcome with FVPS. METHODS: Twenty-five patients surgically treated for IFV were analyzed. In all, a preoperative magnetic resonance imaging assessed the extent of aqueductal obstruction. Patients with an identified short-segment aqueductal stenosis were considered for AST placement; those with long-segment aqueductal obstruction underwent FVPS. RESULTS: Of the 25, 12 were symptomatic, while 13 were asymptomatic (progressive dilation of IFV in 9, persistent dilation with distortion of the brain stem in 4). In 3 with normal ventricles, the ventricles had to be dilated by externalizing the shunt before placing the stent. Nineteen underwent AST placement, whereas in 6 FVPS was performed. Sixteen patients underwent a simultaneous cerebrospinal fluid diversion procedure and fourth ventricular decompression. At follow-up (mean: 45 mo), stent migration was observed in 2 patients. In the FVPS group, 1 had 2 shunt revisions while another developed reversible cranial nerve paresis. Though a reduction of the IFV was observed with both procedures, the extent of reduction was more with FVPS. CONCLUSION: Both FVPS and AST are effective in managing IFV. The extent of aqueductal obstruction and degree of ventriculomegaly are often the deciding factors in choosing the management option.

Spontaneous Thrombosis and Subsequent Recanalization of a Developmental Venous Anomaly.

Developmental venous anomalies (DVA) are among the most common congenital malformations of the cerebral angioarchitecture. Spontaneous thrombosis of this entity is rare, and our review of the literature found only 31 reported cases of symptomatic spontaneous thrombosis of developmental venous anomalies. Here, we report a unique case describing the spontaneous thrombosis of a DVA leading to venous infarction and subsequent recanalization. The patient was a previously healthy 21-year-old male who presented with an acute onset of partial seizures. Following negative hypercoagulability studies and along with CT (computed tomography) and MR (magnetic resonance) imaging, the patient was treated with anticoagulant therapy and demonstrated complete functional recovery. Knowledge from our literature review of similar cases combined with the experience gained from this patient's treatment leads us to suggest that spontaneous DVA thrombosis and venous infarction generally has a good outcome despite initially devastating neurologic deficits. Additionally, the rarity of spontaneous DVA thromboses lends itself to the need to identify possible predisposing risk factors, chief amongst these being hypercoagulopathies.

Delineation of candidate genes responsible for structural brain abnormalities in patients with terminal deletions of chromosome 6q27.

Patients with terminal deletions of chromosome 6q present with structural brain abnormalities including agenesis of corpus callosum, hydrocephalus, periventricular nodular heterotopia, and cerebellar malformations. The 6q27 region harbors genes that are important for the normal development of brain and delineation of a critical deletion region for structural brain abnormalities may lead to a better genotype-phenotype correlation. We conducted a detailed clinical and molecular characterization of seven unrelated patients with deletions involving chromosome 6q27. All patients had structural brain abnormalities. Using array comparative genomic hybridization, we mapped the size, extent, and genomic content of these deletions. The smallest region of overlap spans 1.7 Mb and contains DLL1, THBS2, PHF10, and C6orf70 (ERMARD) that are plausible candidates for the causation of structural brain abnormalities. Our study reiterates the importance of 6q27 region in normal development of brain and helps identify putative genes in causation of structural brain anomalies.