Improving Infant Hydrocephalus Outcomes in Uganda: A Longitudinal Prospective Study Protocol for Predicting Developmental Outcomes and Identifying Patients at Risk for Early Treatment Failure after ETV/CPC

Infant hydrocephalus poses a severe global health burden; 80% of cases occur in the developing world where patients have limited access to neurosurgical care. Surgical treatment combining endoscopic third ventriculostomy and choroid plexus cauterization (ETV/CPC), first practiced at CURE Children’s Hospital of Uganda (CCHU), is as effective as standard ventriculoperitoneal shunt (VPS) placement while requiring fewer resources and less post-operative care. Although treatment focuses on controlling ventricle size, this has little association with treatment failure or long-term outcome. This study aims to monitor the progression of hydrocephalus and treatment response, and investigate the association between cerebral physiology, brain growth, and neurodevelopmental outcomes following surgery. We will enroll 300 infants admitted to CCHU for treatment. All patients will receive pre/post-operative measurements of cerebral tissue oxygenation (SO2), cerebral blood flow (CBF), and cerebral metabolic rate of oxygen consumption (CMRO2) using frequency-domain near-infrared combined with diffuse correlation spectroscopies (FDNIRS-DCS). Infants will also receive brain imaging, to monitor tissue/ventricle volume, and neurodevelopmental assessments until two years of age. This study will provide a foundation for implementing cerebral physiological monitoring to establish evidence-based guidelines for hydrocephalus treatment. This paper outlines the protocol, clinical workflow, data management, and analysis plan of this international, multi-center trial.

Torcular dural sinus malformation

The dural sinus malformation (DSM) are rare congenital vascular anomalies with variable anatomic features, clinical conditions, and outcomes. There are two forms of disease: a lateral subtype, affecting the jugular bulb with associated high flow arteriovenous fistula; and a midline subtype, called torcular DSM. The torcular subtype is more common and characterized by a giant dural sinus lake involving the confluens sinuum (torcular Herophili). We present a case of a 28-year-old woman with an ultrasound at 32 weeks’ gestation showing a fetal intracranial thrombosed lesion, measuring 6x4 cm. An elective c-section was performed at 38 weeks’ gestation, and a male neonate was born. He remained asymptomatic, and a brain MRI performed 9 days later showed a thrombosed midline mass due to a torcular DSM. The outcome with conservative treatment was favorable, and further development was unremarkable. The 2-year follow-up imaging demonstrated spontaneous involution of the clot. The torcular DSM is frequently diagnosed prenatally as an intraluminar lake thrombosis, and differential diagnosis include tumors, subdural collections, vein of Galen aneurysmal malformations, pial malformations, arachnoid and dermoid cysts. A literature review involving 126 patients with torcular DSM found an overall mortality of 22.1%. Torcular DSM seems to have better prognosis than those having dural arteriovenous shunts, and the worst outcomes were associated to brain damage and patent feeders. In addition, antenatal diagnosis and thrombosis of pouch have good prognosis, possibly because the clot may spontaneously obliterate the fistulas. Treatment options must be individualized, and include conservative, embolization, ventriculoperitoneal shunt and endoscopic third ventriculostomy. Although systemic anticoagulation is controverse in infants, it should be considered for patients with sinovenous thrombosis affecting outflow pathways, especially those aggravated by venous congestion. Intervention is recommended for grade III patients because they progress to grade IV, and endovascular treatment is the gold standard therapy due to the risk of fatal intraoperative exsanguination. Whenever possible, embolization should be prioritized over hydrocephalus treatment because it may avoid ventriculoperitoneal shunt. Close follow-up imaging is recommended to detect any changes, with lesion reduction indicating favorable outcome.

Endoscopic Third Ventriculostomy: Our Experience of Consecutive 50 Cases at a Tertiary Care Center

Objective The aim of this study was to do a retrospective analysis of the various neurosurgical pathologies where endoscopic third ventriculostomy (ETV) was used and to evaluate the outcome and prognosis. Methods The retrospective data collection was done for the patients who underwent ETV with or without other adjunct procedures; the results were prepared for clinical presentation, diagnosis, surgical approach, and surgical goal; and success rate and prognosis were analyzed and compared with other studies. Results A total of 50 patients were included in the study, with overall success rate of ETV as 88%; aqueductal stenosis was the most common indication where ETV was used; headache and vomiting were the most common presenting complaints followed by ataxia and visual blurring; and ETV provided flexibility in its use with biopsy, abscess drainage, temporary external ventricular drain placement, etc. Conclusion ETV being superior to ventriculo-peritoneal shunt for obstructive hydrocephalus provides flexibility in its use and possibly is a useful adjunct to prevent postoperative hydrocephalus after endoscopic intraventricular surgery; proper case selection in accordance to ETV success score yields a better success rate. In experienced hands with proper precautions, perioperative complications can be kept at minimum. Wherever possible, in cases of obstructive hydrocephalus, especially in patients >1 year of age, ETV should be the treatment of choice. We recommend a proper case selection, including preoperative detailed reading of sagittal magnetic resonance imaging scan, to improve the success rate with less complication.

Surgical management of quadrigeminal cistern arachnoid cysts: case series and literature review

OBJECTIVE Quadrigeminal cistern arachnoid cysts (QACs) are congenital lesions that can cause pineal region compression and obstructive hydrocephalus when sufficiently large. Management of these cysts is controversial and rates of reintervention are high. Given the limited data on the management of QACs, the authors retrospectively reviewed 20 years of cases managed at their institution and performed a literature review on this topic. METHODS The authors performed a retrospective analysis of patients treated for QAC at their institution between 2001 and 2021. They also performed a literature review of studies published between 1980 and 2021 that reported at least 5 patients treated for QACs. Patient characteristics, radiographic findings, management course, and postoperative follow-up data were collected and analyzed. RESULTS A total of 12 patients treated for a QAC at the authors’ institution met the inclusion criteria for analysis. Median age was 9 months, mean cyst size was 5.1 cm, and 83% of patients had hydrocephalus. Initial treatment was endoscopic fenestration in 92% of these patients, 27% of whom had an endoscopic third ventriculostomy (ETV) performed concurrently. Reintervention was required in 42% of patients. Cases that required reintervention had a statistically significant lower median age at the initial intervention (5 months) than the cases that did not require reintervention (24.33 months; p = 0.018). There were no major complications. At a mean follow-up of 5.42 years, 83% of patients had improvement or resolution of their symptoms. A literature review revealed 7 studies that met the inclusion criteria, totaling 108 patients with a mean age of 8.8 years. Eighty-seven percent of patients had hydrocephalus at presentation. Ninety-two percent of patients were initially treated with endoscopic fenestration, 44% of whom underwent concurrent ETV. Complications occurred in 17.6% of cases, and reintervention was required in 30.6% of cases. The most frequent reason for reintervention was untreated or unresolved hydrocephalus after the initial procedure. CONCLUSIONS Endoscopic fenestration is the most common treatment for QACs. While generally safe and effective, there is a high rate of reintervention after initial treatment of QACs, which may be associated with a younger age at the first intervention. Additionally, identifying patients who require initial treatment of hydrocephalus is critically important, as the literature suggests that untreated hydrocephalus is a common cause of reintervention.

Clinical Impact of the Patient Age Following Endoscopic Third Ventriculostomy: A Review

Background: Clinically erratic cerebrospinal fluid (CSF) outflow within brain ventricles was described as non-communicating and communicating hydrocephalous. Neurosurgical patients are commonly seen with increased intracranial pressure due to increased CSF accumulation. (1) Initially scientists followed ventriculoperitoneal shunt an inexpensive and available technique to treat hydrocephalous disorders. Due to least successful outcomes, Scientists used other alternative endoscopic techniques. These endoscopic techniques include aqueductal endoscopic stenting, aqueductoplasty and third ventriculostomy (2). Defining success rate of Endoscopic third ventriculostomy clinical and radiographic analysis are considered gold standard. Objectives: Our review aims to assess comparative role of patient age, patient selection, hydrocephalus etiology, surgical advancement and epigenetic treatment strategies following endoscopic third ventriculostomy focusing hydrocephalous. The main objectives of current study, to critically analyze available literature till to date and a step forward for the development of standard surgical protocols. Methodology: Clinical studies from Level I-IV published in English language focusing human subject only were only considered by retrieving NCBI/PubMed, Medline databases. Studies purely focusing third ventriculostomy in subject of patient age, hydrocephalus etiology and age associated complications were processed further. Objective based data inspection approach was followed. From each included study focusing third ventriculostomy detailed information demographic information was collected. Further data analysis was done by using SAS and multiple tools of Microsoft Excel Version 2010. Outcomes: From total 11 included studies specifically focusing hydrocephaly treatment via endoscopic third ventriculostomy, 757 patients with equal 1:1 male and female gender ratio and 125 cases of unknown gender were considered. Current study highlighting efficacy of Endoscopic third ventriculostomy at the rate of 77% effective treating obstructive hydrocephalus. On basis of etiological concerns about 36.32 % cases hydrocephalous cases were linked with encephalitis, underweight birth and nonspecific etiology. However, 24 % cases of hydrocephalus were led by group of tumors including, Ependymoma, Medulloblastoma, Meningioma, Cerebella pontine angle Tumors and Pineal gland carcinomas as well. 154 (20%) cases of obstructive hydrocephalus were associated with aqueduct stenosis, 16% cases reporting hemorrhage and only 23 cyst cases. Conclusion and future recommendations: Endoscopic third ventriculostomy (ETV) is a safe and effective (77%) treatment option for the treatment of obstructive hydrocephalus among all age groups. However, specifically highest efficacy was noted among patients of 15-30-year age group. Understanding disease etiology and patient selection criteria both are considered potential components following successful endoscopic third ventriculostomy. We highly recommend further research following a universal age criteria and reporting results in distinct age category to standardize ETV treating hydrocephaly efficiently. Key words: Endoscopic third ventriculostomy, ETV, hydrocephalous, aqueductal endoscopic stenting, aqueductoplasty.

Successful management of symptomatic hydrocephalus using a temporary external ventricular drain with or without endoscopic third ventriculostomy in pediatric patients with germinoma

OBJECTIVE The objective of this study was to describe the management of hydrocephalus in a cohort of pediatric patients with germinoma. METHODS The authors conducted a retrospective chart review of patients with germinoma and symptomatic hydrocephalus treated at the Hospital for Sick Children between 2002 and 2020. Descriptive data included tumor location, CSF diversion procedure (external ventricular drain [EVD], endoscopic third ventriculostomy [ETV], ventriculoperitoneal [VP] shunt) and outcomes. The frontooccipital horn ratio (FOR) method was used to determine the presence of ventriculomegaly. RESULTS Of 39 patients with germinoma, 22 (73% male) had symptomatic hydrocephalus at diagnosis (11 pineal, 4 suprasellar, and 7 bifocal). Management of hydrocephalus included EVD (n = 5, 22.7%), ETV (n = 5, 22.7%), and combination ETV and EVD (n = 7, 31.8%), whereas 5 patients (22.7%) did not undergo surgical intervention. The median FOR at diagnosis was 0.42 (range 0.38–0.58), which correlated with moderate to severe ventriculomegaly. Carboplatin and etoposide–based chemotherapy induced fast tumor shrinkage, avoiding CSF diversion (n = 5) and resolving hydrocephalus with a transient EVD (n = 5). The median duration until EVD removal was 7 days (range 2–10 days). Two of 12 patients with EVD ultimately required a VP shunt. Kaplan-Meier overall survival was 100% and progression-free survival was 96.4% at a median follow-up of 10.4 years. CONCLUSIONS Timely initiation of chemotherapy is imperative to rapidly reduce tumor bulk in children with germinoma and limits the need for VP shunt insertions. In children in whom CSF diversion is required, hydrocephalus may be successfully managed with a temporary EVD ± ETV.

Hydrocephalus in achondroplasia: efficacy of endoscopic third ventriculostomy

OBJECTIVE Ventriculoperitoneal shunts (VPSs) for hydrocephalus in patients with achondroplasia are known to have a higher failure rate than in other hydrocephalus populations. However, the etiology of hydrocephalus in this group is considered “communicating,” and, therefore, potentially not amenable to endoscopic third ventriculostomy (ETV). ETV has, nonetheless, been reported to be successful in a small number of patients with achondroplasia. The authors aimed to investigate the long-term results of ETV in this population. METHODS Patients with achondroplasia who had undergone surgical treatment for hydrocephalus (ETV or VPS placement) were identified. In patients who had undergone ETV, medical records and neuroimages were reviewed to determine ventricular volumes and frontal and occipital horn ratios (FOHRs) pre- and postoperatively, as well as the incidence of surgical complications and reoperation. Patients who underwent VPS placement were included for historical comparison, and their medical records were reviewed for basic demographic information as well as the incidence of surgical complications and reoperation. RESULTS Of 114 pediatric patients with achondroplasia referred for neurosurgical consultation, 19 (17%) were treated for hydrocephalus; 10 patients underwent ETV only, 7 patients underwent VPS placement only, and 2 patients had a VPS placed followed by ETV. In patients treated with ETV, ventricular volume and FOHRs were normal, if measured at birth, and increased significantly until the time of the ETV. After ETV, all patients demonstrated significant and sustained decreases in ventricular measurements with surveillance up to 15 years. There was a statistically significant difference in rates of repeat CSF surgery between the ETV and VPS cohorts (0/12 vs 7/9, p < 0.001). CONCLUSIONS ETV was efficacious, safe, and durable in the treatment of hydrocephalus in patients with achondroplasia. Although many studies have indicated that hydrocephalus in these patients is “communicating,” a subset may develop an “obstructive” component that is progressive and responsive to ETV.

A bi-foraminal craniometric-guided approach to endoscopic third ventriculostomy and biopsy of a pineal tumour

Background: Pineal tumors are very rarely encountered, with an incidence of <1% of intracranial lesions in adults. Life-threatening hydrocephalus due to obstruction of the third ventricle can result from the location of these tumours. Endoscopic third ventriculostomy (ETV) and tumor biopsy is a safe and feasible strategy, particularly if the tumor appears benign. This mitigates the high risks of uncontrollable venous bleeding from open and stereotactic biopsies. While typically performed using either ipsilateral single or dual bur holes, the location of the tumor may require modifications to the standard endoscopic techniques. Case Description: A 55-year-old male presented with signs of intracranial hypertension and was found to have obstructive hydrocephalus due to a pineal tumour. The tumour displayed a right-sided dominance when the pre-operative imaging was assessed, which would risk forniceal injury if biopsied via a right-sided burr hole. Craniometric measurements revealed a superior trajectory to the tumour via the left foramen of Monro. A biforaminal approach was performed, with a traditional ETV using a right coronal bur hole and biopsy via a left frontal bur hole. This minimized forniceal stretching and allowed a safe biopsy. Conclusion: The bi-foraminal approach has not been widely described in the literature but can potentially avoid morbidity with biopsy in patients with right-sided pineal tumours. We believe this technique should be considered, particularly in low-resource settings where neuroendoscopy is not commonly done, and where the use of ipsilateral single or dual-bur holes may lead to forniceal injury.