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Yosef Ellenbogen, Karanbir Brar, Kaiyun Yang, Yung Lee and Olufemi Ajani

OBJECTIVE

Pediatric hydrocephalus is a significant contributor to infant morbidity and mortality, particularly in developing countries. The mainstay of treatment has long been shunt placement for CSF diversion, but recent years have seen the rise of alternative procedures such as endoscopic third ventriculostomy (ETV), which provides similar efficacy in selected patients. The addition of choroid plexus cauterization (CPC) to ETV has been proposed to increase efficacy, but the evidence of its utility is limited. This systematic review and meta-analysis aimed to determine the efficacy and safety of ETV+CPC in comparison to ETV alone for the treatment of pediatric all-cause hydrocephalus.

METHODS

MEDLINE, Embase, Cochrane CENTRAL, ClinicalTrials.gov, and ICRCTN databases were searched from conception through to October 2018 for comparative studies including both ETV+CPC and ETV in a pediatric population. The primary outcome was success rate, defined as no secondary procedure required for CSF diversion; secondary outcomes included time to failure, mortality, and complications. Data were pooled using random-effects models of meta-analysis, and relative risk (RR) was calculated.

RESULTS

Five studies were included for final qualitative and quantitative analysis, including 2 prospective and 3 retrospective studies representing a total of 963 patients. Overall, there was no significant difference in success rates between ETV and ETV+CPC (RR 1.24, 95% CI 0.88–1.75, p = 0.21). However, a subgroup analysis including the 4 studies focusing on African cohorts demonstrated a significant benefit of ETV+CPC (RR 1.38, 95% CI 1.08–1.78, p = 0.01). There were no notable differences in complication rates among studies.

CONCLUSIONS

This systematic review and meta-analysis failed to find an overall benefit to the addition of CPC to ETV; however, a subgroup analysis showed efficacy in sub-Saharan African populations. This points to the need for future randomized clinical trials investigating the efficacy of ETV+CPC versus ETV in varied patient populations and geographic locales.

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Olufemi A. Ajani, Ghanem Al Sulaiti and Issam Al Bozom

Pilomyxoid astrocytomas (PMAs) are low-grade (WHO Grade II) tumors for which the imaging features are similar to pilocytic astrocytomas (PAs), but for which histological features and the clinical course differ. They are classified as a variant of PA. They have only been recently recognized, and they behave more aggressively than PAs. Most cases occur in the hypothalamic-chiasmatic area, although they may be located in any part of the neuraxis. Posterior fossa PMAs are very rare. The authors report a case of a 2-year-old girl with cerebellar PMA.

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Abbas Amirjamshidi and Meysam Alimohammadi

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Mohsin Ali, Michael Bennardo, Saleh A. Almenawer, Nirmeen Zagzoog, Alston A. Smith, Dyda Dao, BHSc, Olufemi Ajani, Forough Farrokhyar and Sheila K. Singh

OBJECT

Although intracranial arachnoid cysts are a common incidental finding on pediatric brain imaging, only a subset of patients require surgery for them. For the minority who undergo surgery, the comparative effectiveness of various surgical approaches is debated. The authors explored predictors of surgery and compared operative techniques for pediatric patients with an intracranial arachnoid cyst seen at a tertiary care center.

METHODS

The authors reviewed records of pediatric patients with an intracranial arachnoid cyst. For each patient, data on baseline characteristics, the method of intervention, and surgical outcomes for the initial surgery were extracted, and cyst size at diagnosis was calculated (anteroposterior × craniocaudal × mediolateral). Baseline variables were analyzed as predictors of surgery by using logistic regression modeling, excluding patients whose surgery was not related to cyst size (i.e., those with obstructive hydrocephalus secondary to the cyst compressing a narrow CSF flow pathway or cyst rupture/hemorrhage). Data collected regarding surgical outcomes were analyzed descriptively.

RESULTS

Among 83 pediatric patients with an intracranial arachnoid cyst seen over a 25-year period (1989–2013), 27 (33%) underwent surgery; all had at least 1 cyst-attributed symptom/finding. In the multivariate model, age at presentation and cyst size at diagnosis were independent predictors of surgery. Cyst size had greater predictive value; specifically, the area under the curve for the receiver-operating-characteristic curve was 0.89 (95% CI 0.82–0.97), with an ideal cutoff point of ≥ 68 cm3. This cutoff point had 100% sensitivity (95% CI 79%–100%), 75% specificity (95% CI 61%–85%), a 53% positive predictive value (95% CI 36%–70%), and a 100% negative predictive value (95% CI 91%–100%); the positive likelihood ratio was 4.0 (95% CI 2.5–6.3), and the negative likelihood ratio was 0 (95% CI 0–0.3). Although the multivariate model excluded 7 patients who underwent surgery (based on prespecified criteria), excluding these 7 cases did not change the overall findings, as shown in a sensitivity analysis that included all the cases. Descriptive results regarding surgical outcomes did not indicate any salient differences among the surgical techniques (endoscopic fenestration, cystoperitoneal shunting, or craniotomy-based procedures) in terms of symptom resolution within 6 months, need for reoperation to date, cyst-size change from before the operation, morbidity, or mortality.

CONCLUSIONS

The results of these exploratory analyses suggest that pediatric patients with an intracranial arachnoid cyst are more likely to undergo surgery if the cyst is large, compresses a narrow CSF flow pathway to cause hydrocephalus, or has ruptured/hemorrhaged. There were no salient differences among the 3 surgical techniques for several clinically important outcomes. A prospective multicenter study is required to enable more robust analyses, which could ultimately provide a decision-making framework for surgical indications and clarify any differences in the comparative effectiveness of surgical approaches to treating pediatric intracranial arachnoid cysts.