Saeed Ansari

Split cord malformation associated with myelomeningocele

OBJECT The coexistence of split cord malformations (SCMs) and myelomeningoceles (MMCs) can be misdiagnosed or ignored and can cause spinal cord tethering. The authors investigated cases involving the coexistence of SCMs and MMCs in patients from the Childrens Hospital Medical Center in Tehran. METHODS Of the 330 patients with MMCs who underwent operations at the Childrens Hospital Medical Center between January 2001 and June 2005, 33 (10%) had an associated SCM. These 33 cases were retrospectively reviewed. RESULTS Eighteen patients (55%) were female, and the mean age of the patients at presentation was 2.9 months. In 17 of the 33 patients, the SCM occurred at the level of the neural placode. A Type I SCM was found in 26 children. Two patients had hypertrichosis. Eight patients had unilateral leg paresis. The MMC sac was located in the lumbar region in 14 cases. Two patients had double spinal dysraphism with meningoceles at the thoracic level. All patients underwent simultaneous repair of both lesions at the time of surgery. CONCLUSIONS Patients with MMCs, especially those with unusual manifestations such as unilateral paresis or skin lesions, should undergo a preoperative clinical examination to check for the presence of an SCM. Use of spinal magnetic resonance imaging can help in identifying the associated abnormalities. The neural placode and the rostral and caudal spinal cord segments should be carefully inspected for dysraphic lesions such as SCMs during the operation to repair the MMC. In this paper, the authors hope to show the wisdom of identifying these anomalies at the time of the initial repair of the MMC.

Spina bifida occulta: is it a predictor of underlying spinal cord abnormality in patients with lower urinary tract dysfunction?

OBJECT The purpose of this study was to evaluate the importance of spina bifida occulta in radiographs of children with lower urinary tract or bowel dysfunction. METHODS The authors prospectively investigated the presence of spinal cord abnormalities in 176 patients with functional urinary and bowel problems: 88 children with radiographic evidence of spina bifida occulta (SBO) and 88 age-and sex-matched controls. Each group included 46 boys and 42 girls (age range 5-14 years). Nocturnal enuresis, isolated diurnal enuresis, enuresis during both day and night, urinary tract infection, urinary frequency, encopresis, intractable constipation, and vesicoureteral reflux were assessed in all patients. Magnetic resonance (MR) images were obtained in all patients and evaluated for spinal cord abnormalities. Sacral ratios (SRs) were calculated on the basis of plain radiographs. RESULTS There was no statistically significant difference between the 2 groups on any of the clinical measures. The most common sites of SBO on radiographs were the S-1 (47%) and L-5 and S-1 (25%). The mean SRs (+/-standard deviations) in the SBO and control groups, respectively, were 0.64+/-0.45 and 0.68+/-0.51 (no statistically significant difference). Sacral agenesis was found in 17 children (7 in the SBO group and 10 in the control group, p=0.44). Abnormal MR imaging findings were observed in 9 children (10.22%) in the SBO group and 3 (3.4%) in the control group. Abnormalities included tethered spinal cord in 5 children, syringomyelia in 4, and club-shaped conus in 2. No significant association was found between the presence of SBO and spinal cord abnormalities identified on MR images (p=0.13, paired t-test). CONCLUSIONS Among children with functional bowel and urinary problems, there was no statistically significant difference in the prevalence of abnormal spinal MR imaging findings in those with radiographic SBO and an age- and sex-matched control group. Spina bifida occulta was not shown to be a reliable indicator of spinal cord structural abnormalities. Its probable role as a finding associated with spinal cord dysfunction remains unclear.

Risk factors associated with occipital encephalocele: a case-control study. Clinical article.

OBJECT An encephalocele is characterized by congenital herniation of the brain tissue and/or meninges through a skull defect. The underlying cause is complex and not fully understood, but environmental agents are suspected. The authors aimed to determine the known risk factors for encephaloceles. METHODS Potential risk factors were studied in 31 children with encephaloceles who had been referred to the outpatient clinic. At the same time, 31 children with non-CNS anomalies were randomly selected from the same hospital as a control group. Both cohorts were assessed in person through interviews with the mothers. RESULTS Most children with encephaloceles were female. There was no significant risk factor in the case group as compared with controls, although the families of patients with encephaloceles had a better economic status (p=0.03) and the fathers had a higher mean age. CONCLUSIONS Although the authors of this study could not identify any significant risk factors for encephaloceles, environmental factors can still be mentioned as probable etiological elements. Additional studies with larger sample sizes and more comprehensive evaluations are required to confirm the role of environmental or genetic factors to prevent the occurrence of encephaloceles.

The cerebral arterial circle (circulus arteriosus cerebri): an anatomical study in fetus and infant samples.

Background: Many studies have investigated the variations in the anatomy of each segment of the cerebral arterial circle while a few have addressed the variations of the cerebral arterial circle as a whole. Methods: Thirty brains of recently deceased Iranian infants and fetuses were dissected. The dissection process was filmed and digitized so as to be readily available for further studies. The variations of the circle as a whole and segmental variations were evaluated. Results: Variants with uni- and bilateral hypoplasia of posterior communicating arteries (PcoAs) were the most common in our study, similar to previous works. No aplasia of the precommunicating part of the anterior cerebral artery (A1), the precommunicating part of the posterior cerebral artery (P1) and anterior communicating artery was seen. Hypoplasia of the right and left PcoA was observed in 8 and 5 cases, respectively. Aplasia of the right PcoA was found in 16.6% and of the left PcoA in 3.3%. Conclusion: In this study, we confirmed the previously described finding that the symmetrical, circular configuration of the circulus arteriosus cerebri is present in only about 42.1%. The main differences between the fetal and adult disposition are the diameter of the PcoA and the circular part of the posterior cerebral artery. According to previous studies, the fetal brain older than 4 months has anatomical characteristics very similar to the adult’s circle; our finding was mostly similar to adult samples as most samples were from infants, not fetuses.

Randomized clinical trial of acetazolamide administration and/or prone positioning in mitigating wound complications following untethering surgeries

OBJECTIVE No evidence-based guideline has been approved for the postoperative management of pediatric patients with tethered cord syndrome (TCS). The purpose of this randomized clinical trial was to evaluate the effectiveness of prone positioning and acetazolamide administration on complication rates following spinal cord untethering surgeries. METHODS From October 2012 to February 2015, patients with a primary diagnosis of TCS who were admitted to the Childrens Medical Center Hospital of Iran were randomly allocated to 1 of 4 intervention modality groups postoperatively: 1) Group A, acetazolamide administration for 10 days; 2) Group B, prone positioning for 10 days; 3) Group C, acetazolamide administration and prone positioning for 10 days; and 4) Group D, no intervention. CSF leakage, CSF collection, wound dehiscence, operative site infection, and secondary surgical wound repair were considered failure. RESULTS A total of 161 patients were enrolled in this study (Group A, n = 39 [24.2%]; Group B, n = 41 [25.5%]; Group C, n = 39 [24.2%]; and Group D, n = 42 [26.1%]). The overall failure rate was 12.42% (20 patients). Complication rates through pooled analyses were as follows: CSF leakage (n = 9, 5.6%), CSF collection (n = 12, 7.5%), wound dehiscence (n = 2, 1.2%), and infection of operation site (n = 3, 1.9%). Two patients (1.2%) required surgical secondary wound repair due to complications. CSF leakage and collection rates were significantly lower in patients who underwent prone positioning (p = 0.042 and 0.036, respectively). The administration of acetazolamide, either isolated or in combination with prone positioning, not only could not significantly lower the complication rates, but also added the burden of side effects. CONCLUSIONS The current study demonstrates the possible role of prone positioning in mitigating the complication rates subsequent to untethering surgeries. Clinical trial registration no.: NCT01867268 ( clinicaltrials.gov ).

Breastfeeding: a potential protective factor against ventriculoperitoneal shunt infection in young infants

OBJECT Previous studies have shown nutritional benefits of breastfeeding for a childs health, especially for protection against infection. Protective factors in human milk locally and systemically prevent infections in the gastrointestinal as well as upper and lower respiratory tracts. It remains unclear whether breastfeeding protects infants against ventriculoperitoneal (VP) shunt infection. METHODS A cohort study was conducted from December 2003 to December 2006 at Childrens Hospital Medical Center in Tehran, Iran. A total of 127 infants with hydrocephalus who were treated using a VP shunt in the first 6 months of life were enrolled. Each infants breastfeeding method was classified as either exclusively breastfed (EBF), combination feedings of breast milk and formula (CFBF), or exclusively formula-fed (EFF). Infants were followed up to determine the occurrence of shunt infection within 6 months after operation. Statistical analysis was performed using survival methods. RESULTS Infants ranged in age from 4 to 170 days at the time of shunt insertion (mean 69.6 days), and 57% were males. Regarding the breastfeeding categories, 57.5% were EBF, 25.2% were CFBF, and 17.3% were EFF. During the follow-up, shunt infection occurred in 16 patients, within 15 to 173 days after shunt surgery (median 49 days). The 6-month risk of shunt infection was 8.5% (95% confidence interval [CI] 4-18%) in the EBF group, 16.5% (95% CI 7-35%) in the CFBF group, and 26.0% (95% CI 12-52%) in the EFF group. There was no statistically significant difference between these 3 groups (p=0.11). The trend test showed a significant trend between the extent of breastfeeding and the risk of shunt infection (p=0.035), which persisted even after adjustment for potential confounding variables (hazard ratio=2.01, 95% CI 1.01-4). CONCLUSIONS This study supports the protective effect of breastfeeding against shunt infection during the first 6 months of life and the presence of a dose-response relationship, such that the higher the proportion of an infants feeding that comes from human milk, the lower the incidence of shunt infection. Encouraging mothers of infants with VP shunts to breastfeed exclusively in the first 6 months of life is recommended.