Mostafa El Khashab

Shunt complications in children with myelomeningocele: effect of timing of shunt placement: Clinical article

OBJECT There has been controversy over whether CSF shunt insertion simultaneously with repair of myelomeningocele (MMC) might increase shunt-related complications. The purpose of this study was to evaluate shunt complication rates in patients who underwent concurrent MMC surgery and shunt placement and compare them to the rates in patients treated with shunt placement in a separate procedure. METHODS The authors retrospectively reviewed the outcome of shunt placement in 127 patients with MMC who were followed up for >or=1 year after shunt surgery. In 65 patients shunt surgery was performed in a second operation after MMC repair and in 46 the 2 procedures were performed concurrently. In 7 patients shunt placement was the initial surgery, and in 9 it was the only procedure performed. The patients were evaluated for shunt complications. RESULTS There was no statistically significant between-groups difference in age at which patients underwent shunt placement. The overall rates of shunt infection and shunt malfunction were 16.5 and 39.4%, respectively. There was a high rate of shunt infection and mortality in those patients treated with CSF shunting only. There was no statistically significant difference between complication rates in patients in whom the 2 procedures were performed concurrently and those who underwent separate operations. CONCLUSIONS The order in which myelomeningocele repair and shunt placement were carried out did not have a significant effect on the rate of shunt complications. Thus, when indicated these procedures can be performed concurrently with a level of risk comparable to that associated with delayed shunt placement.

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.

Cranial Dermal Sinus: Presentation, Complications and Management

Objective: Cranial dermal sinus is a very rare anomaly mostly found in the midline occipital area, presenting with diverse clinical features from asymptomatic dermal sinus to symptomatic situations including meningitis, dermoid tumor or abscess. Methods: Medical records of 18 patients with 19 cranial dermal sinuses operated at the Children’s Hospital Medical Center in Tehran from 1999 to 2011 were retrospectively studied. Their presentations, accompanying anomalies, radiological and surgical findings were evaluated. Results: Patients were aged 1 month to 6 years and were followed for 2–12 years. Most sinuses were located in the midline occipital region. Infection was the most common reason for referral (50%) followed by skin dimple (33.3%). Creamy white discharge was found in 50% of patients. Posterior fossa abscess, hydrocephalus and meningitis were observed in 38.9, 38.9 and 22.2% of children, respectively. All sinus tracts with associated dermoid tumors or abscesses were removed en bloc to decrease the risk of recurrence. Conclusion: The sinus tracts are a portal of entry for pathogens which result in serious complications of meningitis or abscess. In this series, 67% of children presented with serious symptoms; therefore, we recommend prophylactic surgery in all cranial dermal sinuses. The exact time of surgery is not obvious as the natural course of the disease is unknown. If any symptom is observed surgery should be done as soon as possible. For asymptomatic sinuses identified by the pediatrician during a regular visit prophylactic surgery is recommended as soon as the child can tolerate operation.

Risk Factors Associated with Craniosynostosis: A Case Control Study

Background: Craniosynostosis is a relatively common congenital abnormality. The underlying etiology and most probable risk factors of nonsyndromic craniosynostosis are unknown. We conducted a study to identify the risk factors for craniosynostosis. Materials and Methods: In this case-control project, 70 children with craniosynostosis (syndromic or nonsyndromic) hospitalized at the Childrens Hospital Medical Center from September 2010 to 2011 were studied for the potential risk factors. At the same time, 70 age- and sex-matched children hospitalized with other congenital anomalies were reviewed. Information from case and control groups was obtained via personal interviews with parents of patients and a prepared checklist was filled out for each child. Results: The mean (±SE) age at admission time in the case-control group was 13 ± 1.95 (1.5-96) months, and almost half of them were boys (n = 37 or 53%). The most frequent sutures involved in craniosynostosis were coronal (n = 30 or 42.9%) and then metopic sutures (n = 16 or 22.9%), multiple (n = 12 or 17.1%), sagittal (n = 11 or 15.7%) and lambdoid (n = 1 or 1.4%). In the case group, maternal diabetes mellitus and thyroid disease were found in 8 (11.6%) and 6 (8.6%) patients, respectively. The most frequent medication used by mothers (n = 66 or 94%) in the case group during pregnancy were vitamins (including iron supplements, omega 3, folic acid and multivitamins). Conclusion: A positive family history of craniosynostosis [odds ratio (OR) 19.01 and 95% confidence interval (CI) 2.24-160.7] and using clomiphene citrate for infertility (OR 12.71 and 95% CI 1.42-113.6) were the strongest independent risk factors for craniosynostosis. More comprehensive studies with a larger sample size are required to confirm the role of environmental factors in order to decrease the occurrence of craniosynostosis.

Recurrent meningitis caused by cervico-medullary abscess, a rare presentation.

BackgroundRecurrent bacterial meningitis is usually a complication of cranial anatomical defect or the result of impaired humoral immunity, notably, defects of the complement system and agammaglobulinemia. It could present a diverse range of symptoms.DiscussionMeningitis as a presentation of intramedullary abscess is not common. Here we present a 7-year-old boy with recurrent meningitis as the first manifestation of an underlying upper cervical cord abscess.

Sacrococcygeal teratoma with intradural extension: case report

Intradural sacrococcygeal teratoma (SCT) is a rare entity that has been reported in only a few cases previously. The authors present the case of a 2-week-old, otherwise healthy neonate with a mass in the buttock. The imaging findings and the high level of serum alpha-fetoprotein were highly suggestive of SCT. On operation the authors found intradural extension of the teratoma. The lesion was managed successfully without any remaining sequelae. The authors briefly review the currently proposed etiology regarding teratoma formation and the intradural extension of SCT.

Risk factors for latex sensitization in young children with myelomeningocele. Clinical article.

OBJECT Patients with myelomeningoceles (MMCs) are at increased risk of latex allergy and sensitization. Number of surgeries and history of atopy are known risk factors. The object of this study was to evaluate the role of diagnostic procedures and nonsurgical treatments in latex sensitization in young patients with MMC. METHODS Seventy-three children with MMC were included in the study. For each child a questionnaire was administered and serum determination of IgE was performed, and 62 children underwent skin prick tests (SPTs), 60 of which had reliable results. Multivariate logistic regression modeling was performed, using latex sensitization as the dependent variable. RESULTS The mean age of the 73 patients was 3.8 years. The SPT results were positive in 30.6%, whereas results of testing for latex-specific IgE were positive in only 8.2%. In univariate analysis, history of untethering, barium enema, and number of clean intermittent catheterizations (CICs) per day were significantly associated with positive results on the SPT. Although the number of surgical procedures was significantly higher in patients who had shunts, no significant relationship between the presence of a shunt and latex sensitization was seen. CONCLUSIONS The young age of the patients in this study may account for the low prevalence of latex sensitization that was found. In young patients with MMC, the numbers of CICs per day, a history of untethering, circumcision, and a barium enema performed without latex-free equipment could be risk factors for latex sensitization. The use of latex-free gloves in all procedures performed in these cases, nonlatex polyvinyl chloride catheters in CIC, and ordinary nonballoon tips in barium enemas could decrease the risk of sensitization.

Identical twins with lumbosacral lipomyelomeningocele

Lipomyelomeningocele, a congenital spine defect, is presented as skin-covered lipomatous tissue that attaches to the cord in different ways according to its subtypes. Unlike other types of neural tube defects, the exact cause of this birth defect has not been confirmed yet, but it is proposed to be a multifactorial disease with involvement of both genetic and environmental factors. The authors describe identical twins with lipomyelomeningocele of the same subtype and location without any familial history of similar abnormality. Therefore, the same genetic and/or environmental risk factors could have played a part in their condition.

Possible causes of seizure after spine surgery.

Seizure after laminectomy for spinal procedure is very rare and has not been reported after lipomyelomeningocele surgery beforehand. Here, two cases of seizure following laminectomy for lipomyelomeningocele are reported. The exact etiology of the event is unknown but anesthetic material, pneumocephalus, intracranial hypotension subsequent to cerebrospinal fluid leakage after spinal procedures, spinal-induced seizure and the potential toxic effect of fat molecules could be considered.