Is shunt series X-ray necessary before revision of obstructed ventriculoperitoneal shunt?

The purpose of this study is to estimate whether routine preoperative shunt series [POSS] lead to clinically relevant new information, that helps in the management of ventriculoperitoneal shunt [VPS] obstruction, focusing on its role in diagnosing mechanical causes of shunt obstruction. Retrospective review of 64 consecutive patients who underwent revision of obstructed VPS in the neurosurgery division, at King Saud University, Riyadh, Saudi Arabia, between June 2002 and December 2011, assessing the proportion and impact of abnormal findings in the POSS. Sixty-nine POSS were performed for 64 patients before revision of obstructed VPS. Their mean age was 11.8 years, and 25 patients among them were females. Seventeen [24.6%] POSS had abnormal finding, that was statistically significant [P =0.005], and only 10 of them influenced the surgical technique or choice of therapeutic procedure [P =0.0001]. Positive findings were in the form of; broken/disconnected catheter [n =4], intra-abdominal migration of peritoneal catheter [n= 4], coiled/extra-peritoneal distal catheter [n= 2], short peritoneal end [n=1], and retained catheter/more than one shunt [n= 6]. However, majority of shunt series [75.4%] were normal. Routine shunt series X-ray alone is not a diagnostic tool for shunt malfunction, and POSS should be reserved for patients with proven shunt failure on CT or MRI scan. There was a significant impact of POSS on the operative decision for those undergoing revision for VPS obstruction.

Split cord malformation associated with spinal open neural tube defect

To illustrate the clinical and radiological findings of split cord malformation [SCM] in patients with spinal open neural tube defect [SONTD], and report the outcome of their treatment. A retrospective study of the clinical and radiological findings of 11 patients diagnosed with SCM, identified among 83 patients with SONTD at King Khalid University Hospital, in Riyadh, Saudi Arabia between 1995 and 2010. There were 6 girls and 5 boys; their age ranged from less than a year to 9 years [mean 4.2 years]. Six patients had type I SCM, and 5 patients type II SCM. The CT and MRI imaging showed characteristic bony, cartilaginous, or fibrous septum, and other SONTD-associated anomalies. Seven patients were graded A and B according to the Frankel grading score, and none of them required surgery, while worsening neurology led to surgical intervention in 3 patients, with clinical improvement after surgery, and one patient that underwent cord untethering remained stable. Split cord malformation is not uncommon among patients with SONTD. It tends to involve mainly the lumbar spine, and female predominance is more remarkable in type I. Neurological manifestations of SCM may be superimposed with SONTD. Surgery is effective for symptomatic patients, and not indicated in the severely disabled.

Agenesis of the corpus callosum associated with spinal open neural tube defect

To ascertain the incidence and clinical implications of agenesis of the corpus callosum [ACC] in spinal open neural tube defects [SONTD]. All cases of SONTD registered at the Spina Bifida Clinic in King Khalid University Hospital, Riyadh, Saudi Arabia between 1995 and 2010 were retrospectively reviewed, and mid-sagittal MRI of the corpus callosum [CC] area was analyzed in each case. Neurodevelopmental outcome was classified as poor in children with seizures, severe neurodevelopmental impairment, or death. Thirty-eight patients [45.8%] with ACC were identified among 83 cases with SONTD. Patients' age ranged between one and 16 years. Total ACC was found in 10 patients, partial ACC in 25, and in 3 patients, the CC was hypoplastic. Active hydrocephalus was an associated finding in 9 out of 10 patients with total ACC, 22 out of 25 with partial ACC, and in all patients with hypoplasia of the CC. Thirteen patients [34.2%] had normal intellectual function, whereas 24 patients presented with learning disability, epilepsy, or poor intellectual function; and one patient died of respiratory failure. Agenesis of the corpus callosum is found in a significant portion of patients with SONTD. When associated with hydrocephalus, its presence affects neuro-developmental outcome.

Epidemiology of neural tube defects in Saudi Arabia

To evaluate the distribution and pattern of neural tube defects in Saudi Arabia by creating a hospital based registry. All cases registered in the King Faisal Specialist Hospital and Research Center [KFSH and RC] neural tube defect [NTD] registry since it was established in October 2000 until December 2012 were studied through active surveillance comprising a registrar who collects NTD information by reviewing the patient's medical records, and interviewing patient's families. The total number of patients registered from October 2000 to December 2012 was 718 patients. There were more females [417, 58%] than males [301, 42%]. Of 620 mothers who underwent antenatal ultrasonography; 392 [63%] were diagnosed at birth, and 204 [33%] were diagnosed with antenatal hydrocephalus. In our registry sample, most mothers [95%] did not take folic acid 3 months prior to pregnancy, and 76% did not take folic acid during the 3 months after conception with the affected child. Only 5% received folic acid prior to conception. The KFSH and RC-NTD registry has met its objectives as a source of data that may significantly contribute to the prevention of NTDs, and improving quality of care for NTD patients through active publication of registry findings and management approaches.

Acute respiratory failure after endoscopic third ventriculostomy: a case report and review of the literature

Endoscopic third ventriculostomy [ETV] is a relatively safe procedure. However, postoperative acute respiratory failure may be fatal. The authors report an 8-month-old patient with obstructive hydrocephalus secondary to posterior fossa cyst, and Chiari malformation. After ETV he developed difficulty in breathing, and had to be reintubated and ventilated. The infant recovered fully after craniocervical decompression and insertion of cystoperitoneal shunt. We speculate that respiratory failure is related to relative expansion of the posterior fossa arachnoid cyst, causing significant compression on the brain stem. Supportive care with mechanical ventilation and brain stem decompression were the mainstay of treatment

Spinal cord injury without radiological abnormality [SCIWORA]. A diagnosis that is missed in unconscious children

Spinal cord injury without radiographic abnormality [SCIWORA] usually occurs in the hypermobile, immature cervical spine of young children. In a comatose child, a normal spine x-ray, and CT scan exclude most cases of gross fracture and dislocation, but not SCIWORA. We present 2 children that sustained a polytrauma, which rendered each of them unconscious at the outset. In both, cervical spine x-ray excluded bony injuries, however, CT scan raised the suspicious of spinal cord injury in one, and MRI demonstrated significant cord injury in both. The first patient died from severe head and cervical spinal cord injury. The second patient recovered with mild Brown-Sequard syndrome. A high index of suspicious of SCIWORA is necessary in injured comatose children in whom movement of all limbs is not seen. Therefore, spine MRI should be considered if they are expected to remain ventilated for an unknown time