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Natural history of hydrocephalus in children with spinal open neural tube defect.

Elgamal EA - Surg Neurol Int (2012)

Bottom Line: The shunts were revised or replaced in 10 (19.6%) patients due to obstruction or infection.Primary ETV failed in 3/8 patients, and treated by VPS.Children with SONTD should routinely undergo MRI examination of brain and craniocervical junction to clarify ventricular size, and the presence of CM II.

View Article: PubMed Central - PubMed

Affiliation: Neurosurgery Division, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia.

ABSTRACT

Background: The long-term prognosis of patients with Spinal Open Neural Tube Defect (SONTD)-associated hydrocephalus is not well known. This study was conducted to ascertain the incidence and natural history of hydrocephalus in patients with SONTD.

Methods: All 82 patients with SONTD referred to Neurosurgery/Spina Bifida Clinics at King Khalid University Hospital, Riyadh, Saudi Arabia (January 1995 - July 2010) were studied and followed for a period of 1-16 years. Patients were divided into three groups: Group "A" with active hydrocephalus treated with ventriculoperitoneal shunt (VPS), or endoscopic third ventriculostomy (ETV); Group "B" with compensated hydrocephalus; and Group "C" with no hydrocephalus. Timing of shunt insertion, complications of treatment and status of hydrocephalus were analyzed.

Results: The mean age of the 82 patients was 7.4 years (range 1-16 years). Group "A" included 59 (72%) patients, Group "B" 7 (8.5%) patients, and Group "C" 16 (19.5%) patients. Chiari malformation type II was found in 71 (86.6%) patients, 57 of whom (80%) were in Group "A" with active hydrocephalus. They were treated by VPS (51 patients) and ETV (8 patients). The shunts were revised or replaced in 10 (19.6%) patients due to obstruction or infection. Primary ETV failed in 3/8 patients, and treated by VPS. None of those in Groups "B" or "C" required treatment for hydrocephalus during the follow up.

Conclusion: Hydrocephalus affects the majority of patients with SONTD who have Myelomeningocele (MMC) and CM II and requires close surveillance and prompt management. Children with SONTD should routinely undergo MRI examination of brain and craniocervical junction to clarify ventricular size, and the presence of CM II.

No MeSH data available.


Related in: MedlinePlus

Axial T2WI for a child with SONTD, CM II and shunted hydrocephalus, showing a large intra ventricular cyst (a). The cyst was treated by endoscopic fenestration and re-adjustment of the ventricular catheter. Note the slight change in cyst size (b). Axial T2WI taken 2 years (c) and 3 years (d) later, showing collapse of the cyst and decrease in the size of the lateral ventricles
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Figure 7: Axial T2WI for a child with SONTD, CM II and shunted hydrocephalus, showing a large intra ventricular cyst (a). The cyst was treated by endoscopic fenestration and re-adjustment of the ventricular catheter. Note the slight change in cyst size (b). Axial T2WI taken 2 years (c) and 3 years (d) later, showing collapse of the cyst and decrease in the size of the lateral ventricles

Mentions: Among 51 patients with VPS, 5 (9.8%) experienced at least one surgical shunt complication during the follow up period, and another 5 (9.8%) had multiple (two or more) shunt complications. Shunt replacement or removal occurred in 90% of patients with a shunt complication, whereas ETV and removal of the obstructed shunt was performed in one patient. All cases of shunt infection were seen in the first year after insertion while only two of the obstructed shunts occurred after one year of insertion. Three patients with shunted hydrocephalus developed intra ventricular cysts which became symptomatic in one patient after increasing in size and causing significant shunt blockage. He was successfully treated by endoscopic fenestration of the cyst and readjustment of the ventricular catheter [Figure 7]. Primary ETV failed in 3/8 patients within 6 months of the ETV. The reason of failure was not known as those patients were treated by insertion of VP shunt on emergency basis. Two patients died due to complications related to hydrocephalus; shunt failure, repeated revisions and respiratory failure.


Natural history of hydrocephalus in children with spinal open neural tube defect.

Elgamal EA - Surg Neurol Int (2012)

Axial T2WI for a child with SONTD, CM II and shunted hydrocephalus, showing a large intra ventricular cyst (a). The cyst was treated by endoscopic fenestration and re-adjustment of the ventricular catheter. Note the slight change in cyst size (b). Axial T2WI taken 2 years (c) and 3 years (d) later, showing collapse of the cyst and decrease in the size of the lateral ventricles
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3475879&req=5

Figure 7: Axial T2WI for a child with SONTD, CM II and shunted hydrocephalus, showing a large intra ventricular cyst (a). The cyst was treated by endoscopic fenestration and re-adjustment of the ventricular catheter. Note the slight change in cyst size (b). Axial T2WI taken 2 years (c) and 3 years (d) later, showing collapse of the cyst and decrease in the size of the lateral ventricles
Mentions: Among 51 patients with VPS, 5 (9.8%) experienced at least one surgical shunt complication during the follow up period, and another 5 (9.8%) had multiple (two or more) shunt complications. Shunt replacement or removal occurred in 90% of patients with a shunt complication, whereas ETV and removal of the obstructed shunt was performed in one patient. All cases of shunt infection were seen in the first year after insertion while only two of the obstructed shunts occurred after one year of insertion. Three patients with shunted hydrocephalus developed intra ventricular cysts which became symptomatic in one patient after increasing in size and causing significant shunt blockage. He was successfully treated by endoscopic fenestration of the cyst and readjustment of the ventricular catheter [Figure 7]. Primary ETV failed in 3/8 patients within 6 months of the ETV. The reason of failure was not known as those patients were treated by insertion of VP shunt on emergency basis. Two patients died due to complications related to hydrocephalus; shunt failure, repeated revisions and respiratory failure.

Bottom Line: The shunts were revised or replaced in 10 (19.6%) patients due to obstruction or infection.Primary ETV failed in 3/8 patients, and treated by VPS.Children with SONTD should routinely undergo MRI examination of brain and craniocervical junction to clarify ventricular size, and the presence of CM II.

View Article: PubMed Central - PubMed

Affiliation: Neurosurgery Division, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia.

ABSTRACT

Background: The long-term prognosis of patients with Spinal Open Neural Tube Defect (SONTD)-associated hydrocephalus is not well known. This study was conducted to ascertain the incidence and natural history of hydrocephalus in patients with SONTD.

Methods: All 82 patients with SONTD referred to Neurosurgery/Spina Bifida Clinics at King Khalid University Hospital, Riyadh, Saudi Arabia (January 1995 - July 2010) were studied and followed for a period of 1-16 years. Patients were divided into three groups: Group "A" with active hydrocephalus treated with ventriculoperitoneal shunt (VPS), or endoscopic third ventriculostomy (ETV); Group "B" with compensated hydrocephalus; and Group "C" with no hydrocephalus. Timing of shunt insertion, complications of treatment and status of hydrocephalus were analyzed.

Results: The mean age of the 82 patients was 7.4 years (range 1-16 years). Group "A" included 59 (72%) patients, Group "B" 7 (8.5%) patients, and Group "C" 16 (19.5%) patients. Chiari malformation type II was found in 71 (86.6%) patients, 57 of whom (80%) were in Group "A" with active hydrocephalus. They were treated by VPS (51 patients) and ETV (8 patients). The shunts were revised or replaced in 10 (19.6%) patients due to obstruction or infection. Primary ETV failed in 3/8 patients, and treated by VPS. None of those in Groups "B" or "C" required treatment for hydrocephalus during the follow up.

Conclusion: Hydrocephalus affects the majority of patients with SONTD who have Myelomeningocele (MMC) and CM II and requires close surveillance and prompt management. Children with SONTD should routinely undergo MRI examination of brain and craniocervical junction to clarify ventricular size, and the presence of CM II.

No MeSH data available.


Related in: MedlinePlus