This article originally appeared in Pathways, a magazine
published for physicians and the community by UMass Memorial Medical
Being a parent has its challenges. Being the parent of a child with
developmental abnormalities of the central nervous system takes those challenges
to an entirely different level. Comprehending and managing the medical and
surgical needs of these young patients can feel overwhelming. The good news is
that help is now available at UMass Memorial Children's Medical Center.
With pediatric neurosurgeon Oguz Cataltepe, MD, on the staff of the UMass
Memorial Children's Medical Center (CMC), the region has convenient access to a
highly trained specialist with extensive experience in treating this complex and
vulnerable patient population.
The pediatric neurosurgery service at CMC provides a complete range of
counseling, diagnosis and treatment of developmental abnormalities of the
nervous system including neural tube defects (NTDs), Chiari malformation,
syringomyelia, craniosynostosis and tethered cord. Services for surgical
treatment of brain and spinal cord tumors, hydrocephalus, trauma-related central
nervous system injuries and epilepsy in children are also available.
The journey from diagnosis through treatment often begins before birth.
"Some central nervous system pathologies, such as congenital hydrocephalus and
NTDs, can be diagnosed through prenatal tests such as blood screening,
amniocentesis and ultrasonography," Dr. Cataltepe explained, referring to these
conditions as "pretty common problems."
Hydrocephalus, a pathology associated with increased cerebrospinal fluid and
ventricular volume in the brain, is frequently diagnosed during infancy due to
rapidly enlarging head size. "Because of the increased fluid volume and
intracranial pressure, these patients need treatment," he says. "The treatment
options are mainly surgical: ventriculo-peritoneal shunt or neuroendoscopic
third ventriculostomy," he added.
"NTDs (spina bifida) are a group of lesions frequently seen with severe
neurological deficits such as paraparesis and paraplegia as well as accompanying
hydrocephalus," Dr. Cataltepe continued. "Myelomeningocele is one of the most
common pathologies in this group. It is an open form of NTD wherein the spinal
cord is exposed at birth without any bony or skin coverage, so there is a high
risk of infection and neurological deficit." He noted that both hydrocephalus
and myelomeningocele are lifelong pathologies for which patients need ongoing
medical and surgical care.
"These are complicated problems, and we meet with parents before the delivery
to explain the nature of these pathologies, treatment options, future problems
and solutions," Dr. Cataltepe said. "We try to prepare them and provide a
realistic picture of the problem."
The next step is helping parents choose the best treatment after delivery.
Options are mainly surgical, he noted. "We discuss the details of the surgical
intervention and prepare the medical and surgical team for operation after the
delivery. If the baby is stable, we generally do surgical intervention the next
Epilepsy is another fairly common condition that can be treated surgically in
some cases. "Approximately 20 percent of epileptic patients are drug-resistant,"
Dr. Cataltepe said. "These patients may benefit from surgery if there is any
epileptogenic lesion or focus in the brain." Surgery candidates undergo a
comprehensive exam and diagnostic studies. If a lesion is determined during this
evaluation, that region of the brain can be surgically removed without impairing
the child's brain function. If the epileptogenic focus is diffuse or in a
functionally critical region of the brain, placement of a vagal nerve stimulator
can be an option.
Spasticity is a condition for which Dr. Cataltepe and his team take a
multi-disciplinary approach including pediatric neurology, an intraoperative
electro-physiological monitoring team, physical therapy and pediatric orthopedic
surgery. He notes that surgical treatment options for spastic children include
baclofen pump placement and selective dorsal rhizotomy. Yet another area that
can be addressed surgically is birth-related brachial plexus injury. "If the
baby does not recover from injury within the first six months, surgical
intervention is an option," he said.
One of the most exciting advances in pediatric neurosurgery is
neuroendoscopic surgery. "This technique has emerged as a very useful
alternative for shunting in hydrocephalus, treating intraventricular cysts, and
performing brain tumor biopsy if the tumor is in the ventricle," Dr. Cataltepe
The neurosurgeon makes a small hole - about one centimeter in diameter - in
the skull and introduces a neuroendoscope into the ventricles of the brain. The
diameter of the neuroendoscopes used ranges from three to six millimeters. The
neurosurgeon can see the surrounding structures clearly while navigating in the
brain, and can perform a biopsy, treat a cystic mass or perform a third
ventriculostomy or aqueductoplasty to treat hydrocephalus.
"All these procedures can be done through a very small opening without
disturbing larger areas of the brain," Dr. Cataltepe explained. "The treatment
of hydrocephalus with a shunt has a very high complication rate because of the
nature of the shunt system. With the neuroendoscopic technique, we can create a
new cerebrospinal fluid (CSF) circulation pathway with a third ventriculostomy
at the obstruction site, or we can reconstruct the CSF circulation pathways with
aqueductoplasty technique. So the patient with hydrocephalus can be treated
without a shunt and without shunt-related problems."
"Neuroendoscopy is much less invasive, which is a big consideration in such
small patients," noted Marianne Felice, MD, physician-in-chief, Children's