Treatments for Congenital Malformations

The pediatric neurosurgeons at the Pediatric Neurosurgery Program, along with the multidisciplinary team of expert physicians, treat congenital malformations:

Anomalies of the vertebral column
The physicians in the Pediatric Neurosurgery Program have extensive experience in the care and treatment of skeletal dysplasia or segmentation abnormalities of the vertebral column.  Many children with these diagnoses are treated each year at the Maxine Dunitz Children’s Health Center by the Department of Neurosurgery in conjunction with surgeons at the Cedars-Sinai Orthopaedic Center and the Institute of Medical Genetics.

Arachnoid cysts
Arachnoid cysts are cerebrospinal fluid-filled sacs that are formed in the space between the brain or the spinal cord and the arachnoid layer, one of the three membranes that cover the central nervous system.  These cysts can be present at birth and are the result of abnormalities arising during early development or secondary cysts that develop as a result of head injury, meningitis, tumors or complications of surgery.  Symptoms associated with an arachnoid cyst can include headaches, nausea and vomiting, seizures, hearing and visual disturbances and difficulties with balance and walking.

 There is ongoing active debate on the need for treatment of arachnoid cyst and the method of treatment.  

At Cedars-Sinai, our pediatric neurosurgeons diagnose, evaluate and treat children with arachnoid cysts of the central nervous system.  Microneurosurgical and minimally invasive techniques, such as neuroendoscopy, are used to treat patients who need surgery with a focus on obtaining the best results possible.  Whenever possible, our specialists will attempt to treat the cyst with fenestration before resorting to a spinal fluid diversion procedure by using a shunt.

Patients are seen by the physicians in the Minimally Invasive Neurosurgery and Neuroendoscopy Program at Cedars-Sinai.

Click here to learn more about arachnoid cysts.

Chiari malformation and spinal cord syrinxes
A chiari malformation is a benign structural problem affecting the cerebellum and is relatively common. Most children with these malformations who do not have spina bifida will have the form known as type I, which usually has less severe symptoms than type II. In patients with Chiari malformations, the cerebellum blocks the pathway of spinal fluid flow (CSF) in addition to compressing the brain stem at the cervicomedullary junction.  This blockage or restriction usually leads to headaches, neck pain, funny feelings in the arms and/or legs, stiffness, and, sometimes, will cause difficulties with swallowing or gagging. An MRI is usually used to diagnose the problem. Chairi malformations are extremely difficult to see on CT scans and impossible to see on plain X-rays. 

Sometimes these malformations can be made worse by, or can cause, hydrocephalus. They can often lead to a fluid filled cavity in the spinal cord known as a syrinxes (syringohydromyelia). Untreated, the chronic crowding of the brain stem and spinal cord can lead to serious consequences, including paralysis.

There are many ways to treat Chiari malformations but all require surgery. The treatment includes the surgical removal of a small portion of bone at the base of the skull and sometimes the back of the first spinal column segment, and occasionally, additional spinal column segments. Some patients will requirement placement of a "dural patch," which serves to further increase the space available for the cerebellum and the brain stem at the base of the skull. Most children who have the surgery do quite well and have a significant improvement in their symptoms.

Click here to learn more about Chiari malformations.

Craniosynostosis, craniofacial reconstruction and deformational plageocephaly

Craniosynostosis is the premature closure of one or more of the joints between the bones of the skull (cranial sutures) before brain growth is complete. In contrast to normal skull growth, closure of a cranial suture promotes compensatory growth in other parts of the skull, leading to an abnormally shaped skull and possible impairment in the development of the brain.  Although craniosynostosis can be gene-linked or caused by metabolic diseases, most commonly the cause remains unknown.   Single suture craniosynostosis is the most common and typically not associated with a genetic abnormality.  The early sign of craniosynostosis is skull deformities but late signs, which more commonly are associated with early closure of multiple cranial sutures, can include raised intracranial pressure, developmental delays, mental retardations, seizures and blindness.

Deformational (or positional) plagiocephaly refers to a condition where your child has an asymmetrical head from repeated pressure to that area.  Plagiocephaly differs from craniosynostosis in that craniosynostosis is the premature fusion of one or more of the sutures in the skull, whereas in  deformational plagiocephaly, there is no fusion of the skull sutures. A clinical diagnosis is made after a thorough medical history and physical examination by a craniofacial surgeon or neurosurgeon. If your child is diagnosed with deformational plagiocephaly, the doctor will usually prescribe a change in positioning and, perhaps, a corrective helmet.

Click here for more information about the Craniosynostosis and Cranio-facial Program.

In utero neurosurgical conditions
Pregnant women throughout California who have a fetus with a neurologic or neurosurgical diagnosis are routinely seen for prenatal consultation by our team of neurologists, perinatologists, geneticists and neurosurgeons. Cedars-Sinai is the home to the Monthly International Fetal Neurology and Neurosurgery Conference with participants from over 20 medical centers around the world via video teleconference.

Neural Tube Defects 
Spina bifida (myelomeniningocele) is the most common form of open neural tube defect.  It is a spinal cord injury associated with the myelomeningocele that can result in paraplegia, bowel and bladder incontinence, hydrocephalus and sexual dysfunction. 

Dr. Moise Danielpour, head of the Department of Neurosurgery's Pediatric Neurosurgery Program, is one of the few pediatric neurosurgeons with experience with in utero surgical treatment for birth defects such as myelomeningocele.

Click here to learn more about spina bifida.

Pediatric stroke, Moya Moya disease and other vascular malformations

A stroke is a general term used to describe a brain injury caused by bleeding or lack of oxygen to the brain.  While the diagnosis of stroke remains more difficult in children when it occurs there is greater potential for recovery of function.  At the Maxine Dunitz Children’s Health Center experts are brought together from many specialty areas in the hospital to provide advanced diagnostic and therapeutic modalities for treatment of infants and children with strokes.  
 

Tethered cord
A tethered spinal cord is a neurological disorder caused by abnormal limitations in the movement of the spinal cord in the spinal canal.  The condition is progressive with a child's symptoms including weakness, low back pain and loss of bladder and bowel control.  Other signs include, but are not limited to, club feet, scoliosis, incontinence, hairy patches over the spine, dimples or fatty tumors on the lower back.   The degree of the signs and symptoms correlate over time to the strain placed on the spinal cord.  The symptoms can be exacerbated with sports activity, pregnancy and a child's normal growth.  Treatment can improve symptoms and help prevent further neurological deterioration.

Other Congenital malformations
Our caring team of practitioners use their clinical and scientific expertise in the treatment of congenital malformations of the central nervous system, including myelomeningocele (spina bifida), syringomyelia (syrinx), lipomyelomeningocele, lipoma, myeloschisis, split cord malformations, diastematomyelia, Arnold-Chiari malformation, Dandy-Walker malformation, encephalocele, aquaductal stenosis, skeletal dysplasia and hydrocephalus.

Click here for more information about the International Skeletal Dysplasia Registry at Cedars-Sinai.