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Craniofacial Procedure to Treat Encephalocele - Boston Children's Hospital

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Published on Jul 26, 2012

Specialists at Boston Children's Hospital show excerpts from a surgical procedure performed to repair frontal encephalocele (protruding brain tissue through an opening in the skull which also causes elongation of the orbits and forehead) and a life-threatening arachnoid cyst in a young patient who traveled to Boston Children's from Haiti. Diagnosis and surgical treatment of the arachnoid cyst; removal of the encephalocele to expose the opening in the skull; and reconstruction of the elongated forehead bones, orbital areas and nose will be discussed, as well as lifelong management for patients with this anomaly. Additionally, David Walton, MD, a physician with Partners in Health, will share his perspective on the medical care required by a child with encephalocele, and the journey that this family had to take to bring the child from a developing nation to Boston Children's, where he received world-class care.

John Meara, MD, DMD, MBA, plastic surgeon-in-chief at Boston Children's Hospital and associate professor at Harvard Medical School will discuss this patient's diagnosis and treatment for encephalocele during the January Webcast. He will be joined by Edward Smith, MD, assistant in Neurosurgery at Boston Children's Hospital and assistant professor at Harvard Medical School, who will comment on diagnosis and treatment of the arachnoid cyst.

About encephalocele
Encephalocele is a rare defect, characterized by sac-like protrusions of the meninges (membranes that cover the brain) through openings in the skull. Encephalocele can present in various locations on a patients head -- either as a groove down the middle of the skull, between the forehead and nose or on the back side of the skull. Severity varies depending on its location. Although the exact cause is unknown, encephalocele is the result of the neural tube in the brain failing to close completely during fetal development and occurs rarely, at a rate of one per 5,000 live births worldwide. Ethnic, genetic and environmental factors, as well as parental age, can all influence the likelihood of encephalocele, which can also occur in families with a history of spina bifida. If surgical treatment is successful, and developmental delays have not occurred, a patient will likely develop normally. In cases where neurologic and developmental damage has occurred, specialists will focus on minimizing both mental and physical disabilities.

Dr. Meara will narrate the reparative surgery performed on the 11-month-old patient. After the removal of the protruding brain tissue, Dr. Meara reconstructed the elongated forehead bones, orbital areas and created support for a new nose.

About arachnoid cysts
Arachnoid cysts are fluid collections within the space around the brain that are thought to be made during the development of the child before birth. They are sacs of normal spinal fluid, contained by a thin membrane called the "arachnoid" (like a spider web). These arachnoid cysts are very common, with the majority being found incidentally, such as in this patient's case. Most of these cysts do not need any treatment whatsoever and have no effect on the normal development of the brain. However, a small number of arachnoid cysts, like the one found in this patient's neurological exam, are large enough to put excessive pressure on the surrounding brain and require surgical treatment. Dr. Smith will narrate the craniotomy, opening holes in the "bubble" of the cyst (fenestration) and the creation of a drainage path.

Children with arachnoid cysts who require treatment often do quite well. Hospital stays are usually short, ranging from overnight to a few days.

Boston Children's Hospital Department of Plastic Surgery is one of the largest of its kind in the country. In addition to formal training in plastic surgery, the team's eight surgeons have had specialized training in various sub-specialty areas of plastic surgery, including cleft and craniofacial surgery, hand and microsurgery, oral and maxillofacial surgery, orthopedics and otolaryngology. Additionally, the plastic surgery team works hand in hand with numerous other departments on multi-disciplinary treatment teams to care for patients with complex congenital anomalies.

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