Hydrocephalus: A treatable condition when caught early

Maria Endah Hulupi, The Jakarta Post, Jakarta

Twenty-year-old Sariti, a single mother from a small village in Lombok, West Nusa Tenggara, first sensed something was wrong with her son Sarobi when he was two months old.

His head seemed larger than it should be, but she carried on with her life, leaving her small home to work in the paddy fields early in the morning after putting Sarobi down to sleep. She returned late in the afternoon, bringing home a little of the discarded paddy as her wage.

She fed Sarobi plain rice with a little salt twice a day, and brokenheartedly watched as the baby grew weaker and his head grew larger.

Sarobi, now 15 months old and diagnosed with hydrocephalus, was recently flown along with his mother to Jakarta, in the care of Yayasan Sayap Ibu. He is being prepared for surgery at Cipto Mangunkusumo General Hospital in Central Jakarta.

Webster's Medical Desk Dictionary defines hydrocephalus as an abnormal increase in the amount of cerebrospinal fluid within the cranial cavity. It is accompanied by an expansion of the cerebral ventricles, an enlargement of the skull, especially the forehead, and atrophying of the brain.

The condition, according to a neurosurgeon at Mitra Keluarga Kelapa Gading, Dr. Jimmy Sugiarto, can affect fetuses, babies and adults.

Hydrocephalus, he said, is most often the result of the impaired resorption of the cerebrospinal fluid, causing the fluid to pool in the brain's ventricles. In very rare cases, however, the disease is caused by overproduction of the fluid.

In adults, the ventricles expand and compress the brain against the skull bones, increasing intracranial pressure. When left untreated it can lead to brain atrophy and death.

Symptoms include increased intra-cranial pressure, headaches, nausea, double vision, apathy, incontinence, concentration problems and coma.

"The symptoms are often mistaken for other diseases and instead of undergoing surgery to release the brain from the damaging pressure, patients get treatment for some other disease. The delay further exposes patients to increased pressure and greater brain damage, and brain cells simply don't regenerate," Jimmy said.

In fetuses and babies below the age of four, when the skull bones have not yet sutured, hydrocephalus causes the brain's ventricles to enlarge, making the skull expand as well. At this age, the disease is commonly characterized by a distended vein of the scalp, the "sunset phenomena" (where the eyes tend to be directed downward) and a bulging fontanel.

Jimmy said hydrocephalus could be caused by factors like a tumor, infections (such as tuberculosis, toxoplasma or meningitis), congenital malformations and bleeding, and conditions that disturb the circulation of cerebrospinal fluid.

Detection is effectively done by means of a ultrasonography (USG) for pregnant women and a CT scan. Once detected and the underlying cause found, patients should be operated on as soon as possible to drain away the fluid and relieve the pressure on the brain.

"It's a small operation but it can save lives and prevent further impairment of the person's condition," Jimmy said, adding that surgery would not help those patients with extreme cases.

Immediate surgery is especially important for babies under five months old because this is a period of rapid brain growth. Failure to treat the disease could affect the baby's intelligence in later life.

Cerebrospinal fluid, Jimmy said, can be drained off through external or internal surgery. External surgery is performed by inserting a ventricle drain to drain away the fluid in a closed steril container and reduce intracranial pressure. The drain can be left in position after surgery for several days.

Internal surgery is performed by implanting a shunt (a long tube) under the skin to drain away the fluid. The shunt can be implanted either in the abdomen (ventriculo peritoneal shunt) or in an atrium of the heart (ventriculo atrial shunt).

Because the surgery is usually effective, people seldom require a second surgery. However, further surgery is required to adjust the length of the shunt after a child patient has grown up, or to replace a shunt when a blockage occurs.

"Recovery depends on the patient's condition and the underlying cause (of the hydrocephalus)," Jimmy said.

After surgery, an enlarged baby's head will gradually reduce in size. Overlapping skull bones may occur afterward, but this can be corrected through cosmetic surgery.

Babies with hydrocephalus, Jimmy explained, can physically grow normally, but their intelligence may depend on how much damage the disease has caused.

Another neurosurgeon at the hospital, Bambang Setiawan, said doctors needed to talk to the parents and other family members of young hydrocephalus patients to help trace potential causes for the disease, such as the possibility that a toxoplasmosis infection occurred during pregnancy.

He also stressed the importance of a healthy diet and lifestyle to prevent the disease.