Delayed diagnosis of hydrocephalus leads to significant brain damage


This case involves a twin pregnancy in which a prenatal ultrasound identified hydrocephalus in one of the two developing babies.  The twins were born at thirty-four weeks with one twin being unaffected and the other requiring surgical intervention due to complications from the in utero hydrocephalus. Five days after birth, the pediatric neurosurgeon inserted a Delta VP shunt into the hydrocephalic twin without any apparent complication.  A second neurosurgeon evaluated the neonate and determined that the patient required a larger valve to allow for higher CSF flow.  Forty-eight hours following the placement of the first valve, the surgeon performed a second procedure to insert a larger, low-pressure valve. Immediately after the procedure, the medical staff noted the presence of excessive CSF drainage that resulted in a collapse of the patient’s cortical mantle and massive cortical hemorrhages.  The patient is presently twelve years old and continues to suffer from neurological devastation and cortical blindness.

Question(s) For Expert Witness

  • Are there any measures that could have been taken to treat this baby in utero?

Expert Witness Response E-006011

Congenital hydrocephalus occurs in about 1 in every 1,000 live births. It is rarely lethal but frequently severely handicapping. It can be diagnosed by ultrasound as early as twenty weeks of gestation. When not accompanied by other serious malformations, it may be amenable to treatment in utero. Experimental treatment by shunting the lateral ventricle to the amnion has been shown to control ventricular enlargement during fetal life. The mental development of treated babies has, however, been disappointing. At this time no in utero treatment of fetal hydrocephalus is being done.

Expert Witness Response E-004688

Expert-ID: E-004688

Medical treatment in hydrocephalus is used to delay surgical intervention. It may be tried in premature infants with posthemorrhagic hydrocephalus (in the absence of acute hydrocephalus). Normal CSF absorption may resume spontaneously during this interim period. Medical treatment is not effective in long-term treatment of chronic hydrocephalus. It may induce metabolic consequences and thus should be used only as a temporizing measure. Shunts eventually are performed in most patients. Only about 25% of patients with hydrocephalus are treated successfully without shunt placement. The principle of shunting is to establish a communication between the CSF (ventricular or lumbar) and a drainage cavity (peritoneum, right atrium, pleura). It is important to remember that shunts are not perfect and that all alternatives to shunting should be considered first.

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