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Porencephaly

Porencephaly is a type of Cephalic disorder. This is an extremely rare disorder of the central nervous system involving a cyst or cavity in a cerebral hemisphere. The cysts or cavities are usually the remnants of destructive lesions, but are sometimes the result of abnormal development. The disorder can occur before or after birth. more...

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Porencephaly most likely has a number of different, often unknown causes, including absence of brain development and destruction of brain tissue. The presence of porencephalic cysts can sometimes be detected by transillumination of the skull in infancy. The diagnosis may be confirmed by CT, MRI, or ultrasonography.

More severely affected infants show symptoms of the disorder shortly after birth, and the diagnosis is usually made before age 1. Signs may include delayed growth and development, spastic paresis (slight or incomplete paralysis), hypotonia (decreased muscle tone), seizures (often infantile spasms), and macrocephaly or microcephaly.

Individuals with porencephaly may have poor or absent speech development, epilepsy, hydrocephalus, spastic contractures (shrinkage or shortening of muscles), and mental retardation. Treatment may include physical therapy, medication for seizure disorders, and a shunt for hydrocephalus. The prognosis for individuals with porencephaly varies according to the location and extent of the lesion. Some patients with this disorder may develop only minor neurological problems and have normal intelligence, while others may be severely disabled. Others may die before the second decade of life.

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ACOG to target neonatal encephalopathy, CP link: Report builds on 1999 consensus statment - Major Report in the Works
From OB/GYN News, 4/1/02 by Bruce Jancin

VAIL, COLO. -- The American College of Obstetricians and Gynecologists is putting the finishing touches on a forthcoming major report on neonatal encephalopathy and cerebral palsy.

"It will be the most authoritative document produced to date on these subjects. It'll be vitally important for those of us who deliver babies for a living," said Dr. Gary D.V. Hankins, who chairs the ACOG task force charged with creating the report.

The document, which has been 2.5 years in the making, will be published this fall, he said at a conference on obstetrics and gynecology sponsored by the University of Colorado.

The past few years have brought major advances in the understanding of the causes of cerebral palsy (CP). Much of this work is summarized in an important 1999 international consensus statement endorsed by ACOG and 14 other medical organizations entitled "A Template for Defining a Causal Relationship Between Acute Intrapartum Events and Cerebral Palsy." This report, which was published in 1999 in the British Medical Journal (BMJ 319[7216]:1054-59, 1999), isn't widely known in American medical and legal circles. The ACOG document builds upon this consensus statement and should get the word out to a wider audience.

"Something has to be stated to put to rest those individuals who get up in court and totally misrepresent the science. That's one of our efforts in this document--to tell the truth as we know it, say where we don't know it, and, in areas where we're getting hammered unnecessarily, to so state that," Dr. Hankins, professor and vice chair of ob.gyn. at the University of Texas, Galveston, explained.

One of the key points to be emphasized in the ACOG report is that there is now broad agreement that neonatal encephalopathy (NE) is a necessary finding in cases of cerebral palsy due to an intrapartum event, he said. If there's no NE, then the cerebral palsy wasn't due to an intrapartum event, and the obstetrician can't rightfully be sued over the bad outcome.

NE is a clinically defined syndrome occurring during the first week after birth of a term or near-term baby. It involves disturbed neurologic function as evidenced by difficulty in initiating and maintaining breathing, an altered level of consciousness, depressed tone and reflexes, and often seizures.

"You cannot get from intrapartum insult to cerebral palsy without traversing through neonatal encephalopathy. That's a key point that should be worth something to the cost of your career in continuing to deliver babies," Dr. Hankins stressed.

NE is necessary but not sufficient to establish a link between intrapartum hypoxia and cerebral palsy in an individual case. That's because NE has a multitude of possible causes. A pivotal case-control study by Australian neonatologist Nadia Badawi convincingly demonstrated that purely intrapartum risk factors were present in a mere 4% of cases of NE; fully 69% of cases had prepartum risk factors only. (See chart.)

The ACOG document will propose that pediatricians standardize their work-up of babies with NE so that the timing of the hypoxic insult can be addressed, he said. For example, if an imaging study obtained 72 hours after birth shows evidence of a long-standing neurologic abnormality such as ventriculomegaly, porencephaly, or multicystic encephalomalacia, an intrapartum event can be ruled out as the cause of the child's symptoms. An intrapartum hypoxic event will result in evidence of an acute cerebral abnormality on an imaging study at 72 hours, Dr. Hankins said.

The ACOG task force responsible for the forthcoming document is composed of 12 ACOG fellows who drew on input provided by consultants in a range of specialties. AGOG has invited the American Academy of Pediatrics and other pediatric groups to endorse its report. It's not yet known whether that will happen, the ob.gyn. said.

COPYRIGHT 2002 International Medical News Group
COPYRIGHT 2002 Gale Group

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