07 August 2004

CONJOINED CRANIOPAGUS TWINS NO MORE, PART ONE
The Aguirre Twins --- A Neurosurgical Adventure!



WHAT ARE CONJOINED CRANIOPAGUS TWINS?

Conjoined twins are identical twins that form from a single fertilized egg that splits into two embryos. When the split occurs more than 12 days after fertilization, the embryos do not fully divide and the twins may share body parts. Depending on the body part at which they are joined, they are classified as follows:
Craniopagus- united at the cranial part only; 2% of all conjoined twins,

Pygopagus- posterior union of the rump; 19% of all conjoined twins,

Thoracopagus- anterior union of the upper half of the trunk and always involves sharing of the heart; 35% of all conjoined twins, making it the most common type,

Omphalopagus- anterior union of the midtrunk; 30% of conjoined twins, second most common,

Ischopagus- anterior union of the lower half of the body, about 6% of all conjoined twins, and

Parapagus- lateral union of the lower half, extending variable distances upward, 5% of all conjoined twins.
Conjoined twins are always of the same sex, meaning they are either both boys or both girls, but statistically, 70 percent of conjoined twins are female. Conjoined twinning may be caused by a variety of factors, ranging from genetic to environmental. The current medical thought is that the causative factors are responsible for the failure of twins to separate after the 12th day after fertilization. Conjoined twinning has been artificially generated in amphibians by constricting the embryo so that two embryos form --- one on each side of the constriction.

No documented cases of conjoined triplets or quadruplets have been reported.

Carl and Clarence Aguirre, the Filipino conjoined twins in Montefiore Hospital in New York City are classified as conjoined craniopagus twins. They both share skull bones, but have separate trunks and with four arms and four legs.



WHAT HAPPENS TO CONJOINED TWINS WHEN THEY ARE BORN?

Most are stillborn. The outcome really depends on how they are connected; 40-60 percent are born stillborn while 35 percent survive 24 hours or less.

When conjoined twins survive, their parents usually face difficult decisions regarding their future. Some conjoined twins live successful lives like Eng and Chang Bunker, who gave us the term "Siamese twins." Others are plagued by medical complications due to shared organs and vital systems. Others are candidates for surgical separation.



THE AGUIRRE TWINS PROFILE

The Aguirre twins, Carl and Clarence, were born on April 21, 2002 in Hacienda Paz in barangay Rizal in Silay City, Negros Occidental, which is located in Negros island and part of the western Visayas region. For the uninformed, the Visayas region is in the southern part of the Philippine archipelago.

It was on the 4th month of pregnancy when Arlene Aguirre, the twins' 30-year-old single mother, learned that she was carrying conjoined twins after her obstetrician ordered an ultrasound to determine why she looked larger than usual. At one point, the obstetrician thought that Carl and Clarence were joined from the head to the feet. When the twins were born, they had a combined weight of 8.4 pounds only, so small that their mother could hold them in one hand.

When Clarence and Carl were almost a year old, they were severely malnourished and suffering from chronic aspiration pneumonia, and a consultation with Dr. Ceres Baldevia Gay, a pediatrician was the first step in what would be an exciting quest for their separation on the other side of the globe. Dr. Gay coordinated with Silay City Mayor Carlo Gamban, who looked for kind sponsors to finance the separation of the conjoined twins.

Dr. Gay also approached for help Menchu A. Sarmiento, who is the Philippine Airlines Foundation's Executive Director, to facilitate the twins' trip to New York for free, along with their mother Arlene. Ms. Sarmiento contacted Children's Chance Connecticut program manager Dorita Holland Urrata, who in turn turned out to be a close friend of Dr. James T. Goodrich, co-leader of the surgical team at the Children's Hospital at Montefiore (CHAM) that separated Carl and Clarence Aguirre last Thursday.

Dr. Goodrich and Urrata have known each other for years through Children's Chance collaborative work that helps arrange medical care for children from other countries. Dr. Goodrich, in turn phoned Dr. Willy Lopez, who is the head of the Neurosciences department at the University of the Philippines in Manila, to ask him to examine and assess the Aguirre twin situation and the possibilities of a successful operation seaprating the two. Dr. Goodrich knew Dr. Lopez because they trained together in New York to become neurosurgeons.

For months, the two exchanged emails and insights. Dr. Lopez sent cranial X-ray images of the twins thru international courier services. Finally, on September 2003, they decided to send the twins to Montefiore Hospital in New York.

The twin boys were 17-months old when they arrived with their mother in New York on September 10, 2003. They were first taken to Blythedale Children's Hospital in Valhalla where their nutritional status was improved prior to the surgery. In Blythedale, the twins were fed pureed food, and occasionally fed through a nasal tube. Since the twins are always lying down, digestion is a bit difficult. But after 5 weeks, the two gained 5 pounds, and brought their total weight to 31 pounds from a low combined weight of 26 pounds.

Another goal for the stay in Blythedale was to help the twins catch up with their developmental milestones through developmental and physical therapy sessions, where they undergo exercises like being put in a standing position while holding a rubber exercise ball. Blythedale Children's Hospital has decided to donate its services for the twins -- it is also here where they will spend most of their post-operative time after their separation --- at an estimated cost of $400,000.


NEXT: THE SERIES OF OPERATIONS TO SEPARATE THE TWINS



[References: ABS-CBN News, TheJournalNews.com, Newsday.com]


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