Despite sharing several organ systems, 10-month-old conjoined twins Knatalye Hope and Adeline Faith Mata were successfully separated in a world-first marathon procedure that was carried out at Texas Children’s Hospital in Houston on February 17th.
Image credit: Allen Kramer and Paul Kuntz/Texas Children’s
The twins were estimated to weigh just 3 lb 7 oz each when they were born by Cesarean section at 31 weeks of gestation at Texas Children’s Pavilion for Women in April 2014.
It was during a routine ultrasound 3 months earlier that the family – parents Elysse and John Eric Mata, and 5-year-old brother Azariah – learned not only that Mrs. Mata was carrying twins, but also that they were conjoined.
After this, they were referred to Texas Children’s Fetal Center at the Children’s Hospital for extensive prenatal scans and multidisciplinary consultation. Hospital teams also developed plans to ensure safe delivery and postnatal care of the twins.
As the pregnancy ensued, scans and tests revealed the baby girls shared a chest wall, pericardial sac (the lining of the heart), lungs, diaphragm, liver, intestines, colon and pelvis.
To deal with such a complex separation, various specialists were required, including pediatric surgeons, plastic surgeons, heart surgeons, urologists, plus surgeons specializing in liver transplant, orthopedics and pediatric gynecology.
During the marathon procedure, the team of 26 surgeons, anesthesiologists, surgical nurses and others, worked for 23 hours on Knatalye and 26 hours on Adeline. The twins were officially separated 18 hours into the operation.
The separation surgery marks the culmination of months of preparation involving 13 different specialties in the care of the twins, as lead surgeon Darrell Cass, a pediatric surgeon and co-director of Texas Children’s Fetal Center, and associate professor of surgery, pediatrics and obstetrics and gynecology at Baylor College of Medicine, explains:
“This is the first time a separation surgery for thoraco-omphalo-ischiopagus twins with this particular configuration has been successful.
This surgery was not without its challenges with the girls sharing several organ systems. Our team has been preparing for this surgery for months and we’ve done everything from working with our radiology experts to build a 3D model of their organs, to conducting simulations of the actual separation surgery.”
In order to prepare for their separation surgery, the twins underwent a 5-hour operation in December 2014 where surgeons inserted tissue expanders into their chest and abdomen area to stretch their skin.
The twins are now in the Pediatric Intensive Care Unit of the hospital under the care of a multidisciplinary team as they recover over the next two months.
Doctors say the twins will have to undergo further surgery in the future. It is too early to say when they will be discharged.
The following video gives the hospital’s account of the historical separation surgery.
Mrs. Mata says she and her family are very grateful for the enormous amount of planning and time that went into the surgery, and to the teams who cared for Knatalye and Adeline and who have given them “the incredible chance to live separate lives.”
She also wished to thank all the people who have supported the family with their hopes and prayers over the last 10 months.
Births of conjoined twins are rare, occurring around once every 200,000 live births. Many conjoined twins are stillborn or die within 24 hours of birth. The overall survival rate is thought to be between 5% and 25%.
For some reason, conjoined twin girls seem to have a better survival rate than boys, despite the fact that more male conjoined twins form in the womb.
Conjoined twins are genetically identical, and so always the same sex. Like all identical twins, they develop from the same fertilized egg, and they share the same amniotic cavity and placenta.
Conjoining is the result of the single egg not separating properly after fertilization. The developing embryo begins to split into identical twins during the first few weeks after conception but stops before the process is complete.
There are several types of conjoined twins, depending on the part of the body where they are fused and joined.
Attempts to separate conjoined twins are risky and complex and very rare – which is why they so often receive worldwide media interest.
In 2011, Medical News Today reported another marathon separation procedure where 19-month conjoined twins Maria and Teresa Tapia from the Dominican Republic were successfully separated following a 20-hour operation involving a team of 45 health professionals.