The Medical City Performs Third Liver Transplant in a Child with Situs Inversus Totalis

The Medical City performed its third liver transplant in a pediatric patient suffering from end stage liver disease and a rare congenital condition call Situs Inversus Totalis. Five-year-old Marilda Agcaoile Guzman was operated on February 15, 2013 and was discharged a month later on March 16, 2013.

Situs Inversus Totalis (SIT) is a rare condition, characterized by reversal transposition of the thoracic and abdominal organs. Persons with SIT have all their internal organs "flipped" to the opposite side of the body such that the heart is found in the right side of the chest, instead of the customary position on the left. The stomach, normally in the upper left side of the abdomen, is found in the right side. The liver, on the other hand, is in the left of the abdomen, not in the normal position in the right. Associated anomalies of the blood vessels, spleen and intestine may also be found. The unusual position of the organs makes organ transplantation in these patients a more complicated procedure.

Marilda is only one of a very few SIT patients worldwide to have undergone a successful liver transplant. The Medical City's liver transplant team, led by Dr. Vanessa de Villa, head of the Center for Liver Disease Management and Transplantation (CLDMT), undertook the delicate 21-hour surgery which gave Marilda a new lease on life.

“Once again, the TMC team of liver transplant experts, surgeons, nurses, and critical care specialists have saved another Filipino life. They have shown what Filipinos can do for fellow Filipinos – in the Philippines – when given the opportunity to do the right thing … in an exemplary manner,” said Dr. Eugenio Jose F. Ramos, Senior Vice President of TMC Medical Services Group.

Two operations took place simultaneously. Marilda’s diseased liver was removed entirely, after which she received a graft which was taken from the liver of her father, Mario, 42, who was her donor.

Dr. De Villa said that Marilda's liver transplant was a highly complex procedure because of her SIT and her donor father’s complex vascular anatomy. The liver transplant team head added that, in the past, this would have been considered as a contraindication but a handful of successful transplants in patients with SIT have already been reported. Because of careful pre-transplant preparation and surgical planning with her liver transplant team, Dr. De Villa was confident about Marilda's life-saving procedure despite the complexity of her anatomy.

Marilda was born in a hospital in Riyadh, Saudi Arabia on February 24, 2008. Her parents, Imelda and Mario, both from Dagupan, Pangasinan, are Overseas Filipino Workers (OFW). The child was noted to have jaundice soon after birth and was subjected to phototherapy. At two months, Marilda’s jaundice persisted so an abdominal ultrasound was done which indicated her SIT condition. A liver biopsy done on Marilda when she was eight months old revealed cirrhosis or end-stage liver disease secondary to sclerosing cholangitis. Sclerosing cholangitis refers to inflammation, destruction and scarring of the bile ducts inside and outside of the liver. This leads to cirrhosis of the liver which is fatal unless liver transplantation is performed.

According to Dr. Karen Calixto-Mercado, a pediatric gastroenterologist, a liver transplant became imminent for Marilda in August 2012 when she started to have variceal bleeding from dilated blood vessels around the esophagus and stomach due to cirrhosis.

The prohibitive cost of hospitalization and treatment in Riyadh compelled Marilda's parents to take her home to the Philippines in search of a hospital where the child could undergo liver transplant.

The Guzman family first consulted doctors from a government hospital who, in turn, referred them to TMC.

“Sabi nila sa amin kung liver transplant na ang kailangan ng anak namin, dapat sa The Medical City na kami pumunta (They told us if our child needed a liver transplant, we had to go to The Medical City),” said Imelda Guzman.

Marilda was seen at TMC CLDMT by Dr. De Villa and Dr. Calixto-Mercado on the first week of September 2012. Both Marilda and her father were subjected to various tests to prepare them for the liver transplant.

“She again vomited blood in October 2012 which led to her admission at the TMC Pediatric Intensive Care Unit (PICU). Her bleeding was controlled by therapeutic endoscopy but her liver function was deteriorating. She underwent catch-up vaccinations, medical clearance and supportive treatment of decompensated liver disease to prepare for her liver tranplant,” said Dr. Calixto-Mercado.

The operation was originally scheduled in December last year but was rescheduled to a later date when Marilda developed bacterial sepsis. The operation finally pushed through last February 15, 10 days before Marilda’s 5th birthday. Marilda’s parents said they just had a small cake for their daughter’s 5th birthday but still, it was the happiest.

“Best birthday gift na kay Marilda yung successful liver transplant niya (Marilda’s successful liver transplant was the best birthday gift for her),” said Imelda. She is likewise thankful for her husband’s quick recovery.

Dr. De Villa said that although Marilda’s condition is stable and her “new” liver is functioning well, she still needs to be observed and monitored regularly by her doctors.

After her liver transplant operation, Marilda developed two life-threatening conditions, obscure intestinal bleeding and hepatic artery thrombosis. Both complications were managed adequately without requiring a second operation.

Marilda’s operation was TMC’s third liver transplant and second pediatric case. In January 2011, TMC performed the first successful pediatric liver transplant in the country in a three-year-old patient, Catherine Erica Buenaventura. In July 2012, TMC performed its second liver transplant operation giving Amytis Batao, a doctor and former mayor from Cebu a “second” life. The liver transplant allowed them to recover from grave illness within a month after the operation and they enjoyed life with a perfectly functioning liver graft from thereon.

Erica learned to walk at four years old after her liver transplant and was able to realize her dream of going to school. But while her liver transplant was successful, Erica tragically succumbed to the dengue virus in September 2011.

TMC’s second liver transplant patient, Dr. Batao, is now back in Cebu, enjoying a normal and healthy life.

Aside from Dr. De Villa and Dr. Calixto-Mercado, Marilda’s and Mario’s core team of doctors includes transplant hepatologist Dr. Janus Ong; pediatric intensivist Dr. Neva Batayola; pediatric cardiologist Dr. Dexter Cheng; infectious disease specialist Dr. Cynthia Aguirre; hepatobiliary surgeons Dr. Dante Ang, Dr. Anthony Yap, and Dr. Ramon de Vera; and microvascular surgeons Dr. Eric Arcilla, Dr. Glenn Genuino and Dr. Catherine Yap-Asedillo. Drs. Grace Herbosa, Celine Ancheta, Alexandra Odi, Elena Malong, Elke Sauz, and Ace Prodigalidad complete the team as special anesthesiologists for both the donor and the recipient. Other specialists who helped manage the patient were interventional gastroenterologist Dr. Mark Anthony De Lusong, radiologist Dr. Rommel Galsim, interventional radiologist Dr. Ramon Santos-Ocampo, and rehabilitation specialist Dr. Celso Bate. This multi-disciplinary team of experts was ably supported by equally competent and compassionate nurses and counselors.

The Guzman family is grateful to their doctors and nurses for their dedication, to their family and friends who helped them raise funds for the transplant, to TMC for giving them generous discounts and to the Philippine Charity Sweepstakes Office (PCSO) through its Board of Directors who donated a significant amount to defray the cost of the operation.

The CLDMT is a specialty unit that offers a comprehensive array of diagnostic and therapeutic options for patients with liver diseases and liver tumors through an integrated and highly skilled multidisciplinary, patient-partnership approach. It is otherwise known as The Medical City Liver Center and may be reached through 9887000 local 6506 or 0932-88LIVER."

Caption: Marilda Guzman, mom Imelda, and dad Mario with the Liver Transplant team led by Dr. Vanessa De Villa.