PBM and use of factor concentrates are key to successful separation of conjoined twins

Implementation of a Patient Blood Management (PBM) strategy played a pivotal role in life-changing surgery to separate conjoined Haitian twin girls at the Exequiel Gonzalez Cortés Hospital in Santiago, Chile in October 2021 – during the peak of the COVID-19 pandemic in the country.

The girls, born in September 2020, were joined at the pelvis. Prior to the surgery, extensive preparatory studies were performed to determine the extent of abdominal fusion and to assess the potential role of PBM.

Using fibrinogen concentrate to reduce bleeds

“The critical point of surgery was to perform pelvic osteotomies (surgery to reshape the pelvic bones) to facilitate the closure of the abdominal wall and contain the pelvic organs,” explains Dr Ignacio Sarmiento Goldberg, consultant anaesthesiologist and member of the international NATA scientific committee, adding: “The length of the surgery suggested an expected loss of at least 20% blood volume for each twin. So, a revolutionary haemostatic management strategy was planned and implemented.”

During the 16-hour surgery, the coagulation status of the two children was constantly assessed to ensure they each individually received personalised supplementation of both prophylactic and therapeutic fibrinogen concentrate to reach normal values during the marathon procedure. “I think this strategy was fundamental to reducing bleeding during separation and certainly during osteotomy,” suggests Dr Sarmiento Goldberg.

The role of Patient Blood Management (PBM) and importance of fibrinogen

PBM is a multi-disciplinary, evidence-based approach to individualising patient care in order to minimise the use of blood products and improve patient outcomes. In the case of the conjoined twins, the surgical separation involved the key participation of an anaesthesiologist, as well as digestive and plastic surgeons, urologists and traumatologists.

The use of fibrinogen in lieu of standard transfusion to avoid and control bleeding is an important topic for clinical research. Fibrinogen, also known as factor I, is a glycoprotein found in plasma. It is essential for binding blood platelets and forming a stable blood clot by polymerisation, which is critical for stopping excessive bleeding – for example in traumatic injuries, or during surgery.

Fibrinogen is the first blood factor to become deficient during perioperative bleeding or trauma and is often the only deficiency that needs to be treated. Fibrinogen concentrate allows the administration of a precise dose to reach the desired target level. It is immediately available, and has an excellent safety profile.

Individualised solutions for improved outcomes

In the case of the twin girls, Dr Sarmiento Goldberg is sure that the point-of-care assessment and management of coagulation disorders – primarily hypofibrinogenaemia – were pivotal in the successful separation. “In the last case of conjoined twins separated in Chile 10 years ago, massive exposure to blood products was a serious problem and resulted in morbidity. This time, the permanent haemostatic assessment through viscoelastic testing and the use of  Octapharma’s high-purity human fibrinogen concentrate, proved to be essential for our achieving a successful outcome,” he explains. “After 16 hours of surgery, and thanks to the control of haemostasis achieved with Octapharma’s high-purity human fibrinogen concentrate, only one unit of red blood cells was used in one of the girls. That was really impressive.”

Octapharma provided PBM educational programmes throughout 2021 and continues to support its implementation around the world. As Dr Oliver Hegener, VP Head of IBU Critical Care, describes: “Octapharma is committed to raising awareness of PBM within the medical community, particularly among those anaesthesiologists and intensivists who want to go the next steps towards individualised treatment solutions for improved outcomes and safety.”