One of the most intricate and challenging surgeries in medicine is the separation of conjoined twins. These rare cases test the limits of surgical skills and advancements. Conjoined twins often share vital organs and complex vascular systems, so operating on them requires an intricate negotiation between medical and ethical considerations. Each surgery is accompanied by a story of careful preparation, intricate planning, and a determination to give these children a chance at individual lives. This article explores the major challenges surgeons and medical teams face as they perform one of the most complicated surgeries.
To get an insightful opinion Medbound Times reached out to Dr. Preeti Sawant MBBS, DNB General Surgery from Coimbatore who was able to share her knowledge on this topic.
Understanding Conjoined Twins
A rare phenomenon in which two newborns are born physically attached to one another is known as conjoined twins. This condition develops when a single embryo divides partially, resulting in the formation of two people that are connected at certain body parts. Organ sharing is common in conjoined twins, and the degree and location of their joining might affect how complex their relationship is.
Conjoined twins are classified based on where they are physically joined, Common types include:
Thoracopagus twins: Joined at the chest, often sharing a heart, liver, and upper intestine.
Omphalopagus twins: Connected near the bellybutton, frequently sharing the liver and parts of the digestive tract, though they usually do not share a heart.
Pygopagus twins: Joined at the base of the spine and buttocks, possibly sharing parts of the lower gastrointestinal tract and genital or urinary organs.
Rachipagus twins: Rarely joined along the length of the spine, back-to-back.
Ischiopagus twins: Connected at the pelvis, often sharing the lower gastrointestinal system, liver, and urinary or genital organs. They may have two or fewer legs.
Parapagus twins: Joined side by side at the pelvis and abdomen, with separate heads but potentially sharing limbs.
Craniopagus twins: Joined at the skull, usually with separate brains but sometimes sharing brain tissue.
Cephalopagus twins: Joined at the head and chest, often sharing a brain and rarely surviving.
The feasibility is decided by the multidisciplinary team through comprehensive preoperative planning and medical assessment, risk-benefit analysis considering surgical risk, quality of life and long-term prognosis, resource availability, and ethical considerations. It involves balancing the medical, ethical, and emotional factors to determine the best course of action for the twins and their family.
Dr. Preeti Sawant MBBS, DNB General Surgery PGY3 , Coimbatore
There are two types of conjoined twins: those that can be separated apart and those that cannot. If separation is feasible, it is determined by medical imaging and expert opinions. If there is a high possibility of organ sharing, organ separation may not be safe. The ultimate say in such circumstances remains in the hands of parents, but a hospital ethics committee may get involved.
Surgical Planning
It is essential to fully understand how the twins are connected. Surgeons can prepare with the assistance of techniques like preoperative simulations and 3D modeling.
Key steps in the surgical management include:
1. Detailed anatomical analysis to avoid surprises.
2. Early involvement of specialists.
3. Pre-surgical simulations.
4. Clear steps and backup plans for the surgery.
5. Preparing for post-surgical reconstruction.
Dr. Preeti Sawant mentions that surgical challenges are primarily due to complex anatomy, making it one of the most challenging procedures in Pediatric Surgery.
Vital organs
• Liver, heart, and brain as shared vital organs make it an extremely challenging surgery requiring meticulous planning and preoperative preparation with a skilled team.
•The Liver is the most commonly shared organ among conjoined twins and is considered a complex procedure to separate due to its extensive blood supply.
• When twins share a heart, the intricate structure and vital functions make it difficult to divide without compromising one or both twins' survival.
• Craniopagus twins, who are joined at the head, may share brain tissue and blood vessels, requiring utmost planning and precision to prevent neurological damage during separation.
• Twins joined at the abdomen may share the liver along with the gastrointestinal tract, requiring reconstruction to maintain individual digestive function.
Vascular Connections
Division and reconstruction of shared blood vessels with complex microsurgery to ensure each twin has an independent circulatory system.
Risk of complications, blood loss, and organ function
This may necessitate multiple surgeries or medical care postsurgery -
Ethical dilemmas
Parents/Guardians and the medical team must weigh the quality of life and survival chances for both twins and possible expectant surgical outcomes.
Challenges and Ethical Concerns
The administration of medication is complicated by shared circulation since medications might have different effects on each twin. Making the decision gets more challenging if there is a significant danger of surgery for one or both twins, yet being conjoined lowers their quality of life. Separation, on the other hand, usually enhances the twins' chances of survival when there is little to no link and low-risk surgery.
Dr. Preeti Sawant shares that the ethical considerations are not limited to the Surgeon and include the multidisciplinary team in the Surgery.
She mentions that the following factors also play a role in this process:
Informed Consent
The conjoined twins being infant/young cannot provide consent and surrogate decision-making through the parents or guardians is sought in routine practice.
Conjoined versus post-separation quality of life
To weigh the risks and benefits of the potential complications pre and post-surgery is a difficult task. The potential quality of life for each twin after separation is a crucial factor and includes physical, psychological, and social health.
Risk of Harm
High-risk complex surgery with unpredictable outcomes considering difficult anatomy that requires preoperative planning with a multidisciplinary team. Long-term outcomes of such complex surgeries often require multiple surgeries.
Extensive medical resources in limited-resource setting
Ethical impact of economic burden on the family and the healthcare system.
Psychological impact based on the age at separation
In Brazil, a 23-year-old woman gave birth to conjoined twin brothers with separate brains and spinal columns but shared vital organs like the heart, lungs, and liver. Their medical and ethical concerns prevented them from separating because of their condition. The twins died at the age of six months, despite their early stability.
A contrasting case from Chicago in 1993 involved Amy and Angela. They had different lungs, but they shared the same liver and heart. Even though doctors advised against separation given the risk of Amy’s death and Angela’s slim survival possibilities, their parents insisted on their separation. Angela died before her first birthday, while Amy died during surgery. The moral dilemma of whether it is acceptable to compromise the life of one twin in the slim possibility of enhancing the other's life is brought up by this particular case.
In one case, the parents opposed surgery because they believed it was wrong to sacrifice one child to save the other, a view based on the "act-omission" doctrine. This doctrine suggests that actively causing harm, such as killing one twin, is harder to justify than allowing harm through inaction, such as letting both twins die naturally without intervention. However, the doctrine of double effect complicates this stance by arguing that it can be morally acceptable to cause harm if it is an unintended side-effect of saving more lives, as long as the harm isn't the direct goal. This reasoning supports the idea of separating conjoined twins even if one twin may die, but raises broader ethical questions when applied to other controversial cases, like organ harvesting.
Dr. Preeti Sawant MBBS, DNB General Surgery PGY3, Coimbatore
The journey of conjoined twins and the complexities of their surgical separation highlight a distinctive blend of medical knowledge, morality, and empathy. Careful evaluation, cooperation between different medical specializations, and serious ethical concerns go into the decision to separate them.
Surgical procedures and technological advances have made it possible for doctors to separate many conjoined twins, improving their quality of life. However, each case remains unique, highlighting the need for sensitivity and clear communication with the family. The twins' well-being and health must come first ensuring they have the best chance at a fulfilling life post-separation.
References
Mayo Clinic. "Conjoined Twins: Symptoms and Causes." Accessed September 21, 2024. https://www.mayoclinic.org/diseases-conditions/conjoined-twins/symptoms-causes/syc-20353910.
Children's Hospital of Philadelphia. "Conjoined Twins." Accessed September 22, 2024. https://www.chop.edu/conditions-diseases/conjoined-twins.
Spencer, Rowena. "Surgical Separation of Conjoined Twins: Current Concepts." Clinical Anatomy, vol. 28, no. 4, 2016, pages 538–551. Accessed September 23, 2024. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4882632/.
Goodrich, James T., and Oren Tepper. "Ethical Considerations in the Separation of Conjoined Twins." Seminars in Pediatric Surgery, vol. 27, no. 3, 2018, pages 123–127. Accessed September 24, 2024. https://www.sciencedirect.com/science/article/abs/pii/S0146000518300521.
Martinez, Jessica, and Michael Crone. "Conjoined Twins: Clinical Overview." StatPearls. Accessed September 24, 2024. https://www.ncbi.nlm.nih.gov/books/NBK560839/.
MSM
