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Surgical experience with thirteen conjoined twins
J A O'Neill1, G W Holcomb, L Schnaufer
1Department of Surgery, Children's Hospital of Philadelphia, PA 19104.
Insights
Conjoined twin survival rates significantly improve when surgical separation is delayed until infants are 6-12 months old. Advances in imaging and surgical techniques enhance outcomes for these rare births.
Area of Science:
- Medical research
- Pediatric surgery
- Genetics
Background:
- Conjoined twinning is a rare condition affecting approximately 1 in 50,000 births, often with poor survival rates.
- This study reviews 30 years of experience with 13 cases of conjoined twins, encompassing various types like thoracopagus, omphalopagus, ischiopagus, pygopagus, craniopagus, and parasitic twins.
Observation:
- Diagnostic and imaging studies were detailed for each twin type.
- Surgical separation outcomes were analyzed based on the age of the infants at the time of operation.
- Long-term survival was assessed, noting late deaths primarily due to congenital anomalies, particularly cardiac issues.
Findings:
- Delayed separation (6-12 months) yielded a 90% operative survival rate, compared to 50% for neonatal procedures.
- Ten separation surgeries resulted in 16 survivors, with six late deaths within 10 years, predominantly from cardiac anomalies.
- Improved survival is attributed to advanced imaging, anesthesia, and surgical techniques, including immediate reconstruction and use of skin expanders/prosthetic mesh.
Implications:
- Optimal surgical timing is crucial for improving conjoined twin survival rates.
- Continued advancements in medical technology and surgical approaches are vital for managing complex congenital anomalies.
- Future strategies like ex vivo cardiac reconstruction and autotransplantation may offer solutions for separating twins with intricate cardiac connections.
Abstract:
Conjoined twins occur in approximately one in 50,000 or so births, and most do not survive. The authors report herein their experience with 13 conjoined twins over the last 30 years, involving those of the following forms: thoracopagus (4 cases), omphalopagus (1 case), ischiopagus (4 cases), pygopagus (1 case), craniopagus (1 case), and incomplete or parasitic varieties (2 cases). The various diagnostic and imaging studies used are described in detail for each form of twinning. Separation is best delayed until such infants are relatively mature (i.e., 6-12 months of age). Operative survival was 50% in those operated on in the neonatal period, but 90% in those over 4 months of age. Ten separations were attempted in 13 sets of twins, with 16 operative survivors. Significantly, up to 10 years after surgery, there were six late deaths due to serious associated congenital anomalies, predominantly cardiac. Improved recent survival is probably the result of the availability of more accurate imaging studies and better anesthetic and operative techniques, with great emphasis on performing immediate reconstruction whenever possible. Use of skin expanders and prosthetic mesh has facilitated wound closure. In the future, ex vivo cardiac reconstruction and autotransplantation may permit separation of twins with complicated conjoined hearts.