Abstract
BACKGROUND: Parasitic or heteropagus conjoined twins are an exceptionally rare congenital anomaly, with an estimated incidence of 1 in 1-2 million live births. These anomalies pose complex clinical and surgical challenges due to their varied anatomical presentations. Multidisciplinary evaluation and timely intervention are essential for favorable outcomes. The study aimed at clinical presentation, anatomical description, management, and outcomes of various heteropagus twins that were managed in a single tertiary care center. METHODS: This retrospective study included six patients diagnosed with heteropagus twinning. Antenatal ultrasonography (USG) findings were reviewed where available. Postnatal investigations included USG, magnetic resonance imaging, and two-dimensional echocardiography for anatomical delineation and cardiac evaluation. Surgical intervention was performed based on the anatomical site and complexity of attachment. Resected specimens were sent for histopathological examination. RESULTS: All patients presented in early infancy. Presentations were diverse: one had a sacral mass mimicking a myelomeningocele (rachipagus) containing intestinal loops; another had a limb, phallus, and testis with lipomeningocele; the third baby had an extra lower limb attached to the pelvis; the fourth baby had an accessory digit on the back; the fifth baby had rudimentary parasitic tissue near the perineum, and the last baby had a mandibular outgrowth resembling a distorted ill-formed face. All cases underwent successful surgical separation with good postoperative recovery. One of the babies succumbed to neonatal sepsis. All the patients are under a strict surveillance protocol with a minimum follow-up period of 3 years and longest being 8 years. CONCLUSION: Heteropagus twins exhibit a wide range of anatomical variations. Early antenatal diagnosis, detailed imaging, and prompt surgical management are key to achieving favorable outcomes. In addition, preoperative planning must include a detailed anatomical survey through the radiological scans and also cardiac evaluation. Surgical procedure to separate the parasitic twin varies from a simple to a more complex approach, so a multidisciplinary approach ensures comprehensive care and minimizes morbidity.