- Child presented with jaundice and was diagnosed with Langerhans Cell Histiocytosis with severe liver dysfunction, with bilirubin levels rising to 10–12 mg/dL
- Modified low-dose chemotherapy achieved a 90–95% response, while recurrent UTIs led to the diagnosis and surgical correction of congenital vesicoureteric reflux before liver transplantation
- Following liver transplantation, the child received four additional rounds of chemotherapy and is now free of LCH and recurrent urinary infections
Chennai, 30th September, 2026: In yet another case highlighting the importance of a multidisciplinary approach in managing complex pediatric conditions, doctors at Rainbow Children’s Hospital successfully performed a liver transplant on a 3-year-old child diagnosed with the rare systemic disorder Langerhans Cell Histiocytosis (LCH), whose treatment was further complicated by an underlying congenital abnormality of kidneys causing recurrent urinary tract infections. The child’s treatment involved multiple specialties and required the medical team to address one clinical challenge after another before the child could safely undergo transplantation.
The child was initially brought to Rainbow Children’s Hospital with complaints of jaundice. Investigations revealed LCH, a rare systemic disorder, with significant liver dysfunction. Given the severity of the liver involvement, the initial focus was on controlling the underlying LCH and improving the child’s condition before transplantation. Chemotherapy was therefore initiated. However, with the liver already severely compromised, the treatment itself had to be carefully adapted. The child was placed on a low-dose chemotherapy which showed only partial response. Now there is a significant advancement in the newer targeted therapy which resulted in a good response in the LCH.
While this represented a significant response to treatment, the child’s clinical journey became more complex when he began developing recurrent urinary tract infections during chemotherapy. Rather than treating the infections as an isolated complication, the medical team investigated the underlying cause. Evaluation by the pediatric nephrology and urology teams revealed congenital vesicoureteric reflux, a condition in which urine flows backwards towards the kidneys, increasing the risk of recurrent infections.
With a liver transplant being considered and the risk of infection, needing to be minimized, the urinary abnormality had to be addressed before transplantation. The child therefore underwent a successful ureteric reimplantation surgery to reposition the ureter and correct the underlying abnormality. A temporary stent was subsequently placed to support the urinary system and reduce the risk of further infections during the transplant process.
Navigating this intricate medical puzzle required seamless collaboration across multiple specialties. A dedicated expert team at Rainbow Children’s Hospital, comprising Pediatric Nephrologists, Pediatric Surgeons, Hepatologists, Pediatric Transplant Surgeons, Pediatric Hemato-Oncologists, and Pediatric Intensivists came together to execute a carefully staged treatment plan to manage the complications, determine the appropriate sequence of interventions, stabilise the child and prepare him for liver transplantation. Once the urinary abnormality had been corrected and the child was medically stabilised, the liver transplant was successfully performed approximately three weeks later.
The child underwent a living donor liver transplant, receiving a portion of his father’s liver, who was found to be a suitable donor following detailed evaluation. The transplant was performed by Dr. Mettu Srinivas Reddy, Group Director – Department of Liver Transplantation & HPB Surgery, Rainbow Children’s Hospital, Chennai along with Dr. Somashekara H R, Senior Consultant – Pediatric Hepatology & Liver Transplantation, Rainbow Children’s Hospital, Guindy with the multidisciplinary team of doctors Dr. Prahlad N, Senior Consultant – Pediatric Nephrology & Kidney Transplant, Dr. Prasanna Gopal, Pediatric Transplant Surgeon, Dr. Nandhini G, Senior Consultant – Pediatric surgeon and Urologist, Dr. Karthik Narayanan R & Dr. Nataraj P, Consultant – Pediatric Intensive Care, Dr. Meena Sivasankaran, Consultant Pediatric Hemato-Oncology, and Dr. Sathish Chander, who led the anesthesiology department. The living donor liver transplant involved transplantation of the left lateral segment of the donor liver, with the procedure lasting approximately 10 hours. Following the transplant, the child was closely monitored for 8 weeks and after which he continued to receive disease-directed chemotherapy for LCH. He subsequently completed four additional rounds of chemotherapy, with follow-up evaluations showing no evidence of LCH and resolution of the recurrent urine infections.
Speaking on the occasion, Dr. Somashekara H R, Senior Consultant – Pediatric Hepatology & Liver Transplantation, Rainbow Children’s Hospital, said, “This was an exceptionally complex case because we were not dealing with a single problem. We had to manage a rare systemic disease with severe liver involvement, while also dealing with the limitations that the liver dysfunction placed on chemotherapy. When recurrent infections emerged, we had to identify and correct the underlying urinary abnormality before the child could proceed to transplant. Each decision had to be carefully coordinated with the next. The involvement of our hemato-oncology, hepatology and liver transplant, nephrology and urology teams allowed us to address these challenges together and take the child through treatment in a planned and coordinated manner.”
Adding on to this case, Dr. Mettu Srinivas Reddy, Group Director – Department of Liver Transplantation & HPB Surgery, Rainbow Children’s Hospital, said, “Performing a liver transplant in a 3.5-year-old child with significant liver involvement and multiple associated medical complications requires meticulous planning at every stage. Before transplantation, the child had to be stabilised and the recurrent infections and congenital urinary abnormality had to be appropriately addressed. From the surgical perspective, ensuring that the child was medically optimized and ready for transplantation was critical. The successful transplant was made possible through close coordination between the transplant, pediatric, oncology, nephrology and urology teams, followed by careful post-transplant monitoring and continued treatment for the underlying condition.”
Elaborating on the treatment journey, Dr. Prahlad N, Senior Consultant – Pediatric Nephrology & Kidney Transplant, Rainbow Children’s Hospital, said, “Recurrent urinary infections added another significant challenge to the child’s treatment. Given the need for a liver transplant, it was important to identify the reason behind these repeated infections and address it beforehand. Our evaluation, along with the urology team, identified vesicoureteric reflux. Gross reflux in a immunocompromised child like him, negating reflux. This was done by continuous bladder drainage with a silicon folleys prior and after anti reflux surgery. Permanent Correction of this condition was important to reduce the risk of further infections and ensure that the child was medically optimized before transplantation. Managing these associated problems alongside the primary condition was an important part of preparing the child for the next stage of treatment.”
Further to this, Dr. Nandhini G, Senior Consultant – Pediatric Surgeon and Urologist, Rainbow Children’s Hospital, Guindy, said, “Recurrent urinary infections became an important concern as the child was being prepared for liver transplantation. Further evaluation identified congenital vesicoureteric reflux, where urine flows backwards towards the kidneys, increasing the risk of repeated infections. We performed ureteric reimplantation surgery with stent placement to correct the abnormality and close monitoring. Addressing the urinary condition before transplantation was important to reduce the risk of further infections and ensure that the child was medically stable for the next stage of treatment. The child was subsequently able to proceed with the liver transplant after recovering from the procedure.