Innovative Cross-Team Care for Complex Multicystic Dysplastic Kidney Patient Results in Promising Outcome
Coordination Spans Intervention In Utero to Successful Kidney Transplant
It sometimes takes a village to help a baby survive and thrive. For Baby E, diagnosed prenatally with left multicystic dysplastic kidney (MCDK) and absent right kidney, the collective expertise of Cincinnati Children’s fetal team, nephrologists, urologists, transplant surgeons and many others has been lifegiving and lifechanging.
“This is one of only a handful of babies nationally who was anuric in utero to make it from fetal care to transplant,” says David K. Hooper, MD, MS, medical director of Kidney Transplantation.
The fetal team discovered at 21 weeks gestation that Baby E had one nonfunctioning kidney. Maternal-fetal specialists at University of Cincinnati Medical Center intervened by injecting eight amnioinfusions to replace absent amniotic fluid and allow her lungs to develop. As Baby E matured, Foong-Yen Lim, MD, surgical director of the Cincinnati Children’s Fetal Care Center, coordinated teams of specialists at the two hospitals and provided the bridge from prenatal to postnatal care.
“In most places, she would not have survived to birth or beyond,” Hooper says.
Pediatric nephrologist Donna Claes, MD, MS, and her dialysis team adapted equipment to put the newborn on hemodialysis postpartum to filter her blood, and aquapheresis to remove excess fluids and salt. After two weeks, Claes transferred her to peritoneal dialysis, and discharged Baby E to home after four months. The team guided the family over the next two years with home peritoneal dialysis, a gastrostomy tube and medications, and maintained contact during hospitalizations, surgeries and clinic visits.
In the spring of 2021, at age 25 months, Baby E received a kidney donated by her mother.
“Cooperation among our disciplines has been central to our efforts to care for a patient of this complexity,” says pediatric transplant surgeon Greg Tiao, MD.
Tiao performed successful transplant vascular reconstruction for the kidney transplant. Pediatric urologist Pramod Reddy, MD, joined the procedure to perform a ureterostomy, connecting Baby E’s ureter to a stoma in her abdomen.
Reddy’s team also provided prenatal counseling and will discuss options for reconstructing the child’s urinary tract when she is 5 or 6.
Hooper sees Baby E in the transplant clinic and is enthusiastic about her progress: “This is survival with quality of life. She’s happy and playful and learning to walk. This is a paradigm shift.”
Baby E was the first child born without any urine production to receive a kidney transplant at Cincinnati Children’s. Since then, at least two more patients with challenges similar to Baby E’s received a transplant at Cincinnati Children’s.
“This achievement makes us one of the most experienced centers in the world in transplanting this rare patient population,” Reddy says.