Journal of Pediatric Surgery Article Review: February 2022 BAPS Issue

Dr. Adrian Wolf's research focuses on creating renal organoids with glomerular differentiation from pluripotent stem cells to model human genetic kidney and urinary tract diseases and potentially grow a whole new renal tract.

Renal organoids can be used to test novel therapies by identifying the gene that's gone wrong in children born with abnormal kidneys, making stem cells from their blood samples, growing organoids in culture to reproduce the disease in a dish, and looking for druggable targets.

Growing a ureter from stem cells is pretty difficult, and without a ureter, even if a kidney organoid could make urine, the urine would have nowhere to go.

Renal organoids are very small, only a few millimeters across, so if used as renal replacement therapy, hundreds would need to be implanted into a person.

Blood supply is a major problem for renal organoids because they cannot be connected to a big artery, and the glomeruli could never make much urine without high pressure, high flow going into them.

In the UK study of esophageal lengthening by traction (ELBT), 16 out of 22 patients (73%) had successful esophageal anastomosis after their lengthening procedure.

In babies where ELBT doesn't work well, all managed to have a cervical esophagostomy and survive, with reconstruction typically using gastric transposition.

There were three deaths after anastomosis in the ELBT study, which was surprising even though not completely a shock.

The role of ELBT in the management of long-gap esophageal atresia will remain controversial as it is not universally successful.

ELBT should probably be a specialist center procedure, and there is a need to define how to bring these babies to the right centers.

The Netherlands has 17 million people with about 30 to 40 cases of esophageal atresia per year and 6 pediatric surgical centers.

In the Netherlands, centralization of pediatric oncology surgery began in 2014 with one hospital performing all oncological diagnoses, expanded to abdominal, thoracic, head and neck cases in 2015, and added neurosurgical and orthopedic oncology in 2018, so that now every oncological diagnosis is done in Utrecht.

In the Netherlands, biliary atresia and choledocal cysts are now done in only 1 hospital out of 6 pediatric surgical centers.

Esophageal atresia cases in the Netherlands are now done in only 3 hospitals, and anorectal malformations and Hirschsprung disease are done in 4 hospitals out of 6.

There was resistance to centralization within Dutch centers due to giving up what is a major part of practice, but outcomes overall were better when certain centers focused on certain pathologies.

At the centralized Dutch center, they perform about 30 neuroblastomas and about 35 renal tumors per year, and results improved dramatically with centralization.

Kids Operating Room (KidSOR) aims to provide modern operating theater facilities for use by local surgeons in centers in sub-Saharan Africa and Latin America.

Kids OR has a base in the UK where everything is brought in, tested, packed, cleared for customs, and then shipped, and they are sending a new operating room every 12 days.

Kids OR has opened 50 operating rooms in 22 different countries.

Kids OR supports pediatric surgery residency programs run by CSECSA (College of East, Central and Southern Africa) and the West African College of Surgeons.

Kids OR local teams unpack equipment with local engineers who recommission all equipment, provide training to the local engineer, and when they leave it is a fully functioning operating room.

Kids OR provides remote troubleshooting where if there's a problem, the local hospital engineer can do a video call and Kids OR staff can walk them through diagnosis and repair.

Kids OR will have 75 operating rooms open by the end of this year, but the end point is where there is enough capacity that every child can access care.