Reviewer response for version 1
The authors present a fascinating case of Prune Perineum Syndrome - the first case of survival beyond infancy. 
Only two others have been reported with PPS over the past half century. 
The experienced group of pediatric surgeons describe this exceedingly rare condition and offer the child an opportunity to improve quality of life through surgical reconstruction. 
The preoperative imaging studies are highly educational and instructive. 
Unfortunately, a delayed postoperative complication and poor access to emergency medical care lead to the patient’s demise. 
This report, however, provides important clinical information to the limited available literature on this condition. 
This case report will help to educate and guide future generations of patients and their families, as well as physicians and surgeons in the diagnosis and management of PPS.
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