certain colorectal conditions , such as ulcerative colitis and diffuse polyposis of the colon and rectum , and congenital intestinal abnormalities , such as total colonic aganglionosis , require a proctocolectomy and some type of intestinal restoration 1 .
although these procedures are technically demanding for surgeons , they are more challenging for the patients following the restoration of intestinal continuity .
frequency of bowel evacuations typically increases dramatically postsurgery , contributing to induced metabolic derangements 2,3 .
we present a case of juvenile polyposis coli , initially treated with a subtotal colectomy and ileostomy , followed by a proctectomy , ileal - j - pouch and serial transverse enteroplasty ( step ) of the distal ileum 4 .
an eight - year - old female patient was referred to our department with a chronic history of unremitting bloody diarrhea , weight loss , anemia and intractable rectal prolapse .
there was no other significant past medical history or family history and her vaccinations were up to date .
the initial clinical examination revealed a pale child who was underweight for age with mild clubbing of the fingernails .
the abdominal examination was unremarkable , and the rectal examination was unnecessary because the cause of the prolapsed rectum was obvious , with a presentation of multiple polyps ( fig1 ) .
blood tests confirmed clinical evident anemia with a hemoglobin of 7  g / dl , and her albumin level was 19 
rectal polyposis was ratified by sight ( fig1 ) , and total colonic involvement was suspected on the contrast enema ( fig2 ) and was substantiated by colonoscopy ( fig3 ) and by the subtotal colectomy specimen ( fig4 ) .
the histological diagnosis of hamartomatous juvenile polyposis was confirmed from the colonoscopic biopsies and the operative specimen .
despite the initial temporizing approach of subtotal colectomy and ileostomy , anemia and protein - losing enteropathy persisted .
the patient was discharged with dietary supplementation , and she had regular outpatient follow - up .
the child s nutritional status improved over time , and she had albumin levels consistently over 36 
a proctectomy was performed to remove the residual rectal polyps , and the ileostomy was taken down and fashioned as a j - pouch ( fig5 ) .
multiple serial enteroplasties ( fig6 ) starting just above the pouch over a length of approximately 80  cm was performed , followed by a hand sewn ileo - anal anastomosis .
the postoperative course was uneventful , and the patient was discharged 2  weeks later . at that stage , she was passing two soft stools per day without any medication or dietary restrictions .
the follow - up at 3  months postsurgery confirmed a well - child who had gained 1.5  kg .
she had returned to school and continued to pass one to two soft stools per day .
restorative proctocolectomy has replaced disparate surgical options such as a hartmann s procedure and permanent ileostomy or ileo - rectal anastomoses because of their technical inadequacy , inability to improve quality of life and persistent cancer risk 5,6 .
it removes the diseased intestine , thereby improving quality of life and reducing cancer risk 3 .
intestinal leaks and pouchitis are major morbidities that might result in a long term or permanent ileostomy . an increased frequency and volume of enzyme - rich liquid stools
although dietary restrictions and opioids might temporarily reduce the symptoms , they fail to cure these patients 7,8 . in these cases ,
the postoperative quality of life is indisputably not better than the pre - operative status .
it is well reported that patients affected by severe short bowel syndrome benefit from the step procedure , because such an intervention can significantly reduce the frequency of stooling 913 .
such large volumes must therefore have time and an appropriate intestinal length for water , electrolytes and other nutrients to be absorbed .
this process is expected to occur within the j or other pouch configurations following restoration 14,15 .
these pouches are quickly filled , and they easily surge ; the anal sphincter does not effectively counteract and contain the volume , which is usually controlled by the ileocaecal valve .
large amounts of nutrients , electrolytes and water are wasted because evacuation is inevitable and continence training is not possible .
step is a technique for lengthening the intestine in short bowel syndrome by interrupting the intestinal lumen thereby also slowing peristalsis .
this procedure allows time for the nutrients , water and electrolytes contained within the intestinal lumen ( and distant from the distal anal anastomosis ) to be absorbed 4,11 .
the outcome of our technical option was rewarding because the stool frequency and consistency were immediately in an acceptable range of two to three times per day and a soft consistency , respectively .
this outcome was achieved without the use of opioids or the need for dietary restrictions .
the child tolerated an unrestricted hospital diet and was discharged after 2  weeks when we were certain of her bowel habits and that she had maintained her physiological parameters . at 3  months follow - up , she was growing well and maintained a stool frequency of one to two soft stools per day .
stool frequency can be crippling following proctocolectomy , and although opioids might provide temporary relief of the symptoms , they can not prevent relapses .
we believe that step alone , performed in an adequate length of bowel , can control the stooling of such patients .
 key clinical messagewe present a pediatric case of medically unmanageable juvenile colonic polyposis , initially treated with subtotal colectomy and an ileostomy followed by a proctectomy , ileal - j - pouch and serial transverse enteroplasties ( step ) of the distal ileum . the step procedure in an adequate length was able to control stooling of our patient . 
Introduction
Clinical case
Discussion
Conflict of Interest