M. Sica

Herlyn–Werner–Wunderlich syndrome: An “early” onset case report and review of Literature

Highlights • We describe a case of a 3 years old girl, with all features of Herlyn–Werner–Wunderlich syndrome (HWWS) who came to our attention for lower abdominal mass.• Only six cases are reported in Literature with early onset of this syndrome under 5 years.• The aim of this article is to share our experience and focus the attention on the importance of high level of suspicion of HWWS in neonatal period to early diagnosis and treatment.• The possible early presentation of this syndrome should be suspected in all neonates (females) with renal agenesia confirmed postnatally or with prenatal diagnosis.• An error of evaluation with planning of removal of mass, that can damage patients in term of chance for a successful reproductive outcome.• Our team consider HWWS as differential diagnosis in newborn with prenatal ultrasonography of a cystic mass behind the urinary bladder in the absence of a kidney and plan a pelvic ultrasound (with aim to identify an uterus, normal or dydhelfus, and presence or absence of pelvic mass), an examination under anesthesia and cisto-vaginoscopy, if it is necessary. A high level of suspicion, indeed, is the key to early diagnosis.

Split-appendix technique: Alternative urinary diversion for pediatric complete incontinence

We report our series of selected patients with complete incontinence in whom the appendix was divided and utilized for creating two continent catheterizable stomas. All patients were treated for urinary and fecal incontinence by split appendix technique. The appendix was divided into two different parts preserving adequate perfusion and used for creating an appendicocecostomy (ACE) and an appendicovesicostomy at the same time. After a clinical and radiological follow up, our patients referred a good acceptance and an easily management of the stomas in order to stay dry for all day from urine and feces with improving of their quality of life. The combination of ACE and Mitrofanoff principle have revolutionized the management of urinary and fecal incontinence in patients who are unable to utilize their urethra to keep themselves dry.

Isolated Vaginal Agenesis Associated with Multiple Gastrointestinal Anomalies: A Case Report

More than 50% of infants with esophageal atresia have associated anomalies. We present a case report of a 46XX neonate with long-gap esophageal atresia and tracheoesophageal fistula (EA/TEF), anorectal malformation, bowel duplication and vaginal agenesis. This is an unusual association of abnormalities which had not yet described in literature.