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A Staphylococcus epidermidis was isolated both from the ventricular catheter and CSF and from the subphrenic abscess and the gallbladder. To our knowledge, this is the first report of cholecystitis evolving as a descending shunt infection. The current literature related with this unique complication is briefly reviewed.

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We highlight the need to maintain restricted the clinical diagnosis for HDS to those concordant with a great disorganization of morphogenetic inductions affecting the three germ layers, which occur during the first four weeks of development. This is crucial to: (a) perform a correct diagnosis, which is essential to establish the prognosis and surgery procedures, (b) identify which is/are the cause/s, and (c) the adequate genetic counseling.

The incidence of constipation does not differ from that found in the literature. Faecal incontinence is slightly improved compared to what has been published. However, urinary tract infections and incontinence are more prevalent in our series. Five patients out of the eight that responded suffered from psychosocial problems, according to DAS-59 questionnaire. Patients with SCT require urological, bowel, and psychological counselling, until they have a complete functional and emotional development.