A very rare syndrome and its rare urological complication: Incomplet bladder duplication in Robinow syndrome.

OBJECTIVES: Robinow syndrome is a very rare syndrome characterized by short stature, extremity deformities, costovertebral abnormalities, renal/external genital malformations, and fetal facial appearance. It might be inherited by either autosomal dominant or severe recessive form. Diagnosis is generally established by the aid of genetic mutation and phenotypic findings. The urogenital component of the disease frequently presents with microgenitalia such as micropenis and/or cryptorchidism. METHODS: Herein, a four-year-old boy with Robinow syndrome accompanied by incomplete bladder duplication is discussed. RESULTS: The duplication in the bladder was screened by cystoscopy and corrective surgery was performed. CONCLUSIONS: This rare manifestation is the first for urological findings of Robinow syndrome in literature.

Bladder augmentation in exstrophy vesicae: Long-term results of a single experienced center.

BACKGROUND: In this study, we present our long-term treatment outcomes of bladder augmentation (BA), bladder neck closure, and Mitrofanoff clean intermittent catheterization (CIC) in managing bladder exstrophy (BE). METHODS: This was a retrospective medical records' review of 33 children diagnosed as born with BE, followed up at a tertiary pediatric urology clinic from 1988 to 2020. Outcomes such as surgical interventions, presence of renal calculi, hydronephrosis, and continence status were extracted. RESULTS: The median follow-up of the group was 18.2 (4-26) years. Urinary system stones developed in 10 (30.3%) cases about 8.9 years after BA. Stone development was two times more common in patients who underwent colocystoplasty (33.3%) than those who underwent ileocytoplasty (16.6%). The state of continence of the group was satisfactory in 26 (78.7%; excellent in 23; good in 3 cases) and unsatisfactory (wet) in 6 (18.1%) cases. At the last visit, ultrasonography revealed no hydronephrosis in 23 (69.6%) patients, and the voiding cystourethrogram demonstrated low-grade vesicoureteral reflux in 10 (30.3%) and high-grade vesicoureteral reflux in 2 (6%) patients. CONCLUSIONS: An elaborated plan of surgical reconstruction for classic BE can lead to satisfactory long-term urinary continence in most patients. Ultimate predictors of outcome in BE repair are difficult to ascertain. Consistently, BA, bladder neck closure, and Mitrofanoff CIC continue to stand out at a critical point in the management of those patients with classic BE. Our study demonstrated that augmentation is required to achieve acceptable dryness with high satisfactory dryness rates in BE.

A very rare syndrome and its rare urological complication: Incomplet bladder duplication in Robinow syndrome

Objectives: Robinow syndrome is a very rare syndrome characterized by short stature, extremity deformities, costovertebralabnormalities, renal/external genital malformations, and fetal facial appearance. It might be inherited by either autosomaldominant or severe recessive form. Diagnosis is generally established by the aid of genetic mutation and phenotypic findings.The urogenital component of the disease frequently presents with microgenitalia such as micropenis and/or cryptorchidism.Methods: Herein, a four-year-old boy with Robinow syndrome accompanied by incomplete bladder duplication is discussed.Results: The duplication in the bladder was screened by cystoscopy and corrective surgery was performed.Conclusions: This rare manifestation is the first for urological findings of Robinow syndrome in literature. (AU)

The Effect of Technical Problems on the Operation Process in Pediatric Laparoscopy.

OBJECTIVES: The aim of this study was to determine the technical problems in pediatric laparoscopic surgery and to evaluate its results. METHODS: The technical problems encountered in 30 laparoscopic operations performed between 3 November 2012 and 31 December 2017 were retrospectively analyzed. RESULTS: The technical problems experienced in 30 laparoscopic surgeries were analyzed. There were 6 splenectomies, 15 appendectomies, 1 hernioplasty, 2 ovarian cyst excisions, 4 cholecystectomies, 1 intra-abdominal exploration of the testes, and 1 varicocele surgery. The technical errors included instrument failure in 10, human errors in 8, device problems in 12, and multiple problems in 2 cases. In 5 patients, we switched to open surgery. CONCLUSION: Technical problems prolong the operation, and cause a conversion to open surgery. In order to solve these problems, it is necessary to register and report these problems and take the necessary preventive measures.