Giant Omphalocele in an Adolescent Boy.

Omphalocele is a congenital abdominal wall defect that permits herniation of abdominal viscera into the umbilical cord. We here report a case of a giant omphalocele in an adolescent boy that has not been reported at this age before.

Embryogenesis and types of subcostal hernia--a rare entity.

BACKGROUND/PURPOSE: Four infants with congenital subcostal hernia are reported, as it is a rare entity with only two cases previously reported. Further, there are no reports concerning the complex multisystem subtype. Embryogenesis of the associated anomalies and subcostal hernia and their management are discussed. MATERIALS/METHODS: Clinical features, history, investigations, associated anomalies, and management data of four patients with subcostal hernia were collected and analyzed. RESULTS: The following associated anomalies were detected: renal agenesis (2), musculoskeletal abnormality (3), congenital heart disease (2), müllerian-renal-cervicothoracic somite abnormalities and vertebral-anorectal-cardiac-tracheoesophageal-renal-radial-limb anomalies (1). The subcostal hernias were treated by laparoscopic assisted (3) or laparoscopic herniorrhaphy (1). CONCLUSIONS: Subcostal hernia is a rare entity with varied clinical presentations and presents either as an isolated defect or as a complex multisystem defect. The exact etiology is still unknown. Phenotypic manifestation of the complex defect is probably due to developmental gene defect affecting the coordinated growth of mesoderm around 4th to 10th weeks of fetal life.

Ectopic testis in children: experience with seven cases.

BACKGROUND: Ectopic testis is a rare congenital anomaly in which the testis is abnormally located away from normal line of decent. AIM: To report varied clinical presentation, embryogenesis, and management aspects of ectopic testis with a brief review of the literature. MATERIALS AND METHODS: A retrospective chart review of children with undescended testis from January 2008 to August 2011. RESULTS: Seven children (3.6%) treated for ectopic testes were diagnosed among 190 children operated on for undescended testis. There were five perineal testes, one penile testis, and one transverse testicular ectopic testis. Laparoscopy was the diagnostic and therapeutic modality in transverse testicular ectopia. Other ectopic testes were managed by open orchidopexy. The length of the testicular vessels and vas deferens was adequate in every case. CONCLUSION: Examination of boys with an empty scrotum should include examination of ectopic sites as well. The gubernaculum bulb has preprogrammed growth toward the scrotum unless anatomical blockade prevents its descent. Open orchidopexy reveals normal characteristics of perineal, penile testis, and its elements. Surgical correction for ectopic testis as early as possible facilitates proper psychological development and prevents complications.

Chronic epididymitis with scrotal vasocutaneous urinary fistula.

Chronic Epididymitis is a relatively rare problem in boys and is often enigmatic in its etiology. It is often associated with urinary tract abnormalities in infants and prepubertal children. This report describes a rare and unusual case of a chronic epididymitis with acquired scrotal vasocutaneous urinary fistula in an 8-mo-old boy who was found to have a large prostatic utricular cyst and ectopic vas insertion. The authors discuss embryology, pathophysiology, diagnostic dilemma and different treatment options.

Urethral duplication: Experience of four cases.

AIM: Our experience of 4 cases of urethral duplication is reported here. MATERIALS AND METHODS: A retrospective chart review. RESULTS: The age at presentation varied from newborn to 10 years. The clinical presentation ranged from prepubic sinus to diphallus urethra. There were 2 each incomplete duplication with only external openings (Type IA) and complete duplication of Effmann Type IIA2. All underwent complete excision of accessory urethra and corrections of associated anomalies. CONCLUSIONS: Urethral duplications have a varied presentation. At follow up, all are asymptomatic with good cosmetic result.

Megacystis-microcolon-intestinal hypoperistalsis syndrome associated with prune belly syndrome: a case report.

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome is a quite rare congenital anomaly that presents with a functional obstruction of the gastrointestinal tract which is usually fatal. It is three to four times more prevalent in females. We present a case of a rare association of a male neonate with Megacystis Microcolon Intestinal Hypoperistalsis Syndrome who in addition had the classical triad of Prune Belly Syndrome and thus suggest a possibility of different spectrums with a common pathogenesis.