The patient was an 8-month-old girl. Her prenatal ultrasonography at 26 weeks of gestation revealed an abdominal cystic lesion. After birth, the patient was diagnosed with left hydronephrosis and referred to our hospital. Ultrasonography showed grade IV hydronephrosis in the left kidney. MAG3 scintigraphy showed no function in the left kidney. Cystoscopic retrograde pyeloureterography revealed advanced stenosis throughout the entire length of the left ureter. For intraoperative finings, the ureter was extremely thin with 2.6 mm outer diameter and the renal parenchyma was mostly scarred. Since it was difficult to place a stent in the ureter, nephrectomy was performed. For pathological findings, fibrosis with cartilage tissue in the residual renal parenchyma was observed and renal dysplasia was confirmed. Nephrectomy should be considered for advanced ureteral hypoplasia in patients with the entire ureteral hypoplasia because the diseased kidney is often accompanied by dysplasia or severe hypoplasia.

Uretero-appendicular fistulas represents a rare entity, closely related to appendicitis and are usually encountered intraoperatively. A literature review showed six published cases –only one of them involving a pediatric patient. We describe the case of a ten year-old girl with history of recurrent urinary tract infections (UTIs) since the age of three. Following a six-year asymptomatic period, she was referred for evaluation after a new diagnosis of UTI complicated by a right distal ureteral stone. The patient underwent ureteroscopy and laser stone fragmentation. During the endoscopic procedure a mature, well-epithelialized ureteral tract to the peritoneal cavity was encountered. The procedure was converted to open exploration through a right Gibson incision, where an appendicular phlegmon associated with an uretero-appendicular fistula with passing of faecoliths to the ureteral lumen was found. An appendectomy was performed along with primary fistula closure. Uretero-appendicular fistulas represent a rare entity. Our case highlights the relation between this type of fistula with appendicitis, its unexpected intraoperative diagnosis and the unusual presentation, as the patient did not present clinical findings compatible with acute abdomen.

Testicular torsion is one of the most common causes of acute scrotum in children and adolescents. The bell-clapper deformity, which detected in 12% of males, is the most important reason that leads to testicular torsion. In our case, a 14 years old male admitted to our clinic due to testicular torsion developed after ejaculation with manual sexual stimulation of the penis. The most important criteria in determining the loss of testis is the degree and duration of torsion. Here, we discussed the rare cause of testicular torsion along with diagnostic and therapeutic characteristics.

Indirect inguinal hernia is most commonly seen in infants. Prevalence is lower in girls. Both uterus and ovary including hernia sac is extremely rare. Early diagnosis and surgery is critical, since strangulation and necrosis of these organs may lead to serious conditions and infertility. Doppler Ultrasound (US) is the effective method in differentiation and diagnosis of inguinal hernias of infants. In this very rare case, according to the literature knowledge, we aimed to represent the sonographic and surgical features of a 40 day old vaginally delivered term female infant admitted to our clinic with right groin mass and diagnosed as indirect incarcerated inguinal hernia containing viable uterus, fallopian tube and ovary via US. She went under early surgery through which ovary, fallopian tube and uterus were found in hernia sac. All herniated organs were reduced into peritoneal cavity and the hernia sac was high –ligated.

Bladder exstrophy is a rare developmental anomaly. Four principle variants of bladder exstrophy have been described and they themselves are rarer than the bladder exstrophy. Authors describe the management of a case of pseudoexstrophy type of variant in a 9 month old male child with penile duplication with torsion and coronal hypospadias. The rectal fascial defect was repaired without osteotomy as the distance between two pubic bones was <4 cm (3.1 cm). Genital reconstruction with excision of duplicate atrophic penile shaft and repair of coronal hypospadias with detorsion of the functional penile shaft could be accomplished. The patient had good outcome in terms of cosmesis and urinary stream. Total of 18 cases of the pseudoexstrophy have been described till date. Pseudoexstrophy of bladder is a very rare condition and can simultaneously present with other defects like omphalocele, anorectal malformations, pouch colon, multiple or solitary urogenital anomalies. The principles of correction though remain same with correction of abdominal wall defect with or without osteotomy depending upon severity of pubic diastasis. Other anomalies can undergo treatment as per standard protocol.

Management of retained encrusted ureteral stent is challenging. A 5 years old boy with congenital right solitary pelvic kidney found to have missed totally encrusted ureteral stent with large renal pelvis, ureter and bladder stone. The age of patient, anatomical consideration and severity of encrustation make the management options limited. Single incision and pyelolithotomy, ureterolithotomy and cystolithotomy with removal of the stent was carried out. Key Words:

Hair tourniquet syndrome is characterized by circumferential strangulation of an appendages or genitalia by human hairs or fibres. Here we are reporting a rare case and its successful management of coronal urethrocutaneous fistula developed due to penile hair tourniquet syndrome (PHTS) in a 5 years old child.