Pediatric Urology Case Reports

119-122

Authors: Vini Joseph, Suraj Gandhi, Syamantak Basu, Neha Sisodiya Shenoy, Hemanshi Shah

We present a rare case of exstrophy variant - inferior vesical fistula. A two-year-old boy with normal continence presented with a suprapubic pit and mucosal plate on the penile dorsum. Examination and cystoscopy revealed vesical fistula. The fistula was closed and the penis was reconstructed. Recognition of the exstrophy variant is important because the treatment and prognosis are very different.

127-131

Authors: Volkan Sarper Erikci, Ali Sayan, Gokhan Koyluoglu

Hair tourniquet syndrome (HTS) is a rare condition and may pose diagnostic dilemma. The purpose of this study is to report our experience in 3 female cases with HTS involving genitalia. All the patients in this series presented with swollen and edematous genitalia and clitoris accompanied by strangulation in clitoris or labia minora with a hair thread. Strangulating hair was excised and removed under local anesthesia. The pain was cleared away and edematous discoloration disappeared immediately. Local application of antibiotic ointment was added to the treatment in these cases for a period of 6-7 days. The anatomy of the genitalia was found to be normal in these children during the follow up exam. A brief literature review on this subject is also given in this review.

137-140

Authors: Cristiana Maximiano, Albina Silva, Jorge Correia-Pinto, Angélica Osório

The acute scrotum is usually due to testicular pathology, but, less often, it could be related to abdominal or retroperitoneal conditions, such as adrenal hemorrhage. The adrenal hemorrhage in the newborn is a rare condition with prevalence of 0.2%. We present a case report of an adrenal hemorrhage in a full-term neonate. The conservative management was performed, with periodic clinical and radiological follow-up. There was a favorable outcome with complete resolution of hematoma at 12 weeks. Within this unusual case, the authors want to emphasize the fact that in presence of acute scrotum it is imperative to exclude testicular torsion. The Doppler ultrasonography is a valuable resource but it should be allied with minicious physical examination. The conservative management is recommended in the majority of the cases of adrenal hemorrhage, avoiding unnecessary exploration.

95-99

Authors: Ruankha Bilommi

Malignant peripheral nerve sheath tumors (MPNST) are rare, aggressive soft tissue sarcomas arising from peripheral nerves that present great challenges to known treatment modalities. Here, we present a case of MPNST involving the dorsum of a penis in a 13-month-old boy. The mass was incomplete excised and a circumcision was performed. Histological study revealed spindle cell tumor of the lesion. Immunohistochemistry showed a strong positive staining for tumor cells. A high grade malignant peripheral nerve sheath tumor was diagnosed.

106-110

Authors: Agnese Roberti, Francesco Turrà, Maria Esposito, Anna Maria Cotrufo, Federica Marinoni, Giovanni Di Iorio

In 1986, Fowler and colleagues described a condition currently known as Fowler's syndrome in young women with unexplained urinary retention associated with polycystic ovary syndrome. Herein, we present a case of a 13-year-old woman diagnosed with Fowler's Syndrome as a result of clinical, radiological and urodynamic electromyogram (EMG) findings. Fowler's syndrome should be considered in the differential diagnosis in children with unexplained chronic urinary retention.

114-118

Authors: Yasar Issi, Sedat Simsik, Unal Bicakci

A four-year-old boy presenting with neuropathic bladder and hydrocephalus developed Fournier’s gangrene of the scrotum and perineum. At first, he was in septic shock and intubated. After debridement and grafting, he was taken to the normal service room from the intensive care unit. He was discharged on the 29th postoperative day. We performed bilateral orchiectomy due to testicular involvement and also repair and flap application to urethra due to its involvement. We think that our case is interesting because Fournier’s gangrene is already rare in children and our case also contains atypical involvement sites such as testes and urethra.

70-73

Authors: Ahmed Ibrahim Balah, Ahmad Beltagy, Mohammed Youssef, Waleed Dawood

We reported a case of donkey bite to the external genitalia of a 10 years old child. We had performed delayed reconstruction of the penis following adequate saline washing and wound debridement; under antibiotic coverage. Previous studies have shown that delayed repair together with strict wound management resulted in satisfactory outcomes in most victims of such animal bites.

79-82

Authors: Loganathan Arun Kumar, Jujju Jacob Kurian

Ureteral atresia is a rare congenital anomaly and is usually associated with renal dysplasia. Distal atresia is more common and other associated urinary anomalies are rare. We report a case of an eight year old boy who presented with left flank pain and vomiting. Ultrasound abdomen and pelvis and renal scintigraphy were suggestive of hydroureteronephrosis with obstructed drainage. He was suspected to have ureteric stricture and taken up for surgery. Cystoscopy and retrograde pyelogram revealed an incomplete duplication with lower moiety ureter ending blindly. Exploration of left renal fossa showed a duplex anomaly with normal upper moiety and hydronephrosis of lower moiety with a ureteric stump. Ureteropelvic anastomosis was done between the dilated renal pelvis and the blind ending ureter. Patient was doing well post operatively with good function in both moieties. Review of literature shows only two previously reported cases of proximal ureteric atresia. Preservation of renal function in an older child has also been reported only once. Ureteral atresia is usually diagnosed intra-operatively and various reconstruction options are available in patients with preserved function.

88-94

Authors: Rajendran Ramaswamy, Rayan Ahmed Baz, Marwan Alchami, Ghazi Mukattash

Absence of any orifice in perineum, presence of smooth perineum, and absence external genitalia are characteristic of cloacal dysgenesis sequence (CDS). Newborn of 40w gestational age, born to mother with oligohydramnios, had very low APGAR scores, and was put on ventilator. Child had dysmorphic facies, short neck, compressed chest, no urinary bladder swelling, absence of any perineal orifice, a raised fold of skin at the site of phallus and bilateral talipes calcaneovalgus. Esophageal atresia (EA) was diagnosed clinically and radiographically. Pulmonary hypoplasia, distal tracheoesophageal fistula (TOF), thin, flat and broad cervical vertebrae with spina bifida, partial sacral agenesis and cardiac anomalies were demonstrated by radiographs and echocardiography respectively. Child progressively more desaturated on ventilator and died at 16h after birth before detailed assessment including sex could be done. CDS results from failure of urorectal septum to fuse with cloacal membrane at 7th week of intrauterine life. It can occur in male and female. CDS is lethal resultant from associated severe pulmonary hypoplasia and renal insufficiency. Very rarely such child is born alive as in our case. Association of CDS with EA+TOF is extremely rare. In view of 5 long-term survival reports of CDS, prenatal diagnosis is warranted and treatment should be initiated.

51-54

Authors: Hamdi Ozcan, Canan Ceran

Behçet’s disease (BD) is a rare systemic inflammatory disorder of the blood vessels and tissues. It is rarely seen in children. The clinical course of the disease in children is similar to that of the adults by symptoms such as recurrent oral ulcers, genital ulcers and ocular inflammation. Additionally, urological manifestations rarely seen in BD. Here we present two children with BD and epididymoorchitis confusing with testicular torsion. Since BD is rarely observed in children, it is very difficult to diagnose the patients with epididymo-orchitis. Treatment for the testicular torsion is immediate surgical therapy to prevent the testicular necrosis. On the other hand epididymo-orchitis caused by BD should be treated medically. Unnecessary surgery on BD patient can affect morbidity negatively. We aimed to take attention to this rare condition to prevent the patients from unnecessary operation.

59-61

Authors: Tiffany Perkins, Dennis Peppas, Eran Rosenberg

Children are more likely to be exposed to blunt trauma-induced kidney damage due to the lack of perirenal adipose tissue, weakness in the abdominal muscles, and less ossified thoracic cage. Grade 5 renal injury may be an indication for urgent surgical intervention. Here, we describe a case of pediatric blunt trauma with initial computerized tomography (CT) scan demonstrating complete renal devascularization suggestive of grade 5 renal injury. However, subsequent imaging demonstrated normal vascularization of the kidney. Renal artery spasm was likely the cause for the initial CT findings, which mimicked a grade 5 renal injury.

65-69

Authors: Mehmet Mazhar Utangac, Serdar Gundogdu, Bilge Turedi, Mehmet Ogur Yilmaz, Mehmet Emin Balkan, Nizamettin Kilic

Presence of multiple calculi in a single system ureterocele is a rare condition. A 3-year-old boy presented with recurrent urinary tract infections in whom multiple calculi were noted in the urinary bladder on x-ray and ultrasound scan. In addition, ultrasound showed the presence of ureterocele in the size of 18x15x12 mm. Open surgery revealed an ureterocele with multiple stones. Here, we present a boy with multiple stones in the left ureterocele diagnosed intra-operatively due to its rarity, etiology and treatment options.

21-32

Authors: Deidra C. Crews, Aminu K. Bello and Gamal Saadi; for the World Kidney Day Steering Committee

Kidney disease is a global public health problem, affecting over 750 million persons worldwide. In many settings, rates of kidney disease and the provision of its care are defined by socio-economic, cultural, and political factors leading to significant disparities. World Kidney Day 2019 offers an opportunity to raise awareness of kidney disease and highlight disparities in its burden and current state of global capacity for prevention and management.

38-41

Authors: Tunc Ozdemir, Ali Sayan, Ahmet Arikan

We herein report two patients who underwent Leadbetter-Politano ureterocystoneostomy whose ureters were passed through sigmoid colon during operation. Presentations and treatment strategies of these patients were discussed.

46-49

Authors: Erika Llorens de Knecht, Santiago Guadarrama Vega, Claudia Quintian Schwieters, Anna Bujons Tur

Thrombosis of an extraspermatic blood vessel has been reported to be an unusual cause of acute scrotum. We describe the case of a 7-year-old boy who presented with an acute painful left scrotal swelling. Imaging demonstrated a heterogeneous left scrotal mass, which was surgically resected. The anatomopathologic study revealed a thrombosed vascular structure. Three months later, another extraspermatic venous thrombosis occurred and was also resected. A complete thrombophilia study was done. A G20210A prothrombin gene heterozygous mutation was identified as the cause of the thrombotic event. A venous thrombosis should be considered as a possible cause of acute scrotum in European Caucasian patients, due to its increased prevalence. We reviewed the G20210A prothrombin gene mutation, which is associated with thrombosis.