Pediatric Urology Case Reports

25-34

Authors: Kamyar Kalantar-Zadeh, Philip Kam-Tao Li, Ekamol Tantisattamo, Latha Kumaraswami, Vassilios Liakopoulos, Siu-Fai Lui, Ifeoma Ulasi, Sharon Andreoli, Alessandro Balducci, Sophie Dupuis, Tess Harris, Anne Hradsky, Richard Knight, Sajay Kumar, Maggie Ng, Alice Poidevin, Gamal Saadi, and Allison Tong, for the World Kidney Day Steering Committee

Living with chronic kidney disease (CKD) is associated with hardships for patients and their care partners. Empowering patients and their care-partners, including family members or friends involved in their care, may help minimize the burden and consequences of CKD related symptoms to enable life participation. There is a need to broaden the focus on living well with kidney disease and re engagement in life, including an emphasis on patients being in control. The World Kidney Day (WKD) Joint Steering Committee has declared 2021 the year of “Living Well with Kidney Disease” in an effort to increase education and awareness on the important goal of patient empowerment and life participation. This calls for the development and implementation of validated patient-reported outcome measures to assess and address areas of life participation in routine care. It could be PEDIATRIC UROLOGY CASE REPORTS ISSN 2148-2969 http://www.pediatricurologycasereports.com Pediatr Urol Case Rep. 2020; 8(2):25-34 26 supported by regulatory agencies as a metric for quality care or to support labelling claims for medicines and devices. Funding agencies could establish targeted calls for research that address the priorities of patients. Patients with kidney disease and their care-partners should feel supported to live well through concerted efforts by kidney care communities including during pandemics. In the overall wellness program for kidney disease patients, the need for prevention should be reiterated. Early detection with a prolonged course of wellness despite kidney disease, after effective secondary and tertiary prevention programs, should be promoted. WKD 2021 continues to call for increased awareness of the importance of preventive measures throughout populations, professionals, and policy makers, applicable to both developed and developing countries.

40-43

Authors: Luiz G. Freitas Filho, André Lazzarin Marani, Luiz Felipe Brollo, Natasha Mourão, Bruna Cecilia Neves Carvalho, Luiz J. Budib

A four-year-old boy presented painful episodes of priapism always before emptying the bladder since he was two years old, which led to investigation for a possible arteriovenous fistula of the perineal vascular system or a cavernous artery hyper flow. Following different exams in an attempt to establish the diagnosis, the child, actually, was found to have posterior urethral valves. The diagnosis was totally unforeseeable and the urethral valves endoscopic treatment reduced the priapism events with a positive effect on the disease.

49-54

Authors: Kevin Nguyen, Mark Pisaneschi

We present a case of ectopic urethral ureteral insertion in a 2-week old female diagnosed on a voiding cystourethrogram after abnormal prenatal and postnatal renal ultrasounds. The case is remarkable due to initial opacification of only the left upper pole moiety without bladder opacification on the voiding cystourethrogram due to direct canalization of the left ureter. Prompt diagnosis is important to prevent frequent urinary tract infections and urinary incontinence with lower ectopic urethral ureter insertions. Voiding cystourethrogram is an important tool in the workup of renal anomalies and is typically used to evaluate for reflux, however, keen attention can reveal other abnormalities such as ureteral ectopia in rare instances.

1-6

Authors: Omar Alfraidi, Safiyh Aljohani, Tariq Burki, Faris AlHarbi , Rahaf BinManie , Ahmad Alshammari, Fayez Al Modhen

There are many different variants of classical bladder exstrophy (BE) and covered BE is one of these rare variants which has an association with heterotropic hind gut duplication cyst. We report a case of a 20 months old girl with covered BE, who had a 5 cm cystic mass attached anteriorly to the bladder, which turned out to be a hind gut duplication cyst on histopathology examination. So a possibility of a heterotopic hind gut duplication cyst should be kept in mind if a cystic mass is found anterior to a covered BE.

11-14

Authors: Farzaneh Sharifi Aghdas , Seyyed Mohammad Ghahestani , Mohammad Hamidi Madani , Alireza Zadmehr , Hossein Fowzi Fard, Hamed Hasani

Acute scrotum is a common clinical problem in children and torsion event superimposed on complete dissociation of the testicle and epididymis can be difficult to diagnose and often requires scrotal exploration. To the best of our knowledge, four cases of isolated torsion of the epididymis have been reported in the literature. We report a 17-year-old male patient with epididymal torsion who presented with testicular pain and hemi-scrotum swelling. Due to painful symptoms, emergency scrotal surgery exploration of the patient revealed a complete epididymis infarction due to isolated epididymal torsion with dissociation of the testis and epididymis.

21-24

Authors: Dua Cebeci , Sirin Yasar , Pembegul Gunes

Lichen scleroatrophicus (LS) is a chronic, progressive, inflammatory dermatosis of the skin and mucous membranes, initially characterized by ivory-colored, oval and sharply demarcated papules, plaques and atrophy. The cause of the disease is still unknown, although autoimmune, genetic, hormonal and infectious factors are to blame. The disease is usually anogenital in childhood. Extragenital placement is very rare during this period. The abrasive appearance can be confused with child abuse, especially in genital LS. In this article we present a 4-year-old girl case with genital and extra genital LS lesions.

139-142

Authors: Padmapriya Balakrishnan , Thirunavukkarasu Arun Babu , Vijayan Sharmila

Cystic partially differentiated nephroblastoma (CPDN) is among the rare kidney tumors of childhood. Here, a case of CPDN in a baby who was operated with the diagnosis of multicystic kidney is reported. Clinical and imaging studies have a limited role in the differential diagnosis and diagnosis of this entity. A careful histopathological study is required to distinguish between CPDN and other types of multicystic kidneys. Because of the rare potential for aggressive behavior of this tumor, nephrectomy is the treatment of choice alone.

149-154

Authors: Erin Choi , Erin K. Barr , Viviane Mamlok , Cynthia Smith , Matthew Timberlake

Venous tumor thrombus is a known manifestation of Wilms’ tumors in a minority of cases but has not been previously described in association with cystic nephroma. We report an original case of a histologically-benign cystic nephroma presenting with venous tumor thrombus extending to the inferior vena cava. The tumor thrombus was not detected by preoperative Doppler ultrasound. The patient was successfully treated with radical nephrectomy, tumor thrombectomy, and IVC closure. Postoperatively, the patient underwent genetic testing which revealed a DICER1 mutation—known to predispose affected individuals to a variety of benign and malignant tumors—and requires intensive surveillance for associated conditions.

159-163

Authors: Syamantak Basu, Apoorva Makan, Akriti Tulsian, Suraj Gandhi, Neha Sisodiya Shenoy, Hemanshi Shah

Ureteral anomalies are difficult to diagnose on routine investigations in the background of a megaureter. Ureteric valves are a rare cause of congenital obstructive uropathy. Mid ureteric valves are rarer than proximal or distal ureteric valves, and most patients are misdiagnosed preoperatively. Therefore, intraoperative identification is critical. We present a case of mid ureteric valve which presented as unilateral hydroureteronephrosis.

116-120

Authors: Paarth Kapadia, Shreeya Popat, Angela G. Mittal

Hydrocolpos is a rare congenital genitourinary anomaly that can cause clinically significant urinary tract obstruction with oligohydramnios and hydronephrosis antenatally and renal failure postnatally. Here, we discuss the management of a neonate with hydrocolpos and renal failure. Vaginostomy tube and bladder catheterization have been described previously. However, in this population, external tubes can be difficult to manage and have a high propensity of failure. Therefore, the patient was managed via creation of a vaginostomy, which allowed for continuous passive drainage of fluid into a diaper and maintained a decompressed urinary tract until further reconstruction could be pursued.

126-130

Authors: William J. Scott, Aaron J. Shaw, Kenice D. Ferguson-Paul

Perinephric abscess is an uncommon infection in children, but it is usually caused by Escherichia coli, Staphylococcus aureus, Proteus mirabilis and Pseudomonas aeruginosa. Group B Streptococcus (GBS) is an uncommon cause of perinephric abscess or serious bacterial infections beyond the neonatal period. Comorbid conditions such as diabetes mellitus and immunosuppression can increase the risk of GBS invasive disease. We describe a 10-year-old female who presented with 1-month of right-sided flank pain and swelling with ultrasound showing large (>3 cm) right perinephric and subcutaneous abscesses. She was additionally diagnosed with new onset type 2 diabetes mellitus (DM) during admission. Abscess cultures obtained after placement of two percutaneous drains and the initiation of broad-spectrum intravenous antibiotics grew beta-hemolytic GBS. Here, we present to our knowledge, the first known documented case of GBS perinephric abscess in a school-aged child with DM. Much of the knowledge of perinephric abscess management is extrapolated from adults, therefore making optimal treatment in the pediatric population challenging. We propose that GBS be considered in the etiology of perinephric abscess in children with DM and other immunosuppressing conditions. In addition, percutaneous drainage of larger abscesses (>3 cm) in conjunction with antibiotic therapy is a reasonable management strategy.

136-139

Authors: Sevinc Akdeniz, Ulas Alabalik, Mehmet Hanifi Okur

We report the case of a 16-year-old female with a huge mass (40 × 30 × 40 cm) on the left ovary, causing mild bilateral hydronephrosis. Ultrasonography and computed tomography allowed us to diagnose this condition and showed an associated uretero-hydronephrosis. A unilateral salpingooophorectomy was performed on the left ovary, and final pathology revealed a mature cystic teratoma. The patient did not experience any problems in the follow-up six months later, and her hydronephrotic appearance disappeared.

88-93

Authors: Marianna Iaquinto, Roberto De Castro, Maria-Grazia Scarpa, Alessia Cerrina, Daniela Codrich, Edoardo Guida, Federica Pederiva, Maria Antonietta Lembo, Flora Maria Murru, Jürgen Schleef

Twin obstructive megaureters associated with duplex renal system is a rare malformation, particularly when a giant ureter dilation is present. We report an original case, initially misinterpreted, of a right incomplete duplex renal system with huge disproportion between the upper pole giant megaureter and the lower pole moderate megaureter, with severe obstruction at the uretero-ureteral junction and minor obstruction at the uretero-vesical junction. Uro-magnetic resonance imaging allowed conclusive diagnosis and the laparoscopic approach was effective for definitive, successful treatment.

98-101

Authors: Nirmalya Ray, Ujjwal Gorsi, Tejeshwar Singh Jugpal, Ravi Prakash Kanojia, Manavjit Singh Sandhu

Proximal migration of double J-stent (DJ stent) can be a reason for failure of its cystoscopic removal. Antegrade percutaneous nephrostomic removal of the stent can be done in such cases. Although the technique is described in adults and older children, the technique requires some modification when used in infants because of smaller calibre of pelvicalyceal system. Here we describe a case of a 6 month old male child who was treated by robotic assisted pyeloplasty for left sided ureteropelvic junction obstruction followed by placement of DJ stent. Cystoscopic removal of the stent 6 weeks after surgery was not possible due to proximal migration of the stent. Hence, antegrade percutaneous nephrostomic removal of the stent was performed using micro-snare. Certain novel technical modifications, like advancement of a diagnostic angiographic catheter in pelvicalyceal system, has been used in this case to reduce the risk of urothelial injury. Thus, antegrade removal of DJ stent with few technical modifications can be used safely in infants for removal of proximally migrated stents.

107-111

Authors: Han Kun Ng, Shankaran Thevarajah, Ann Feng Pan

Spindle cell rhabdomyosarcoma (RMS) is a rare variant of embryonal rhabdomyosarcoma. This tumor commonly involves paratesticular region of young males. Herein we report a case report of right paratesticular spindle cell rhabdomyosarcoma in a 16-year-old boy, who was referred to us for painless right scrotal mass. Staging computerized tomography (CT) showed no nodal or distant metastasis. The right radical inguinal orchidectomy was performed. Diagnosis of paratesticular spindle cell rhabdomyosarcoma was confirmed by histopathological and immunohistochemical examination. Paratesticular spindle cell RMS is a rare tumor and morphologically it may mimic other spindle cell neoplasms. Immunohistochemistry plays a pivotal role in its diagnosis. Making a correct diagnosis is crucial owing to the different treatment modalities available for this tumor