Pediatric Urology Case Reports

202-205

Authors: Zehra Pınar Koç, Pelin Özcan Kara, Gökhan Berktuğ Bahadır, Taylan Kara

We report a case of intrathoracic kidney that has additional congenital anomalies and an interesting follow up. A 30 month old female patient with history of spinal disraphism, anterior ectopic anus who were followed up for recurrent urinary tract infection by Tc-99m dimercaptosuccinic acid (DMSA) scintigraphy is presented. An interesting observation was noticed in her follow up that the left kidney displaced between two different time point static renal images into the left thorax. To our best knowledge, this is the reported first case of wondering intrathoracic kidney observed in Tc-99m DMSA follow up.

211-215

Authors: Lelah Grossman, Mark Horowitz, Jeremy Neuman, Richard Sidlow

Canal of Nuck cysts/hydroceles are rare occurrences in females of all age groups. We describe a case of a female neonate presenting with a labial mass which sonographically resembled a testicle. Upon surgical exploration, it was found to be a hydrocele of the canal of Nuck. We also review the literature on canal of Nuck cysts/hydroceles in the pediatric age group.

221-226

Authors: Jessica N. Jackson, Rebecca S. Zee, C.D. Anthony Herndon

Simple intratesticular cysts (SIC) are rare in infancy. There is a paucity of data with regards to management. Concern for malignancy often prompts the pediatric urologist to perform a radical orchiectomy which may impact endocrine function and fertility potential later in life. Recently, there has been a movement towards testis-sparing surgery for SIC. Herein, we describe a case of a 7-month-old infant who presented to pediatric urology clinic with left intraparenchymal SIC that was treated with a testis-sparing approach. In summary, this case report supports the use of testis-sparing surgery for management of preoperatively well-defined SIC.

232-237

Authors: Paddy Dewan, Padma Rao, John Lazarus

A 16 year old previously well boy presented with voiding difficulty that progressed to intermittent retention over a two month period. A renal ultrasound showed a structure consistent with a utricular remnant behind the base of the bladder that endoscopically appeared to terminate in the posterior wall of the prostatic urethra as a cystic, enlarged verumontanum. Following an uneventful endoscopic fulguration of the anterior wall, the boy remained catheterized overnight. The following day the catheter was removed at the time of a radiologic study of the lower urinary tract, which showed a dilated posterior urethra, but no reflux into the tubular retro-vesical structure that was noted to have emptied on a post-operative ultrasound.

147-152

Authors: Ilse Van Horebeek, Michel Wyndaele, Piet Verlinde, Dieter Ost, Abraham Cherian, Gunter De Win, Dominique Trouet

We present a ten-year-old boy with episodic left flank pain, vomiting and microscopic hematuria. Imaging showed hydronephrosis characteristic for ureteropelvic junction (UPJ) obstruction. Pyeloplasty revealed a ureteral polyp, histopathologic examination confirmed the diagnosis of a fibroepithelial polyp. Fibroepithelial polyps of the ureter are a rare cause of UPJ obstruction in children. The most common presentation is episodic flank pain and/or hematuria. Imaging can be guiding, but pre-operative diagnosis is difficult. After surgical resection recurrence is rare.

158-163

Authors: Letícia Alves Antunes, Carolina Talini, Bruna Cecilia Neves de Carvalho, Naiane Mayer, Sabrina Tristão Longo, Antônio Carlos Moreira Amarante

Genitourinary tuberculosis is rare in children. The aim of this study is to describe an unusual case of renal tuberculosis in a 16 years old girl, diagnosed with Hinman Syndrome and chronic renal failure. During a hematuria investigation the cystourethrogram revealed grade V right vesicoureteral reflux and small bladder. Ultrasonography demonstrated various cysts in left kidney with irregular content, suggesting dysplastic multicystic kidney. Scintigraphy showed functional exclusion of the left kidney. Total laparoscopic nephrectomy was proposed. Gregoir right ureteral reimplantation and Mitrofanoff mechanism using the distal left ureter were also indicated. During surgery presence of multiple cysts in the left kidney with caseous aspect, left ureter obstruction with caseous content in the lumen and bladder mucosa with friable and thickened wall were found. Histopathological study revealed chronic necrotizing granulomatous inflammation. Mantoux tuberculin skin test was strongly reactive, and family history was positive for pulmonary tuberculosis 10 years earlier. This paper shows the importance of bringing extrapulmonary tuberculosis to the roll of differential diagnosis of hematuria. Early treatment is important to avoid disease progression with functional loss of the organs as happened in this case.

170-175

Authors: Secil Conkar, Ipek Kaplan Bulut, Sevgi Mir

Renal abscess is uncommon in childhood. The common manifestations include fever, lumbar pain, abdominal pain and occasional flank mass. Renal ultrasonography enables us to achieve an early diagnosis, however; it may still be insufficient to distinguish it from pyelonephritis. Renal abscesses are usually associated with different predisposing conditions. In the present report, we aim to describe a case of a previously healthy child who developed a renal abscess. Keywords

181-187

Authors: Carolina Talini, Letícia Alves Antunes, Bruna Cecília Neves de Carvalho, Douglas Fagundes Teixeira, Michael Malca Sepúlveda, Murilo da Silva Padilha, Maria Helena Camargo Peralta del Valle

Xanthogranulomatous pyelonephritis (XGPN) is a rare and severe variation of chronic pyelonephritis characterized by suppurative destruction of renal parenchyma and its substitution with chronic inflammatory infiltrate. Male, 8 years old, admitted to the pediatric urology service at 7 years of age, with bladder and renal lithiasis. First symptoms started when he was 2 years old but presented no relapse until he turned 7 years old. During two years he had recurrence of renal lithiasis and underwent multiple surgical procedures. He remained asymptomatic for a short period of time and when presented fever and urinary symptoms again and underwent CT scanning that demonstrated left kidney enlargement associated with calculi and air bubbles in its interior, suggesting xanthogranulomatous pyelonephritis. Uretherostomy was performed and the patient had good clinical improvement. Renal exclusion was documented through scintigraphy and total left nephrectomy was performed, with a large amount of pus drained. Histopathology confirmed XGPN diagnosis. One year after surgery, there has been no recurrent renal lithiasis or urinary tract infection. Despite being a rare condition in children, XGPN has great importance as it can be often confused with pediatric renal tumours. Early diagnosis is important in order to promote better survival and clinical outcomes.

193-198

Authors: Prashant S Patil, Gupta Abhaya, Kothari Paras L, Kekre Geeta, Dikshit Vishesh K, Shahaji Deshmukh, Apoorva Kulkarni

Nutcracker syndrome is caused by a compression of the left renal vein between the aorta and the superior mesenteric artery. It results in left renal venous hypertension, and the subsequent development of venous varicosities of the renal pelvis, ureter, and gonadal vein. We report a rare case in an 8-year-old boy who presented with a history of intermittent episodes of hematuria leading to anemia and left flank pain. Ultrasound examination of abdomen revealed bulky left kidney and features of subacute medical renal disease. Computed tomography of abdomen showed compressed left renal vein between aorta and superior mesenteric artery. Authors report the successful operative management of this rare syndrome in a pediatric patient.

89-93

Authors: Patil Prashant S, Gupta Abhaya, Rahul Gupta, Kothari Paras L, Kekre Geeta, Vishesh Dikshit.

Fibroepithelial polyps of glans penis are rare in children. We present two male children with polyp over glans penis. The lesion was excised completely in both cases. Histopathological examination revealed fibroepithelial polyp in both patients. All of the reported cases published previously, except two are described in adults and all of them have been associated with the history of long-term condom catheter use. Only two cases have been reported in English literature in pediatric patients. We add two more cases to this rare entity in children.

99-104

Authors: Adem Emrah Coguplugil, Ali Guragac, Salih Hamcan, Emin Aydur

We present the first case of human chorionic gonadotropin (hCG) induced recurrent ischemic priapism in a 3 years-old boy in the English literature. The patient underwent second right orchidopexy and hCG treatment (1500 IU twice a week for three weeks) was initiated the day after the operation. Twenty-three days after the last injection, he presented to our clinic with the complaint of painless erections. After the diagnosis of ischemic priapism, conservative management and oral diazepam therapy was initiated and the result was successful. hCG therapy may rarely cause recurrent ischemic priapism in children.

110-115

Authors: Biserka Pigac, Silvija Mašić, Valentina Mašić

Dirofilariasis is a zoonosis caused by parasites the genus Dirofilaria, most common species are D. repens and D. immitis. This parasitosis rarely affects humans and it is usually asymptomatic. Canines are considered to be the reservoir of the parasite, yet the parasite is transmitted via mosquito bite. We present a case of subcutaneous scrotal dirofilariasis in a 3-year-old boy from north- western part of Croatia, where this zoonosis's not endemic, which characterizes this entity as rarity in this part of the country. In this case, parasitic infestation was manifested as painless scrotal nodule up to 1 cm in its largest diameter. Surgical excision revealed a thread- like material exiting the incision site. According to well –preserved morphologic characteristics, the specimen was characterized as a helminth of the genus Dirofilaria. We emphasize the importance of dirofilariasis in patients with subcutaneous nodules. And increase the awareness level of these parasites among clinicians and pathologists.

121-127

Authors: Letícia Alves Antunes, Carolina Talini, Bruna Cecilia Neves de Carvalho, Naiane Mayer, Sabrina Tristão Longo, Antônio Carlos Moreira Amarante

Ochoa syndrome, also known as urofacial syndrome, is a rare genetic disease (OMIM #236730) with autosomal recessive inheritance of mutations in the heparanase 2 (HPSE2) and the LRIG2 genes, characterized by functional obstructive uropathy and unusual facial abnormalities. Progression to renal failure if not early diagnosed is inevitable. The therapeutic goals are to restore bladder emptying, preventing damage to the urinary tract with the use of prophylactic antibiotics, clean intermittent catheterization, anticholinergic use, botulinum toxin injection, urinary diversion and bladder augmentation to slow the evolution of the disease. This article aims to report a patient with late diagnosis of Ochoa Syndrome, as well as describe its characteristics and clinical outcome.

134-138

Authors: Parkash Mandhan, Falah Ismail, Mansour Ali

Accessory urethra is a rare congenital anomaly and has varied presentations. Multiple surgical techniques have been described for its correction. We report a case of accessory urethra in a male infant, which was recognized incidentally. Diagnosis requires a high index of suspicion and optimal knowledge of the anomaly is mandatory for the surgical team to achieve a good outcome.

56-62

Authors: Amit Sharma, Radhyeshyam Chaudhari, Mukund Andankar

Anterior urethral valve (AUV) is a rare cause of obstructive uropathy in children. These are much rarer than posterior urethral valves. We report a case of a child with AUV and high grade bilateral vesicoureteric reflux. He was managed by transurethral laser ablation. The left sided reflux resolved after ablation. The right sided reflux persisted. The child is awaiting ureteric reimplantation.

68-75

Authors: Sreeranga YL, Anil Kumar N, Tyagi A, Vijaya Lakshmidevi B, Chowhan AK

Wilms’ tumor (nephroblastoma) is the most common renal tumor in children. It is uncommon in adolescent and skeletal metastasis at the time of presentation is even rare. The present case is a rare monophasic blastemal variant of Wilms’ tumor presenting with multiple skeletal metastases in a young female. We report a case of 23-year-old female presented with severe low backache and colicky right loin pain of 1 month and constant dull aching left upper arm pain of 20 days duration. Contrast-enhanced computed tomography (CECT) abdomen revealed heterogeneously enhancing soft tissue density mass with central nonenhancing areas arising from the upper pole of the right kidney. Whole body 18F-Sodium Fluoride (NaF) Positron emission tomography– computed tomography (PET/CT) bone scintigraphy revealed multiple skeletal metastases. The patient underwent right radical nephrectomy and the tumor was confirmed as adult Wilms’ tumor by histopathology and immunohistochemistry. Skeletal metastasis may be the first presentation in a case of adolescent Wilms’ tumor, and this possibility should be considered when an adolescent patient presents with flank pain and a renal mass. The clinical course of adult Wilms’ tumor with blastemal component is very aggressive with high chances of recurrence and mortality.

84-88

Authors: Clecio Piçarro, Thiago Luiz do Nascimento Lazaroni, Fabio Mendes Botelho Filho, Marcelo Eller Miranda, Atila Magalhães Victoria, Paulo Custódio Furtado Cruzeiro

Posterior Urethral Valve (PUV) is the most common cause of bladder outlet obstruction in boys, with an incidence of 1 in 5,000 to 8,000 male births. Congenital lobar emphysema (CLE) is an uncommon cause of congenital pulmonary airway malformation, with an incidence 1 per 20,000 to 30,000 deliveries. These congenital malformations have never been described together before. A boy newborn with prenatal suspicion of PUV, confirmed in first days of live presented respiratory distress that lead CLE diagnosis. Our multidisciplinary team submitted him to a twostep surgery correction successfully.

28-36

Authors: Julie R Ingelfinger, Kamyar Kalantar-Zadeh, Franz Schaefer

World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD) in childhood differs from that in adults, as the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and CKD in later childhood or in adult life. Children born early or who are small-for date newborns have relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely in order to help detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults, if they receive kidney replacement therapy including dialysis and transplantation, while only a minority of children may require this ultimate intervention Because there are disparities in access to care, effort is needed so that those children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic or economic circumstances. Our hope is that World Kidney Day will inform the general public, policy makers and caregivers about the needs and possibilities surrounding kidney disease in childhood.

42-45

Authors: Ryuta Watanabe, Takeshi Sugahara, Hitoshi Hamada

We report a case of the complete separation of the testis and epididymis in an undescended testis, which is a very rare phenomenon. It is a situation associated with an undescended testis and appears to be one of the cases of fusion abnormalities. In all reported cases, vas deferens andepididymis or the vas deferens alone were located more distally than the undescended testis. In our case, histological examination of the left vas-epididymis complex showed rete testis-like tubular tissue which has a risk for malignant transformation. Long-term observation is essential.

52-55

Authors: Alev Suzen, Nazile Erturk, Ozgur Ilhan Celik

Fibroepithelial polyp of vagina (FEPV) is a mucosal polypoid lesion with a fibro vascular stalk and oedematous or hypocellular stroma covered by squamous epithelial surface. FEPV is extremely rare before menarche and after menopause. This article describes a two year old girl with a vaginal polyp and the histological analysis of which revealed to be a FEPV. Surgical excision of the mass was performed, and in 1 year of follow-up no recurrence was detected. A review of the literature showed that there were four cases of FEPV in neonatal girls. While these reported cases were neonatal, our case is the first case of FEPV in a two year old girl.