Pediatric Urology Case Reports

238-242

Authors: Jyoti Bothra, Hemanshi Shah, Charu Tiwari

Tumor markers have become important in the armamentarium of diagnostic and prognostic tools in the field of oncology. With advances in the scientific research they have created a place in the management protocols of various tumors. Alpha-fetoprotein (AFP) is one such tumor marker which is known to be elevated in liver tumors and germ cell tumors, especially those with the yolk sac elements. However, there are only a few reports describing elevated AFP levels in Wilms’ tumor, the second most common primary intra-abdominal malignant tumor in children. We present a case of classic Wilms’ tumor with raised AFP levels which followed the trend of treatment.

248-252

Authors: Govindarajan Krishna Kumar

Labial adhesion is a common gynecologic problem in childhood. It is probably associated with hypoestrogenic state of the female infant. Labial adhesions are usually diagnosed during regular checkups or when the fusion results in post-void dripping, vaginal irritation, dysuria, urinary tract infection or obstruction. Hence, we recommend physicians to perform genital examination in girls who show symptoms of urinary tract infection. Treatment options include topical estrogen or betmethasone creams, manual separation or adhesiolysis. Follow up of these children is important to ensure complete resolution without residual adhesion or recurrence.

256-261

Authors: Farshideh Alishahi, Reza Farjad, Farideh Dehghan Manshadi

The present study, conducted on three children with cerebral palsy who had voiding dysfunction. After collecting demographic information, symptoms of voiding dysfunctions were recorded. Then; we did an ultrasonographic evaluation of the bladder to measure residual urine volume. Physiotherapy treatment through percutaneous electrical stimulation of the posterior tibial nerve was performed in 12 sessions. Bladder ultrasonography was repeated after treatment. Abnormal residual urine volume became normal in patients with urinary retention. Percutaneous posterior tibial nerve stimulation reduces symptoms of voiding dysfunctions in children with cerebral palsy.

267-274

Authors: Elisa Cerchia, Francesco Molinaro, Rossella Angotti, Marina Sica, Adrian Bianchi, Mario Messina

We report our series of selected patients with complete incontinence in whom the appendix was divided and utilized for creating two continent catheterizable stomas. All patients were treated for urinary and fecal incontinence by split appendix technique. The appendix was divided into two different parts preserving adequate perfusion and used for creating an appendicocecostomy (ACE) and an appendicovesicostomy at the same time. After a clinical and radiological follow up, our patients referred a good acceptance and an easily management of the stomas in order to stay dry for all day from urine and feces with improving of their quality of life. The combination of ACE and Mitrofanoff principle have revolutionized the management of urinary and fecal incontinence in patients who are unable to utilize their urethra to keep themselves dry.

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.