Report of seven children with Y-type urethral duplication (YUD).
Materials and Methods:
(A) Four staged operations were performed in patients having extensive perineal dissection (for rectourinary fistula separation and anterior mobilization of ventral urethra (VU)), tension rectocutaneous anastomosis, and children who were not toilet trained). These stages are (1) diversion sigmoid colostomy with anterior mobilization of VU as perineal urethrostomy via anterior sagittal approach; (2) Orthotopic urethral (OU) reconstruction; (3) anastomosis of OU and perineal urethra; (4) colostomy closure with management of complications. (B) The patients having VU onto the perineum underwent single stage urethral reconstruction.
The VU was urethrorectal/urethroanal in five and urethroperineal in two. Low anorectal malformation and upper urinary tract anomalies were present in 57.1% (4/7) and 14.7% (1/7) patients, respectively. Buccal mucosa free graft, transverse inner preputial flap, and perineal skin were tubularized for OU reconstruction. Mean age at 1st , 2nd , 3rd , and 4th surgery was 5 ± 0.78, 28 ± 0.78, 36 ± 0.78, 49 ± 0.78 months respectively. Three patients needed surgery for complications (urethrocutaneous fistula in two and urethral diverticulum in one) in a mean 3.12 ± 0.34 years of follow-up. Final uroflowmetry and fecal continence were good in all patients.
The YUD is a difficult entity to manage. Although, staged procedure appears to be time consuming, but good and promising results can be achieved by staging the procedure.
Anourinary fistula; perineal urethrostomy; rectourinary fistula; urethral duplication; Y-type urethral duplication
To evaluate the outcome of definitive repair of anogenital injuries (AGI) in child victims of acute sexual assault.
Settings and Design:
It is a prospective study of emergency care provided to child victims of acute sexual assault at a tertiary care Pediatric Surgical Unit in Maharashtra, India.
Material and Methods:
Out of 25 children, who presented during January 2009-December 2010 with suspected sexual assault, five children (one male and four female, between 4-9 years of age), had incurred major AGI. These children underwent definitive repair and a diverting colostomy. Perineal pull-through was performed in the male child with major avulsion of rectum. One 4-year-old girl with intraperitoneal vaginal injury required exploratory laparotomy in addition.
The postoperative period and follow-up was uneventful in all our patients. Four out of five patients have excellent cosmetic and functional outcome with a follow-up of 2-4 years. Our continence results are 100%.
Children with acute sexual assault need emergency care. To optimally restore the distorted anatomy, all major AGI in such children should be primarily repaired by an expert, conversant with a child’s local genital and perineal anatomy. Along with provision of comprehensive and compassionate medical care, prevention of secondary injuries should be the ultimate goal.
Anogenital injuries; acute sexual assault; child abuse; emergency management; definitive surgical repair
Congenital anterior urethral diverticulum is an infrequent but important cause of infravesical obstructive uropathy in children. Clinical spectrum usually includes obstructive or irritative urinary symptoms or penile ballooning during the act of micturition. We share our experience in a case of giant anterior urethral diverticulum with a contained calculus presenting as a huge inguino-scrotal swelling and masquerading as left inguinal hernia. The fluctuation in the size of the swelling related to the act of micturition was mistaken for cough impulse. He was subjected to a left inguinal herniotomy, following which he developed urine leak from the surgery wound and was subsequently referred to our centre for further management. The importance of a detailed history, meticulous physical examination, and diagnostic imaging has been stressed. The surgical approach in such cases has also been highlighted.
Anterior urethral diverticulum; calculus; inguino-scrotal swelling; infra-vesical obstructive uropathy; left inguinal hernia
A renal tumor in a 14-month- old child, who was initially diagnosed as mesoblastic nephroma, but on review post surgery was diagnosed as hyper-differentiated metanephric stromal tumor, with its excellent prognostic outcome. An attempt is made to document imaging features that may enable one to suspect this rare condition. The literature is reviewed with emphasis on its distinction from its look-alikes in the pediatric age group.
Atypical Wilms’ tumor; congenital mesoblastic nephroma; metanephric stromal tumor; pediatric renal tumor
Neonatal intestinal obstruction is the most common surgical emergency in a newborn. Although, large numbers of newborns are operated in our country, limited published literature is available on advances in diagnosis, and management of this problem with outcome analysis in newborns. We report a premature (32 weeks) newborn who developed acute onset symptoms of small bowel obstruction in 3rdweek of life, and discuss the approach to diagnosis and management with the minimal access surgery and successful outcome.
Meckel’s band obstruction; minimal access surgery; neonatal intestinal obstruction
Invasive fungal infection is common in the present day NICUs - generally manifesting as candiduria or candida sepsis. Fungal balls in the kidneys are very uncommon and most are amenable to higher antifungal agents. However, we had a child who did not respond to such measures and ultimately needed a surgical removal of the fungal ball in his kidney.
We report this case along with a review of literature to highlight about this uncommon, but an important cause of persistent sepsis in pre-term infants and to review the treatment options including a surgical removal.
Invasive candidiasis; pre-term infants; renal fungal balls
Aortoesophageal fistulae (AEF) are rare and are associated with very high mortality. Foreign body ingestions remain the commonest cause of AEF seen in children. However in a clinical setting of tuberculosis and massive upper GI bleed, an AEF secondary to tuberculosis should be kept in mind. An early strong clinical suspicion with good quality imaging and endoscopic evaluation and timely aggressive surgical intervention helps offer the best possible management for this life threatening disorder. Our case is a 10-year-old boy who presented to the pediatric emergency with massive bouts of haemetemesis and was investigated and managed by multidisciplinary team effort in the emergency setting.
Aorto-esophageal fistula; children; tuberculosis
To report the results of an early series of patients who underwent modified Koyanagi repair for severe hypospadias.
Materials and Methods:
A total of 24 boys (age: 9 months to 11 years) with proximal hypospadias, chordee, and poor urethral plate underwent modified Koyanagi repair between September 2008 and January 2012. Nine boys had associated penoscrotal transposition that was corrected simultaneously. Vascularized parameatal based foreskin flap was used to correct the hypospadias in a single stage. The follow-up ranged from 6 months to 3.5 years.
A total of 13 of the 24 children had a good outcome and were voiding normally, while 11 boys developed complications, 3 of which were major and 8 minor. The major complications were complete breakdown (n = 1), meatal and distal neourethral stenosis requiring laying open of distal urethra (n = 1), and glans breakdown (n = 1). The minor complications included fistulae (n = 5), meatal stenosis amenable to dilatation (n = 1), and lateral chordee (n = 1). Majority of the complications were in the initial patients, with successful outcomes in the last 1 year. Most of these complications were successfully managed by minor second procedures.
Modified Koyanagi repair not only corrects severe hypospadias with chordee but also corrects the associated penoscrotal transposition in a single stage. The results are good once the learning curve is crossed.
Chordee; modified Koyanagi; proximal hypospadias; parameatal foreskin flap
To describe a technique of phalloplasty that is devoid of donor site scarring and suitable for urethral inlay and penile prosthesis in subsequent stages in cases of aphallia.
Materials and Methods:
Four patients with various disorders of sex development with 46 XY and severe penile deficiency, including one with complete androgen insensitivity syndrome who was initially raised as female, have been operated using a “Bird Wing” lower abdominal skin crease incision.
The patients’ age ranged from 6 to 17 years with preoperative stretched penile lengths between 1 and 2.5 cm. Phallic sizes between 7.5 and 12.5 cm was achieved leaving the donor site unremarkable with lower abdominal skin crease linear scar and excellent postoperative recovery.
This phalloplasty technique can be utilized as a definitive procedure in many situations of penile insufficiency. Subsequent stages of urethral repair and insertion of penile prosthesis can be easily added.
Androgen insensitivity syndrome; Aphallia; bladder exstrophy; disorders of sex development; gender dysphoria; phalloplasty
To evaluate and compare the extent of surgical stress following laparoscopic nephrectomy (LN) and open nephrectomy (ON) in children.
Materials and Methods:
Twenty consecutive children undergoing nephrectomy were randomized to LN or ON groups. Acid-base balance, blood glucose, acute phase proteins (C-reactive protein [CRP]) and inflammatory markers (interleukin-6 [IL-6]) were measured pre-operatively, as well as 4 and 24 h after surgery. The differences between the two groups were analyzed statistically (significance value for P < 0.05).
The overall acid base status was more stable in LN. The fall in pH 4 h after surgery was more in ON (P = 0.440) and the difference in pH in ON 4 h and 24 h post-operatively was statistically significant (P = 0.002). In LN, significant difference was found in the base excess mean pre-surgery (mean –3.280 mEq/L) and 4 h post-surgery (mean –7.480 mEq/L) (P = <0.05), as well as between 4 h and 24 h after surgery (mean –2.660 mEq/L) (P = 0.011). The acute rise in CRP 24 h post-operatively in the ON (88.972 mg/L) was significantly higher when compared to both the pre-operative and 4 h post-operative values (P < 0.05). This rise was however, not statistically significant when compared to the 24 h post-operative value in LN (46.399 mg/L) (P = 0.062). The rise in IL-6, 24 h post-procedure in LN (mean 44.444 pg/ml) was statistically lower than that in the open group (mean 343.333 pg/ml) (P = 0.041).
The stable acid-base status and lesser rise of CRP and IL-6 in LN lead to the conclusion that surgical stress caused by LN is less than ON.
Children; laparoscopic nephrectomy; open nephrectomy; stress response
To study, the effects of ipsilateral ureteric obstruction on contralateral kidney and the role of renin angiotensin system (RAS) blockade on renal recovery in experimentally induced unilateral ureteric obstruction.
Materials and Methods:
Unilateral upper ureteric obstruction was created in 96 adult Wistar rats that were reversed after pre-determined intervals. Losartan and Enalapril were given to different subgroups of rats following relief of obstruction.
The severity of dilatation on the contralateral kidney varied with duration of ipsilateral obstruction longer the duration more severe the dilatation. There is direct correlation between renal parenchymal damage, pelvi-ureteric junction (PUJ) fibrosis, inflammation and severity of pelvi-calyceal system dilatation of contralateral kidney with duration of ipsilateral PUJ obstruction.
Considerable injury is also inflicted to the contralateral normal kidney while ipsilateral kidney remains obstructed. Use of RAS blocking drugs has been found to significantly improve renal recovery on the contralateral kidney. It can, thus, be postulated that contralateral renal parenchymal injury was mediated through activation of RAS.
Renin angiotensin system; ureteric obstruction; ureteropelvic junction obstruction
To share our experience of doing tubularized incised plate urethroplasty with modifications.
Materials and Methods:
This is a single surgeon personal series from 2004 to 2009. One hundred patients of distal hypospadias were subjected for Snodgrass urethroplasty with preputioplasty. The age range was 1 to 5 year with mean age of 2.7 years. Selection criteria were good urethral plate, without chordee and torsion needing complete degloving. Main technical modification from original Snodgrass procedure was spongioplasty, preputioplasty, and dorsal slit when inability to retract prepuce during surgery.
Average follow-up period is 23 months. Seven (7%) patients developed fistula and one patient had complete preputial dehiscence. Phimosis developed in three (3%) patients and required circumcision. Dorsal slit was required in seven patients. One patient developed meatal stenosis in postoperative period. All other patients are passing single urinary stream and have cosmesis that is acceptable.
Modified tubularized incised plate urethroplasty with preputioplasty effectively gives cosmetically normal looking penis with low complications.
Preputioplasty; Snodgrass; spongioplasty; urethroplasty
To evaluate the efficacy of calretinin immunostaining in diagnosing Hirschsprung's disease (HD).
Materials and Methods:
Sixty cases were studied over a period of 1 year (July 2010-June 2011). There were 36 full-thickness biopsies and 24 resected specimens. Calretinin processing was done on the paraffin-embedded blocks after routine histopathological examination.
Of the 36 biopsy specimens, in 19 cases HD was diagnosed by hematoxylin and eosin (H and E) staining earlier. In 2 patients, ganglion cells were seen and HD was ruled out. In 15 cases, there was a diagnostic dilemma and calretinin was used. Ganglion cells were found in 3 specimens and nerve fibers in 5. In all 24 resected specimens, calretinin correlated with the findings on H and E staining.
Calretinin was extremely useful in solving the suspicious and indeterminate cases of HD. It can serve as a valuable cost-effective diagnostic aid in the centers where acetylcholinesterase enzyme histochemistry is not available.
Hirschsprung's disease; calretinin; immunohistochemistry
To evaluate the outcome of innervation preserving sphincteroplasty along with anatomical bladder neck reconstruction (IPS-ABNR) compared to classic Young-Dees-Leadbetter (YDL) bladder neck reconstruction in exstrophy with insufficient bladder capacity requiring detubularized-ileocystoplasty.
Materials and Methods:
Sixteen male patients of exstrophy bladder who required ileocystoplasty from 2004 to 2010 were randomized into group A (n = 7) and group B (n = 9). After detubularized-ileocystoplasty with Mitrofanoff stoma and ureteric reimplantation in all, group A received YDL bladder neck repair while group B received IPS-ABNR repair through a midline scrotoperineal approach. Outcome measurement included operative and postoperative problems, continence, and upper tract status.
In group A, two had incompetent bladder neck with gross incontinence, while four had a dry interval of more than 3 h without the ability of voiding per urethra. In group B, seven patients had dry interval of more than 3 h with an ability of urethral voiding and midstream holding in five.
Exstrophy patients requiring augmentation cystoplasty and repaired with IPS-ABNR can achieve dynamic bladder outlet resistance with adequate leak point pressure and ability to void voluntarily with midstream holding capability. The children had the satisfaction of voiding per urethra with ability to stop in midstream similar to that in normal children.
Anatomical bladder neck reconstruction; augmentation cystoplasty; exstrophy bladder; ileocystoplasty; innervation preserving sphincteroplasty; Young-Dees- Leadbetter bladder neck reconstruction
To study renin angiotensin system (RAS) activity after posterior urethral valve ablation and the role of early induction of angiotensin converting enzyme-inhibitors (ACE-I) on the outcome of renal function.
Materials and Methods:
Thirty four children underwent valve ablation in which therapy with ACE-I was started 40.5 ± 4.1 (range 32-47 months) formed the study group. Post-ACE-I data were collected after mean duration of 18.2 ± 4.0 (12-28 months). Plasma renin activity (PRA), urinary micro albumin, glomerular filtration rate (GFR), and serum creatinine, before and after therapy were monitored.
Therapy with ACE-I resulted in a fall in micro albuminuria by 45.7% and 42.0% in patients without and with vesico ureteral reflux, respectively, and improvement in split renal function by 6.6% and 5.9% GFR respectively. A similar response was noted in patients without and with renal scars.
The decline in renal function after valve ablation is accompanied by activation of RAS reflected in a gradual rise in PRA. Therapy with ACE-I stabilizes and then improves renal function, thereby, retarding the pace of renal damage.
Angiotensin converting enzyme-inhibitors; glomerular filtration rate; microalbuminuria; plasma renin activity; posterior urethral valves; renal scars
We report a rare case of prune belly syndrome associated with congenital pouch colon, which was managed successfully.
Congenital pouch colon; hydronephrosis; prune belly syndrome
This report describes a girl with congenital pouch colon (CPC), uterus didelphys with septate vagina, and a cloacal anomaly. The girl underwent cloacal reconstruction at the age of 15 months. Subsequently, at puberty, the child had primary amenorrhea with severe cyclic abdominal pain due to endometriosis of both the uteruses and adnexal cysts with hematometra and hematosalpinx. Laparotomy with removal of both uteri and the left fallopian tube was performed. Both uteri had atresia of the cervix uteri. This report emphasizes the need for comprehensive evaluation and a long-term management strategy for associated gynecologic anomalies in girls with CPC, especially with regard to patency of the outflow tract.
Anorectal malformation; cervical atresia; cloaca; congenital pouch colon; septate vagina; uterus didelphys
Extrahepatic portal hypertension is not an uncommon disease in childhood, but isolated inferior mesenteric portal varices and lower gastrointestinal (GI) bleed have not been reported till date. A 4-year-old girl presented with lower GI bleed. Surgical exploration revealed extrahepatic portal vein obstruction with giant inferior mesenteric vein and colonic varices. Inferior mesenteric vein was joining the superior mesenteric vein. The child was treated successfully with inferior mesenteric – inferior vena caval anastomosis. The child was relieved of GI bleed during the follow-up.
Extra hepatic portal hypertension; giant inferior mesenteric vein; inferior mesenteric vena caval shunt
Paratesticular germ cell tumors are extremely rare. A 12-month-old boy with yolk sac tumor involving only the paratesticular tissue is reported. Pre-operatively raised alpha fetoprotein levels fell to normal levels after high inguinal orchiectomy. This appears to be the youngest and only the 3rd case reported in the English literature.
Alpha fetoprotein; paratesticular tumor; primary extragonadal germ cell tumor; yolk sac tumor
A case of unilobar Caroli's disease in an 8-year-old girl treated with left hepatectomy is reported here.
Caroli's disease; cholangitis; cholelithiasis; hepatectomy