Modified Pediatric Penile Perception Scale to Evaluate Cosmetic Outcome in Children With Hypospadias Repair.

OBJECTIVES: To compare the interpretation for cosmesis post-hypospadias repair by child, parents and surgeons using modified Pediatric Penile Perception Scale (PPPS). METHODS: This cross-sectional study involving 50 children (aged 2 to 17 years) with hypospadias was conducted at the pediatric surgery department of our public sector tertiary care hospital. Subjects were assessed 6 months after completion of all stages of hypospadias repair. Cosmetic assessment was done using modified PPPS. We clubbed together the variables 'meatus' and 'glans' as MG (meatus-glans) complex due to their extreme proximity (embedding), while cosmesis of phallus was considered independently. The modified PPPS scoring parameters included phallus, MG complex, shaft skin, and general appearance. Independent assessment by surgeon, patients and parents was compared, and analyzed using SAS 9.2 statistical software. Cosmetic results of single vs staged repair, and different repair types was compared. RESULTS: Assessment using modified PPPS showed that MG complex cosmesis and skin scarring were the most heeded parameters by all three categories of observers. PPPS by surgeons remained least affected by phallic cosmesis and that of the patient by the overall phallic appearance. Tubularized incised plate urethroplasty (TIPU) scored better on cosmesis. CONCLUSION: Phallic cosmesis should be considered an independent variable for assessing cosmetic outcome of hypospadias, apart from MG cosmesis.

Simultaneous Endoscopic Management of Urethral Duplication and Postposterior Sagittal Anorectoplasty Urethral Diverticulum in a Boy with Anorectal Malformation.

We report a case of urethral duplication with high anorectal malformation (ARM). A 12-year-old boy who had undergone transverse colostomy on day 2 of life and posterior sagittal anorectoplasty (PSARP) for ARM (rectobulbar fistula) at 8 years and also had urinary dribbling from a midscrotal opening in addition to normal voiding, presented with recurrent urinary tract infections and dysuria. Midscrotal dribbling had continued postoperatively. Colostomy had been closed 3 months after PSARP. Investigations revealed a large urethral diverticulum at the site of rectourethral fistula due to a segment of the gut being left behind during fistula closure. In addition, he had a sagittal urethral duplication originating at bulbar urethra and opening externally at midscrotum. The highlight of this report is successful endoscopic management of both the urethral duplication and the diverticulum.

Congenital Central Overinflation with Mainstem Bronchial Stenosis: Pre- and Postnatal Imaging and Outcome-Case Report and Brief Review of Literature.

Congenital overinflation of lung is underdiagnosed prenatally as the imaging features of the same are not well described. We describe a very rare case of central variety of congenital overinflation, secondary to right mainstem bronchial stenosis, not previously described in our knowledge, which presented as an enlarged homogenous echogenic/hyperintense lung with cardio-mediastinal shift and was misdiagnosed as congenital pulmonary airway malformation (CPAM). We reviewed imaging features helpful in prenatal diagnosis of this condition on ultrasound and magnetic resonance imaging and discussed an approach for differential diagnosis. The congenital central overinflation may be considered in prenatal detected echogenic lung lesions with the absence of cysts or systemic arterial supply and presence of hypervascularity or dilated bronchi within the lesion.

Biliary Pseudocyst: A Rare Complication of Antenatal Perforation of Forme Fruste Choledochal Cyst.

Spontaneous perforation of choledochal cyst (CDC) is rare with an incidence of 1.8%-7%. Perforation with pseudocyst formation is even rarer, there being only two case reports. We report the first case of antenatal perforation of CDC with biliary pseudocyst formation in a newborn and its management.

Esophageal mucormycosis in an immunocompetent child: A rare presentation.

Invasive mucormycosis of the esophagus (rare) and gastrointestinal tract is emerging as an important cause of concern in children. It usually affects immunosuppressed individuals. However, infection of immunocompetent children is also seen. The diagnosis of mucormycosis is difficult both at the clinical and the laboratory level, hence leading to unsatisfactory treatment and high mortality rates. The infection is usually life threatening so an early diagnosis and prompt administration of antifungal therapy is imperative.

Management of iatrogenic RV injury - RV packing and CPB through PTFE graft attached to femoral artery.

Cardiac injuries during repeat sternotomy are rare. While undergoing debridement for chronic osteomyelitis (post arterial septal defect closure), a 4-year-old girl sustained significant right ventricular (RV) injury. Bleeding from the RV was controlled by packing the injury site, which helped in maintaining stable hemodynamics till arrangements were made for instituting cardiopulmonary bypass (CPB). Since the femoral artery was very small and unsuitable for direct cannulation, a polytetrafluoroethylene (PTFE) graft sutured end-to-side to the femoral artery was used for establishing CPB. The injury was successfully repaired.

Complications of long-standing foreign body in the airway and their outcomes after endoscopic management: an experience of 20 cases.

AIM: To study the outcomes after endoscopic treatment of chronic foreign bodies (FBs) in the airway. MATERIALS AND METHODS: A retrospective study (2008-2013) of 20 cases with chronic airway FBs (>2 weeks) was done with emphasis on endoscopic management. All cases were initially evaluated by the pediatric pulmonologist. Flexible and rigid bronchoscopy was done for diagnosis and retrieval, respectively. The techniques of FB retrieval, problems encountered, and their solutions were analyzed. Follow-up flexible bronchoscopy was done in symptomatic cases. Outcomes were assessed in terms of successful removal of the FB, clinical recovery, lung expansion, and need for further procedures. RESULTS: Twenty cases (16 boys, 4 girls) with a mean age of 7 years had a chronic airway FB diagnosed on chest X-ray (n=6) and flexible bronchoscopy (n=14). Six cases had computed tomography evaluation. On rigid bronchoscopy, the FB was successfully retrieved in 16 cases. Two cases required open surgery for FB-induced tracheoesophageal fistula. One case required pneumonectomy because of a battery eroding into the lung parenchyma. One patient died. Of the 16 who had successful retrieval, 11 recovered with full lung expansion. Four recovered after additional bronchoscopic procedures (cauterization of granulation [n=2] and balloon dilatation of bronchial stenoses [n=2]). One case required pneumonectomy for persistent collapse despite multiple dilatations. CONCLUSIONS: An airway FB producing chronic respiratory symptoms may be missed because of lack of definite history of an inhaled FB. Clinical suspicion and flexible bronchoscopy are instrumental in diagnosis. Treatment is challenging because of chronicity-related complications and requires innovative ideas to make best use of the available urologic and bronchoscopic equipment. Addition of tracheotomy provides safety in difficult cases. Bronchoscopic removal leads to clinical and radiological recovery in most cases.

Laparoscopic-assisted transanal pull-through for Hirschsprung's disease: Comparison between partial and near total laparoscopic mobilization of rectum.

BACKGROUND: Transanal pull-through with laparoscopic assistance is gaining popularity. How much rectal dissection to do laparoscopically and how much transanally is not clear. Laparoscopic rectal mobilization is akin to open pelvic dissection of Swenson's operation - the most physiological procedure. Through this comparative study, we aim to evolve a technique that maximizes the benefits of Swenson's technique and minimizes the problems of a transanal procedure. MATERIALS AND METHODS: Twenty patients (19 boys and one girl, newborn to 6 years) with Hirschsprung's disease (HD) were randomized for laparoscopic-assisted transanal pull-through (LATAPT) either by near complete (Group A) or partial (Group B) laparoscopic mobilization of rectum. Patients were followed up for at least 3 months. Demographic profile; operative details (time taken, blood loss, operative difficulty, and complications); postoperative course (duration of urinary catheter, oral feeding, and hospital stay); and follow-up stooling pattern, consistency, and continence were compared in the two groups. RESULTS: The time taken for laparoscopic mobilization was marginally higher in group A, but the time taken for transanal dissection in this group was significantly less than in group B. All other comparisons showed no significant difference in the two groups. Stool frequency and continence improved with time in both groups. CONCLUSION: Extent of laparoscopic mobilization of rectum does not appear to be a factor deciding the outcomes. No recommendations could be made in view of the small number of cases. However, it shows that laparoscopic assistance can be used to maximize the benefits of Swenson type of operation and a transanal pull-through.

Laparoscopic repair of hernia in children: Comparison between ligation and nonligation of sac.

AIM: The essence of the current techniques of laparoscopic hernia repair in children is suture ligation of the neck of the hernia sac at the deep ring with or without its transection. Some studies show that during open hernia repair, after transection at the neck it can be left unsutured without any consequence. This study was aimed to see if the same holds true for laparoscopic hernia repair. MATERIALS AND METHODS: Sixty patients (52 boys and eight girls, 12-144 months) with indirect inguinal hernia were randomized for laparoscopic repair either by transection of the sac alone (Group I) or transection plus suture ligation of sac at the neck (Group II). Outcome was assessed in terms of time taken for surgery, recurrence, and other complications. RESULT: Thirty-eight hernia units in 28 patients were repaired by transection alone (Group I) and 34 hernia units in 29 patients were repaired by transection and suture ligation (Group II). Three patients were found to have no hernia on laparoscopy. Recurrence rate and other complications were not significantly different in the two groups. All recurrences occurred in hernias with ring size more than 10 mm. CONCLUSION: Laparoscopic repair of hernia by circumferential incision of the peritoneum at the deep ring is as effective as incision plus ligation of the sac.

Extraperitoneal Pelvic laparoscopic disconnection of accessory urethra from normal urethra in a case of urethral duplication.

We report an extraperitoneal pelvic laparoscopic approach to disconnect accessory urethra from normal urethra in complete urethral duplication. First stage consisted of chordee correction, partial excision of the accessory urethra and glansplasty. In the second stage the remaining accessory urethra was disconnected from the normal urethra through a pre-peritoneal minimal access approach to the retropubic space. The remaining distal mucosa was ablated using monopolar cautery.

Endodermal sinus tumor of vagina posing a diagnostic challenge and managed by chemotherapy and novel posterior sagittal surgical approach: lessons learned.

Vaginal germ cell tumor (GCT) is a rare gynecological malignancy with no more than 100 reported cases in the international medical literature. It is an unusual, but an important, cause of premenarchal vaginal bleeding in a child. This article describes a 2-year-old child with vaginal GCT, initially misdiagnosed as rhabdomyosarcoma (on imprint smear cytology) and then as clear cell adenocarcinoma. The authors highlight the salient differentiating clinical, radiological and histological features to prevent misdiagnosis in future. The report emphasizes the need for increased awareness and screening for vaginal GCT by estimation of serum α-fetoprotein levels, in all patients with premenarchal vaginal bleeds, to prevent inadvertent operative interventions.

Single stage repair of tetralogy of fallot associated with left pulmonary artery sling and tracheal stenosis.

We report a rare case of tetralogy of Fallot (TOF) with left pulmonary artery (LPA) sling with tracheal stenosis. The patient underwent successful surgery in one stage involving intracardiac repair of TOF, LPA reimplantation and resection of tracheal stenosis with end-to-end anastomosis.

Empyema thoracis in children: a short term outcome study.

This study prospectively evaluates clinical course of pyogenic empyema thoracis in 25 children (2 mo to 12 y) treated with injectable antibiotics and chest tube drainage, and followed for 6 weeks. The median (range) age at presentation was 3 y (4 mo to 11 y). The pleural fluid culture was positive in 24% of patients. Staphylococcus aureus was the most commonly isolated organism. The median (range) duration of injectable antibiotics was 14(14-52) d; median duration of total antibiotics (injectable and oral) was 4 weeks. The median (range) duration of chest tube insertion and hospital stay was 8(5-45) and 14(14-56) days, respectively. All patients were discharged without any surgical intervention besides chest tube drainage. At discharge, pleural thickening was present in 84% and crowding of ribs was seen in 60% of the subjects on radiological examination. All these patients were asymptomatic at discharge. Chest deformity was present in 20% of the patients at 6-weeks follow up. Antibiotics and chest tube drainage is an effective method of treating pyogenic empyema thoracis in children in resource-poor settings.

Soft tissue covers in hypospadias surgery: Is tunica vaginalis better than dartos flap?

AIM: To compare tunica vaginalis with dartos flap as soft tissue cover in primary hypospadias repair. MATERIALS AND METHODS: 25 cases (age range: 12-132 months; all fresh cases) of primary hypospadias were prospectively repaired by tubularized incised plate (TIP)/TIP + graft urethroplasty using tunica vaginalis flap (TVF) as soft tissue cover to urethroplasty (group A). Their results were compared with another set (group B) of age- and anatomy-matched controls (25 patients operated during the previous 3 years) who had undergone TIP repair using dartos flap as soft tissue cover. Statistical analysis of results was done with Fischer's exact test. GROUP A: No fistula, skin necrosis, meatal stenosis, urethral stricture. One case had partial wound dehiscence that resolved on conservative treatment with no sequelae. One case required catheter removal on 3(rd) day because of severe bladder spasm. There was no testicular atrophy/ascent. Group B: 3 fistulae - all required surgery. There were three cases of superficial skin necrosis that healed spontaneously without sequel. There was no meatal stenosis/urethral stricture. The difference in fistula rate between both the groups, however, was not statistically significant (P = 0.4). CONCLUSION: TVF may have an edge over dartos fascia for soft tissue coverage of the neourethra.

Use of pre and intra-operative bronchoscopy in management of bronchial injury following blunt chest trauma.

Blunt chest trauma resulting in right bronchial tear in an 8-year-old girl is reported. Use of bronchoscopy in the management of such an injury is highlighted.

Cogenital intrapericardial herniation of liver.

Intrapericardial herniation of liver is a rare form of diaphragmatic hernia. We report a 30-hour old baby with right congenital anterior diaphragmatic hernia masquerading as congenital pneumonia with cardiomegaly. It is prudent to consider congenial anterior diaphragmatic hernia in any newborn with unexplained respiratory distress, cardiomegaly and pericardial effusion.

Traumatic strictures of the posterior urethra in boys with special reference to recurrent strictures.

PURPOSE: We report 18 years' experience of traumatic urethral strictures in boys with emphasis on recurrent strictures. MATERIALS AND METHODS: Thirty-four boys with pelvic fracture urethral strictures underwent 35 repairs: 23 in the primary group (initial suprapubic cystostomy, but no urethral repair) and 12 in the re-do group (previously failed attempt(s) at urethroplasty elsewhere). The median age at operation and stricture length was 8.4 years and 3 cm in the primary and 9 years and 5.4 cm in the re-do group, respectively. Anastomotic urethroplasty was performed wherever possible, or failing this a substitution urethroplasty. Median follow up was 9 years for primary group and 8 years for re-do group. RESULTS: Primary group: urethroplasty was successful in 22/23, with 10 by perineal and 13 by additional transpubic approach. Two have stress incontinence. Erectile function is unchanged in all and upper tracts are maintained. One had recurrent stricture. Re-do group (12 including 1 recurrence from primary group): anastomotic urethroplasty was done in 5 and substitution urethroplasty in 7. Patients needing substitution had long stricture (>5 cm), stricture extending to distal bulb, or high riding bladder neck. All patients are voiding urethrally. Two patients with substitution required dilatation for early re-stenosis. One appendix substitution required delayed revision. Two have stress incontinence. Erectile function was unaffected. Upper tracts are maintained. CONCLUSIONS: Anastomotic urethroplasty was successful in over 95% of primary cases. In re-do cases it was viable in only 41% of cases; the rest required substitution urethroplasty. Urethral substitution also gave acceptable results.

Laparoscopic or laparoscopic-assisted pelvic surgery in small infants: our experience.

OBJECTIVE: The objective of this study was to review our experience of laparoscopic (LA) or laparoscopic-assisted pelvic surgery (LAPS) in small infants. MATERIALS AND METHODS: The medical records of 35 patients who underwent an LA or LAPS between January 2007 and June 2010 were studied and 21 patients who were younger than 1 year or whose weight was less than 10 kg were included. Indications, procedures, results, and complications were analyzed. RESULTS: The indications of surgery and procedures done were impalpable undescended testes (7 cases--3 single-stage orchidopexy and 4 Fowler Stephen stage I ligation of testicular vessels), Hirschsprung's disease (HD; 5 cases--all laparoscopic-assisted transanal pull-through), anorectal malformation (ARM; 3 cases--all laparoscopic-assisted pull-through), disorders of sexual differentiation (3 cases--1 herniotomy and 2 gonadal biopsy), sacrococcygeal teratoma (1 case--laparoscopic mobilization of pelvic component and posterior sagittal excision), and ovarian mass (2 cases; laparoscopic oophorectomy). There were 14 males, 4 females, and 3 of indeterminate sex. All procedures could be successfully completed without conversion. There was no anesthesia-related complication or need for postoperative ventilatory support. There were no immediate procedure-related complications. One female child with ARM had rectal mucosal prolapse; 1 HD case had rectovaginal fistula that healed following fecal diversion. The major advantages of laparoscopy in different indications were magnified access into the depth of pelvis and early frozen section biopsies in HD, accurate placement of neoanus in the center of muscle complex in ARM, early ligation of vessels and avoidance of laparotomy in sacrococcygeal teratoma, ease of decision making and better mobilization of vessels in undescended testes, and diagnostic accuracy and therapeutic procedure in ambiguous genitalia. CONCLUSION: Laparoscopic pelvic surgery in small infants is a safe procedure with advantage of magnification, access, and cosmesis.

Epidermoid cyst of clitoris mimicking clitoromegaly.

Clitoromegaly in pediatric and adolescent age group is usually indicative of a disorder of sexual differentiation. We report a girl child presenting with clitoral enlargement due to an epidermoid cyst. The cyst was excised with complete cosmetic recovery.