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.

A rare presentation of Y-duplication of the pancreatic tail with enteric duplication cyst as intussusception in a child: A case report.

We report a case of a 2-year-old girl who presented to us with complaints of pain abdomen and non-bilious vomiting. USG was suggestive of intussusception with necrotic bowel and free fluid in the left lumbar region. Operative findings were of enteric duplication cyst of the pancreas which was later confirmed on histopathology. Enteric duplication cysts can occur anywhere in the gastrointestinal tract but duplication cyst within the pancreas is rare. The most common presentation in other reported cases is pain abdomen due to pancreatitis but in our case, we had findings of intussusception. Children usually present with a severe complication like perforation of duplication cyst as in our case. Excision of the cyst and the duplicated pancreatic tail is the mainstay of treatment.

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.

Stuttering Priapism in a Teenage Boy: Lesson to be Learnt.

Untreated priapism can lead to ischemic damage of the penis and impotence. This case report describes a 14-year-old boy who presented with a history of priapism for 2 months, which was undiagnosed, ridiculed, and ignored even by medical practitioners. The underlying etiology was later identified to be chronic myeloid leukemia. Despite the usage of multimodal treatment, it took 7 days for control of priapism. The young boy is now left with an erectile dysfunction. The case highlights that priapism in children is a medical emergency needing aggressive evaluation and treatment.

Vascular malformation of ileum: A possible cause of neonatal intestinal obstruction.

Vascular lesions of gut are rare and they may rarely cause luminal obstruction in neonates. A baby boy born at 36 weeks of gestation presented with small bowel obstruction on day 3 of life. X-ray suggested small bowel obstruction. Contrast enema showed microcolon with multiple filling defects. Exploration revealed thin floppy gut with multiple segments of stenoses and dilatation with surface bluish discoloration but no perforation, two areas were resected. Proximal anastomosis was done and distally a stoma was formed. Histopathology showed vascular malformation in all the layers of gut. The diagnosis was impossible preoperatively and could be established after surgery only.

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.

Role of uroflowmetry before and after hypospadias repair.

AIMS: To study the role of uroflowmetry in the preoperative and early postoperative period in children undergoing hypospadias repair. MATERIALS AND METHODS: Twenty-six cases undergoing hypospadias repair over 1 year (tubularized incised plate [TIP] Snodgrass [17 patients], TIP with Snodgraft [5 patients], Duckett's onlay flap [2 patients], and Duckett's tube [2 patients] urethroplasty) were prospectively evaluated with preoperative ultrasound and uroflowmetry and postoperative uroflowmetry at 3 months after the surgery and at 6 and 9 months interval if these dates fell within the study period on follow-up. The parameters studied were maximum flow rate (Qmax), average flow rate (Qav), total voided volume, voiding time, and type of curve. Preoperative and postoperative uroflow data were compared. RESULTS: Twenty-six cases comprised of anterior hypospadias (n = 8), mid penile (n = 11), and posterior hypospadias (n = 7). Fourteen patients had obstructed flow rates preoperatively. While 69% patients (18/26) had obstructed flow rates at 3 months postoperatively, it dropped to 43% at 9 months. Following TIP (Snodgrass) repair, 88% (15/17) had obstructed flow rates postoperatively. Best results were seen in patients undergoing circumferentially epithelialized urethral reconstruction (TIP with Snodgraft, Duckett's onlay flap, and Duckett's tube). CONCLUSIONS: Abnormal uroflow is an inherent aspect of hypospadias in 50% of the cases. Both preoperative and postoperative uroflow evaluation is necessary for meaningful conclusion. Patients with preoperative normal flow rates but obstructed postoperative flow rates need clinical evaluation. Obstructive flow rates are more common after TIP (Snodgrass) repair. The urinary flow rates improve with time.

Clinical importance of duodenal recesses with special reference to internal hernias.

INTRODUCTION: The detailed knowledge of the peritoneal recesses has great significance with respect to internal hernias. The recesses are usually related to rotation and adhesion of abdominal viscera to the posterior abdominal wall and/or the presence of retroperitoneal vessels which raises the serosal fold. The duodenal recesses are usually related to the 3rd and 4th parts of the duodenum. Internal hernias with respect to these recesses are difficult to diagnose clinically and usually noticed at the time of laparotomy. So, the knowledge of these recesses can be valuable to abdominal surgeons. MATERIAL AND METHODS: The present study was conducted in 100 cases including 10 cadavers, 45 post mortem cases and 45 cases undergoing laparotomy. RESULTS: We found superior and inferior duodenal recesses in 28% and 52% respectively, paraduodenal in 12%, mesentericoparietal in 3%, retroduodenal in 2% and duodenojejunal in 18% of cases. Two abnormal duodenojejunal recesses were found, one on the right (instead of the left) of the abdominal aorta, and in the other the opening was directed upwards instead of downwards. The incidence of internal hernias was 3%. CONCLUSIONS: Thus it was observed that there is low incidence of superior and inferior duodenal recesses, and high incidence of paraduodenal recess. The abnormal recesses might be due to malrotation of the gut. In laparotomy cases, the internal hernia was noticed when the abdomen was opened for intestinal obstruction. The incidence of internal hernia was found to be high.

Association of Levels of Serum Inhibin B and Follicle-stimulating Hormone with Testicular Vascularity, Volume, and Echotexture in Children with Undescended Testes.

AIMS: The aim of our study was to assess the association between reproductive hormones (inhibin B [inh B], follicle-stimulating hormone [FSH]) with testicular volume, echogenicity, and blood flow (resistive index [RI]) in children with undescended testis (UDT). SETTINGS AND DESIGN: This was a prospective study of 1-year study duration. MATERIALS AND METHODS: A total of 33 patients (16 unilateral and 17 bilateral) UDTs aged 5-12 years with palpable UDT were included in the study. Morning fasting blood samples were taken for estimation of serum inh B and FSH as well as inh B/FSH ratio. Testicular ultrasound was done to compute testicular volume, testicular echogenicity, and testicular vascularity in terms of RI. RESULTS: The mean age of patients enrolled in the study was 8.29 years for unilateral UDT and 7.97 years in bilateral UDT and it was comparable. The study groups were further subdivided into two age-wise subgroups school goers (5-8 years) and prepubertal (9-12 years). The values of inh B, FSH, and inh B/FSH ratios as well as mean testicular volume were comparable between both groups and subgroups. Overall mean testicular volume had a positive correlation with FSH, inh B, and inh B/FSH, but statistical significance was reached only for inh B (P < 0.001) in children with both unilateral and bilateral UDT. Apart from five patients with hypoechogenicity within the testis, all remaining testes were of homogenous echotexture with no instances of irregular echogenicity or tumor. Children with RI >0.6 were separately studied. The incidence of high RI (>0.6) was also comparable in unilateral or bilateral disease. These subjects had unfavorable biochemical parameters in terms of low inh B levels and high FSH levels. CONCLUSIONS: Our findings hint to the fact that palpable UDT forms a homogenous group, whether unilateral or bilateral, whereas impalpable testes may form a separate category and need further studies to substantiate this hypothesis.

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.

Hormonal profile in children with isolated hypospadias associates better with comprehensive score of local anatomical factors as compared to meatal location or degree of chordee.

BACKGROUND: To evaluate if hormonal profile of children with isolated hypospadias (IH) associates better with comprehensive local anatomical factor score (LAFS) than with clinically adjudged urethral meatus location or severity of chordee/k.j. MATERIAL AND METHODS: Ninety-nine children with IH were enrolled, as per inclusion criteria. Meatal location was recorded at first clinical examination in OPD; while LAFS was computed per-operatively using indigenously devised scale, except for neonates. Hypospadiacs were first classified into three standard meatal based groups and subsequently into LAFS based two groups (≤19, >19). For all participants, pre HCG and post HCG (96 hour post- injection) estimation of serum gonadotropins, DHEA-S, estrogen (E), progesterone (P), testosterone (T) and Dihydrotestosterone (DHT) was done. Statistical tests were applied to assess significance of hormonal levels with respect to meatal location, chordee and LAFS. RESULTS: Only FSH levels differed significantly among meatal based groups; while among LAFS groups, multiple hormonal differences were noted; with poor LAFS associated significantly with higher FSH, LH and lower E, T/DHT. Children with severe degree of chordee had poorer T output and a significantly lower LAFS as compared to those with moderate/mild chordee. CONCLUSION: Serotoli cell dysfunction, indirectly indicated by high FSH was found among midpenile hypospadiacs and those with poorer LAFS. Since groups based on LAFS revealed multiple intergroup hormonal differences than what was seen for meatal/chordee based groups; LAFS should be considered a better guide for prognostication and for deciding about hormonal supplementation. Lower androgenic output was particularly noted in children with severe chordee.

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.

Inflammatory myofibroblastic tumor: an unusual mimicker of childhood intrathoracic tuberculosis.

Inflammatory myofibroblastic tumors are rare lesions of uncertain etiology that are often difficult to diagnose because of their myriad clinical presentations. Not uncommon, they mimic persistent pneumonia. We report a 4-year-old girl who presented with prolonged pyrexia, weight loss, severe anemia, hepatosplenomegaly, and nonresolving pneumonia. Initial investigations including flexible bronchoscopy and bronchial washing for usual causes of persistent pneumonia, such as tuberculosis and other infections, were negative. Chest computed tomography revealed a well-defined lesion involving the lingula and left upper lobe with extension into the subpleural space. Pleural tap and biopsy was also noncontributory. Thoracoscopic biopsy was suggestive of an inflammatory myofibroblastic tumor. As the lesion was encasing the major vessels, it was considered inoperable. The patient did not respond to steroid therapy and etoricoxib and later succumbed to the illness. This uncommon tumor should be considered in the differential diagnosis of children who presented with unresolving consolidation with pyrexia.