Penile Epithelioid Hemangioendothelioma in a Child.

An 8-year-old boy initially thought to have a penile arteriovenous malformation was later diagnosed with a rare vascular sarcoma, epithelioid hemangioendothelioma (EHE). Despite challenges in diagnosis, he underwent supraselective angioembolization and partial penectomy for oncological clearance. EHE, a low-grade malignancy, requires prompt identification and treatment due to potential systemic involvement.

Extragonadal Germ Cell Tumor with Hepatic Infiltration in a Child: Aberrant Somatostatin Receptors and the Diagnostic Conundrum.

Extragonadal germ cell tumors (GCTs) are challenging to diagnose. We present a case of suprarenal GCT, with hepatic infiltration where differential diagnosis included neuroblastoma and hepatoblastoma. The positive positron emission tomography scan further obfuscated the situation. The diagnosis was clinched by fine-needle aspiration cytology and cell block immunohistochemistry.

Differential invasiveness & expression of antimicrobial peptides in Shigella serotypes.

Background & objectives: The study of Shigella pathogenesis at present is severely hampered by the lack of a relevant animal model that replicates human bacillary dysentery. Different Shigella serogroups cause varying severity of clinical illness. Ex vivo colonization of Shigella flexneri, S. dysenteriae and S. sonnei were characterized in human paediatric colonic pinch biopsies in the in vitro organ culture (IVOC) model to study the invasiveness of Shigella by gentamicin protection assay (GPA). Furthermore, the expression of antimicrobial peptides (AMPs) in response to different serotypes of Shigella was also studied in IVOC model. Methods: IVOC explants were inoculated with 109 colony forming units of different serotypes of Shigella and recovery of bacteria studied. Histopathological analysis was carried out to study inflammatory immune responses. GPA was done to elucidate the invasiveness of different serotypes of Shigella. Secretions of AMPs were measured by enzyme-linked immunosorbent assay (ELISA). Western blotting was performed to check the expression of AMPs and nuclear factor kappa B in IVOC explants. Results: After 24 h post-infection, the colon biopsies showed intense inflammatory reaction. In both IVOC and GPA, S. dysenteriae 1 was the most invasive as compared to S. flexneri and S. sonnei. S. sonnei was the least invasive. ELISA demonstrated that S. sonnei dampened the HBD (human ß-defensin)-2 responses whereas there was augmentation by S. dysenteriae and there was a modest but non-significant increase by S. flexneri. A modest increase in HBD-3 by S. sonnei and S. flexneri was observed but was not found to be significant. However, western blotting data showed upregulation of all AMPs by all serotypes. Western blotting is more sensitive than ELISA. Interpretation & conclusions: In the present study, differences in invasiveness and AMP production induced by different serotypes of Shigella were found. Human intestinal IVOC represents a model system to investigate early interaction between pathogenic bacteria and the human gut.

Colonic Atresia Associated with Biliary Atresia.

Colonic atresia (CA) is an uncommon type of intestinal atresia commonly associated with other anomalies, while biliary atresia (BA) is also rare but usually an isolated anomaly. The pathogenesis for either of the anomalies is unclear. The co-occurrence of both pathologies has not been mentioned in the literature. We here discuss the management of CA with BA and the review of pertinent literature.

A Randomized Control Trial to Evaluate the Effect of Local Instillation of Mitomycin-C at the Porta after Kasai Portoenterostomy in Patients of Biliary Atresia.

Background: Kasai portoenterostomy (KPE) is the initial treatment for biliary atresia (BA). Even after initial jaundice clearance, a significant number of children presented with the reappearance of symptoms due to ongoing fibrosis involving porta and intrahepatic ducts. Mitomycin-C (MMC) is an antifibrotic agent, and the study hypothesized that local application of MMC at porta can decrease fibrosis, which can improve jaundice clearance and lead to better native liver survival (NLS). Materials and Methods: This prospective randomized control trial included children with BA, who were allocated to groups A or B. The patients in both groups underwent standard KPE; in addition, a 5 French infant feeding tube (IFT) was placed near the porta through the Roux limb in Group B children. During the postoperative period, MMC was locally instilled over the porta in Group B children through IFT. Postoperative jaundice clearance and NLS were assessed and compared. Results: A total of 27 children were enrolled in the study, 16 in Group A and 11 in Group B. Both groups were comparable preoperatively. Although the NLS was not statistically significant in Group B, the survival was quite higher, that was 91%, 81%, and 73% at 6 months, 1 year, and 2 years, respectively, compared to 63%, 50%, and 38% in Group A. Conclusion: Children in Group B clinically showed an early jaundice clearance and a better trend of serial bilirubin levels as well as longer NLS than Group A, but it was not statistically significant. The procedure was technically easy, and no complication was encountered related to surgical technique or MMC instillation.

Comparative Assessment of Autogenous Cancellous Bone Graft and Bovine-Derived Demineralized Bone Matrix for Secondary Alveolar Bone Grafting in Patients With Unilateral Cleft Lip and Palate.

OBJECTIVE: Comparison between bovine-derived demineralized bone matrix (DMBM) and iliac crest graft over long term for secondary alveolar bone grafting (SABG) in patients with unilateral cleft lip and palate (UCLP) in terms of radiological and clinical outcomes. DESIGN: Prospective, randomized, parallel groups, double-blind, controlled trial. SETTING: Unit of Oral and Maxillofacial Surgery, Oral Health Science Centre, Postgraduate Institute of Medical Education & Research, Chandigarh. PARTICIPANTS: Twenty patients with UCLP. INTERVENTIONS: Patients were allocated into group I (Iliac crest bone graft) and group II (DMBM) for SABG. Outcomes were assessed at 2 weeks, 6 months, and then after mean follow-up period of 63 months. OUTCOMES MEASURES: Volumetric analysis of the grafted bone in the alveolar cleft site was done through cone beam computed tomography using Cavalieri principle and modified assessment tool. Clinical assessment was performed in terms of pain, swelling, duration of hospital stay, cost of surgery, alar base symmetry, and donor site morbidity associated with iliac crest harvesting. RESULTS: Volumetric analysis through Cavalieri principle revealed comparable bone uptake at follow-up of 6 months between group I (70%) and group II (69%). Modified assessment tool showed no significant difference between horizontal and vertical bone scores over short- and long-term follow-up. In group II, there was higher cost of surgery, but no donor site morbidity unlike group I. CONCLUSIONS: Demineralized bone matrix proved analogous to iliac crest bone graft as per volumetric analysis over shorter period. However, although statistically insignificant, net bone volume achieved was lower than the iliac crest graft at longer follow-up.

Transcutaneous Electrical Nerve Stimulation in Management of Neurogenic bladder Secondary to Spina Bifida.

Aims: To assess the role of transcutaneous electrical nerve stimulation (TENS), alone or in combination with anticholinergic drugs in the management of neurogenic bladder (NB) in spina bifida (SB). Materials and Methods: All the consecutive patients, visiting outpatient clinic between July 2017 and December 2018, who were toilet trained and at least 1 year post-SB surgery with clinical and/or urodynamic evidence of NB, were included in the study. Out of 65 patients, 40 fulfilled the inclusion criteria and were randomised into: group A (ten patients, placebo TENS with anticholinergic agents), Group B (14 patients, TENS therapy with placebo medications) and Group C (16 patients, TENS therapy with anticholinergic medications). All the patients maintained a voiding diary and underwent assessment before and after the intervention. The study was approved by the Institutional Ethics Committee. Results: The presenting symptoms were urinary incontinence (100%), increased frequency (45%), straining during micturition (22.5%), urgency (22.5%), and hesitancy (30%). The demographic parameters were comparable in all the groups. After group specific intervention, the wet episodes/day significantly improved in Group C (P = 0.001). Similarly, the mean wet days/week also improved significantly in Group C (6.5-4.37 days/week, P = 0.01). Out of 40 patients, 29 had abnormal findings on ultrasonography before the start of the therapy. Following intervention, only two patients in Group C showed normalization of findings. On Urodynamic studies, detrusor pressure (Pdet max) decreased in all the groups; however, the patients in Group C, showed the maximum reduction (56.6 ± 11-30 ± 6.7 cm H2O). Similarly compliance (9.4 to 14.5 cm H2O, P = 0.02) and bladder capacity (68%-88% of EBC, P = 0.001) also improved significantly in Group C as compared to other 2 groups Overall, nine patients (Group A, B, and C = 1, 3, and five patients, respectively) showed detrusor instability, while post therapy, only one patient (Group B) had unstable bladder. Maximum decrease in postvoid residue (mean) was also observed in Group C (77-41 ml, P = 0.01). Conclusions: The application of TENS in NB secondary to SB is effective and its application led to improvement in symptoms, decrease in the wet episodes/day, maximum detrusor pressure, instability, bladder compliance, and capacity. TENS therapy in combination with anticholinergic agents had a better outcome as compared to monotherapy with either of the two modalities.

Congenital Malignant Rhabdoid Tumor of Neck.

Malignant rhabdoid tumors (MRT) are uncommon, highly aggressive tumors arising usually from the central nervous system and kidneys. Nonrenal and noncentral nervous systems MRT are rare in neonates. To the best of our knowledge, only five cases of congenital MRT of neck have been described till date. We present a rare case of congenital MRT of the neck in a neonate along with review of literature.

Not All Pediatric Bladder Masses are Rhabdomyosarcoma: Report of a Rare Case With Review of the Literature.

Leiomyoma of the urinary bladder in the pediatric age group is one of the rarest tumors with only 2 case reports in the English literature. Rhabdomyosarcoma is the commonest tumor at this site in this age group. It needs to be distinguished from rhabdomyosarcoma by histomorphology for appropriate treatment and management. We report the third case of urinary bladder leiomyoma arising in a 6-year-old boy with a short review of the literature.

Choroid plexus cysts-antenatal course and postnatal outcome in a tertiary hospital in North India.

PURPOSE: To assess the gestational course and postnatal outcome of antenatally diagnosed choroid plexus cysts. METHODS: From January 2015 to October 2017, mothers carrying foetuses with choroid plexus cysts (CPCs) were followed up prospectively till delivery, and subsequent postnatal assessment of all the babies was done. Progress of the lesions was monitored during pregnancy with ultrasound (USG) scans and postnatally analysed with clinical assessment and USG examination of the cranium. RESULTS: A total of 67 mothers of antenatally diagnosed CPCs were referred to the Paediatric Surgery Unit for opinion. Mean gestational age at the time of diagnosis was 19 ± 2.1 weeks (17-28 weeks) while mean age of the mothers at the time of referral was 26 ± 3.3 years (19-35 years). Sixty four mothers have undergone repeat antenatal scans after the diagnosis, and the CPCs disappeared completely in 60 (93.8%) of them. The CPCs persisted but reduced in size in 2 (4.1%), and the other 2 have been lost to follow-up. Thirty eight patients (56.7%) have unilateral CPCs. Mean size of cysts is 6.08 ± 3.0 mm (2.4­14.8 mm). One foetus, in which the CPC disappeared antenatally, developed it again on the postnatal scans, but is doing well on follow-up. Thirteen babies (19.4%) have other associated congenital malformations as well. CONCLUSIONS: CPCs may involve one or both ventricles and disappear in approximately 95% of the cases, mostly within 2 months from the diagnosis. The size of the cyst has no relation to its rate of disappearance. Postnatal persistence of CPCs is uncommon and the neurological sequel is unlikely; hence, prospective parents should be reassured accordingly.

Anorectal Malformations in Males: Pros and Cons of Neonatal versus Staged Reconstruction for High and Intermediate Varieties.

BACKGROUND: High and intermediate types of anorectal malformations (ARMs) in male neonates may be managed either by primary neonatal reconstruction without colostomy cover or by traditional policy of staged reconstruction after neonatal colostomy. Posterior sagittal anorectoplasty (PSARP) is the current widely practiced reconstructive technique with varied results. AIM: To assess our functional results of PSARP without colostomy in male neonates with high and intermediate ARMs compared to 3-stage (neonatal colostomy - PSARP - colostomy closure) methodology in a high volume tertiary care institution of a developing country. PATIENTS AND METHODS: The number of colostomies performed for male high/intermediate anomalies and the number of babies who completed 3-stage reconstruction during a 10-year period is analyzed. The outcome of primary neonatal PSARPs during the same period was analyzed. Eighty primary PSARPs were compared to 81 staged reconstructions for outcome analysis, using Kelly score. RESULTS: A total of 453 colostomies were performed, but only 253 of them completed all stages of reconstruction (52%). Good continence was achieved in 45% of cases of primary PSARP versus 26% in 3-staged surgery. CONCLUSIONS: Primary PSARP in neonatal period without colostomy is a good option for high and intermediate ARMs in males if the treating surgeon is reasonably skilled in neonatal surgery and PSARP procedures.

Hyperchloremic Metabolic Acidosis Following Total Gut Irrigation with Normal Saline in Pediatric Patients: A Rare Occurrence.

Use of 0.9% sodium chloride for total gut irrigation (TGI) through nasogastric route is an effective method of bowel preparation in children undergoing colorectal surgeries. TGI with normal saline (NS) can result in nausea, vomiting, abdominal distension, and mild electrolyte imbalance; however, hyperchloremic metabolic acidosis has not been documented. We report two cases of hyperchloremic metabolic acidosis in children posted for colorectal surgery following TGI with NS who were successfully managed.

Tubularized incised plate urethroplasty and grafted tubularized incised plate urethroplasty: systematic review, meta-analysis and trial sequential analysis.

Background: Hypospadias is one of the most common genital birth defects. There are around 300 various techniques available for the repair of hypospadias. This study aims to compare the reported outcomes of Tubularized incised plate urethroplasty (TIP) and Grafted TIP (GTIP) repair in children undergoing primary hypospadias repair. Methods: This meta-analysisadhered to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, and we framed our research question using the population, intervention, control and outcomes format. We conducted comprehensive electronic searches across various databases, employing a Boolean search strategy with predefined search terms. Only randomized controlled trials (RCTs) were included for quantitative analysis. Results: Totally, 10 RCTs met our inclusion criteria for quantitative analysis. The results indicated that urethrocutaneous fistula, glans dehiscence, and stricture rates were comparable between the two groups. The incidence of meatal stenosis was found to be significantly lower in the GTIP group with a relative risk (RR) of 0.32 (95% confidence interval (CI) 0.15 to 0.67). Conclusion: The coucomes UCF, glans dehiscence, and stricture rates were comparable between the two groups. Notably, the incidence of meatal stenosis was found to be significantly lower in the grafted TIP group. In terms of operative time, our quantitative synthesis demonstrated that the TIP group had a shorter operative time than the GTIP group with significant heterogeneity.

A randomized study of surgical site infiltration with bupivacaine or ketamine for pain relief in children following cleft palate repair.

BACKGROUND: Wound infiltration with ketamine reduces postoperative pain after tonsillectomy by NMDA receptor blockade and local anesthetic effect. OBJECTIVES: To evaluate the postoperative analgesia after surgical site infiltration with bupivacaine or ketamine in children undergoing cleft palate surgery. MATERIALS AND METHODS: After institutional ethics committee approval and parental consent, 50 ASA-1 children of age 1-6 years undergoing palatoplasty were included in this prospective randomized double-blind study. A standardized technique of general anesthesia was used. The surgical site was infiltrated with either 2 mg·kg(-1) of bupivacaine (Group B) or 0.5 mg·kg(-1) of ketamine (Group K). Pain (Children Hospital Eastern Ontario Pain Score), sedation, dysphagia, nausea, vomiting, and sleep pattern were assessed postoperatively up to 24 h. RESULTS: CHEOPS scores were similar in both the groups up to 12 h but were lower with ketamine compared with bupivacaine at 24 h postoperatively (P = 0.01). Fewer children required rescue analgesics in Group K (28%) than in Group B (64%; P < 0.01). The time to first rescue analgesic and the amount of analgesics used in 24 h were similar in the two groups. Fewer children suffered from dysphagia in Group K (52%) than in Group B (88%; P < 0.01). More children in Group B had disturbed sleep than in Group K at 6 (88% vs 56%; P = 0.012) and 12 h (60% vs 24%; P = 0.01) postoperatively. None of the children had deep sedation, desaturation or respiratory depression. CONCLUSION: Surgical site infiltration with either bupivacaine or ketamine provides adequate analgesia and is devoid of major side effects. Ketamine is superior to bupivacaine in terms of requirement of rescue analgesic, peaceful sleep pattern and early resumption of feeding.

Vesicoureteral reflux: Endoscopic therapy and impact on health related quality of life.

AIM: To evaluate the health related quality of life (HRQOL) after endoscopic injection treatment for vesico ureteral reflux (VUR) in children. MATERIALS AND METHODS: Fifty four children received treatment and were prospectively evaluated for their quality of life scores, according to resolution of reflux on cystograms and status of renal scars. RESULTS: Of the 81 refluxing units, 72 (89%) had resolution of reflux whereas 9 (11%) did not resolve. The total average QOL was higher for the patients in the resolved group as compared to the non resolved group. Comparison of pre and post procedure renal DMSA scans in 44 patients showed status quo in 26, regression of scars in six, progression in two and formation of new scars in 6. The total increase in HRQOL was highest in regression group (67.91), and lowest in progression group (36.45). CONCLUSIONS: Successful endoscopic treatment of VUR is associated with improved quality of life, as indicated by higher HRQOL scores in the resolved group.

Differential dendritic immune cell responses to infection with various serotypes of Shigella.

PURPOSE: Dendritic cells (DC) are key regulators of immune response with the ability to affect both the innate and adaptive immune responses and are abundant in the gut mucosa. The severity of shigellosis varies with the serotype involved with S. dysenteriae producing the severest infections with complications and S. sonnei being at the other end of spectrum usually causing mild self-limiting diarrhea. While Shigella are known to induce the apoptosis of mature DCs, there is no information on cytokine milieu of DCs incubated with different serotypes of Shigella. METHODS: Monocyte derived dendritic cells (MoDCs) were developed from healthy human PBMC after 8 days of culture. DCs were infected with different Shigella serotypes. After 24 â€‹h post infection, relative expression of cytokines IL-1ß, IL-6, IL-8, TNF-α, IL-12p70, IL-17, IL-22 and IL-23 was studied by Real Time PCR and cytometric bead arrays (CBA). RESULTS: We found that different serotypes of Shigella significantly stimulated production of IL-1ß, IL-6, IL-8, IL-12, IL-23, INF-γ and TNF-α compared to uninfected DCs and there were significant differences among these serotypes. At transcriptional level, highest levels of expression of IL-1ß, IL-6, IL-8, TNF-α, IFN-γ, IL-17, IL-22 and IL-23 were observed in S. dysenteriae infected DCs. Significant serotypic differences were noted between S. dysenteriae & S. flexneri and between S. dysenteriae &S. sonnei. CONCLUSIONS: DCs are critical sentinel cells that relay microbial presence either directly or indirectly to naive T cells. In this study we found that S. dysenteriae caused maximum expression of pro-inflammatory cytokines. Similarly, S. dysenteriae also caused highest expression of IL-17A and IL-22A. It was the only serotype, which increased IL-23. These findings could explain more severity of SD as compared to SF and SS.

Pen Cap Aspirations: Maneuvering for Successful Extraction.

Aim of this study was to analyze the therapeutic approach with enhanced success of bronchoscopy. We retrospectively analyzed the results of bronchoscopic extraction of pen cap aspirations in our unit from 2000 to 2014. Out of 239 bronchoscopies performed for foreign bodies in airways, there were 8 cases of pen cap aspirations. All 8 were males and age ranged from 5 to 11 years. Seven cases had definitive history, while one patient had a history of chronic respiratory ailment. The foreign bodies were lodged in right and left bronchi in four cases each and seven had their open-end toward the proximal airways. Bronchoscopic extraction was successful in seven patients. The maneuver of rotating the optical forceps by 90 degrees so as to align the pen cap with the vocal cords resulted in success in all the cases. One patient underwent thoracotomy and bronchotomy for removal. Pen cap aspirations occur in school-going children. Aligning the optical forceps with vocal cords during extraction avoids the need for tracheostomy or thoracotomy.

Blunt Trauma Pancreas in Children: Is Non-Operative Management Appropriate for All Grades?

PURPOSE: Blunt trauma of pancreas in children is uncommon and its management varies from observational to early operative intervention. We analysed the feasibility and outcome of non-operative management in all grades of paediatric pancreatic injuries. METHODS: A total of 15 patients of pancreatic trauma seen in a Paediatric Surgery Unit were retrospectively analyzed. RESULTS: Age of the patients ranged from 3-11 years (mean, 7.7 years). The mode of injury was local trauma in 9 children. Only 3 patients had associated injuries and all were haemodynamically stable. Serum amylase levels were raised in 12 patients at admission which ranged from 400-1,000 IU. Computed tomography scan made a correct diagnosis in 14 patients. Grades of the injury varied from grade I-V (1, 3, 6, 4, 1 patients respectively). Fourteen patients were managed conservatively. One patient underwent laparotomy for suspected superior mesenteric hematoma. The average duration of enteral feeds was 3.7 days and of hospital stay was 9.4 days. Six patients formed pancreatic pseudocysts; two were managed conservatively while the other four underwent cystogastrostomy. The patients were followed up for a period of 1-12 years. All remained asymptomatic and none had exocrine or endocrine deficiencies. CONCLUSION: Non-operative treatment for isolated blunt trauma of pancreas in children may be safely followed for all the grades of injury; if associated injuries requiring surgical intervention are ruled out with a good quality imaging and the patients are hemodynamically stable. It did not increase the hospital stay and morbidity and avoided operative intervention on acutely injured pancreas.