Newer Insights into Prostatic Utricle in Proximal Hypospadias.

Introduction: Prostatic utricles (PUs) are the remnants of Mullerian ducts found in the male posterior urethra. Enlarged symptomatic utricles are known to be associated with hypospadias. There is a dearth of literature defining an enlarged utricle and also its clinical significance. Aims: The aim of this study was to describe anatomical difference of PUs of cases with severe hypospadias and also to find their clinical significance if any. Materials and Methods: A prospective study was carried out, and all patients with proximal hypospadias were enrolled. Cystoscopy was performed in all cases. Symptomatology, size, and location of PU were recorded. PU of >0.5 cm was considered enlarged. Results: In the present series, a total of 70 cases were included over a period of 2 years. Enlarged utricle was found in 47/70 (67.14%) on cystoscopy. They were wide-mouthed and negotiated 9 Fr/11 Fr cystoscope with ease. Recurrent epididymo-orchitis, recurrent urinary tract infection, and obstructive features were the most common complications requiring intervention. Conclusions: PUs in boys with hypospadias are enlarged as well as wide-mouthed. Most of these remain asymptomatic, but few of them carry the potential of complications. Cystoscopy helps in direct visualization of utricular anatomy, so it should be a preferred investigating modality for investigating a PU.

Laparoscopic Surgery in Pediatric Upper Tract Urolithiasis: An Alternate Modality.

INTRODUCTION: Incidence of pediatric urolithiasis has increased over the last few decades. Procedures such as extracorporeal short wave lithotripsy, percutaneous nephrolithotripsy, and ureterorenoscopic lithotripsy are not widely available for pediatric age group in many developing countries. It is desirable that advantages of minimally invasive surgery be offered to selected cases with urolithiasis. MATERIALS AND METHODS: All patients with pediatric upper tract urolithiasis managed laparoscopically from January 2015 to April 2020 were retrospectively reviewed. RESULTS: A total of 38 patients were included. The mean age of the patients was 8 ± 2.85 years. Thirty-four patients (renal and upper ureteric) were managed through retroperitoneal approach, while those with lower ureteric calculi (n = 4) were approached transperitoneally. A total of eight patients required conversion to open technique. The stone clearance rate was 79% by laparoscopic approach alone. There were no procedure-related complications. CONCLUSION: Our study suggests that laparoscopic management for pediatric upper tract urolithiasis is a radiation-free, single-time curative treatment and is feasible in centers where facilities for other endoscopic procedures are unavailable.

Evaluation of Risk Factors Affecting Outcome in Outborn Surgical Neonates.

BACKGROUND: Mortality in surgical neonates contributes to neonatal mortality rates. The study was conceptualized to study clinical and nonclinical factors affecting mortality in surgical neonates so that timely intervention could result in improved survival of the neonates. MATERIALS AND METHODS: The study was initiated after approval from the institutional ethics committee and included 120 surgical neonates over a period of 18 months after obtaining consent from the parents/caregivers. Predesigned pro forma was used to record the details of antenatal care received, place of birth, travel history, maternal education and gestational age, and clinical condition at the time of admission. Values of biochemical tests such as serum electrolytes, serum creatinine, and arterial blood gasses were recorded. The need of inotrope support, blood or blood product transfusion, and postoperative ventilator support and intensive care unit (ICU) care was recorded. The results of the two groups, i.e., survivals and mortality, were compared. Outcome was recorded as mortality at 30 days or earlier. RESULTS: Irrespective of the surgical condition, the survival rate was significantly better in those babies who weighed more than 2.5 kg at the time of admission, had capillary refill time of <3 s, had serum ionized calcium levels more than 1 mmol/L, and did not require inotropes, blood or blood product transfusion, and postoperative ICU care and ventilator support. The place of birth, educational status of the mother, gestational age, and distance traveled for care had no statistically significant effect on survival. CONCLUSION: There is a statistically significant correlation between the survival of the babies who weighed more than 2.5 kg and are more physiologically preserved at the time of admission. Mortality rates can be decreased by timely interventions to reduce the need of inotropes, blood or blood products, and ICU care and ventilator support during their postoperative recovery.

Childhood Phimosis Secondary to Lichen Sclerosus: Is There a Spatial Pattern of Histopathological Changes?

INTRODUCTION: The accurate histopathological diagnosis of the phimotic prepuce is indispensable because early diagnosis, treatment, and close follow-up are crucial in genital dermatosis such as lichen sclerosus (LS). This study analyzes the histopathological spectrum of childhood phimosis with special emphasis on LS. We also highlight a peculiar pattern of histopathological evolution in LS, prepuce. MATERIAL AND METHODS: The histopathology slides of all the pediatric preputial circumcision specimens performed for the indication of pathological phimosis (n = 43) during the study period (2012-2017) were analyzed. Eight histopathological features viz. hyperkeratosis, hypergranulosis, epidermal atrophy, acanthosis, dermoepidermal cleft, upper dermal edema and homogenization, mid dermal lymphocytic band, and interface dermatitis were studied in each case, separately in inner preputial surface, tip, and outer preputial surface. On the basis of evolution of the disease and histopathological features, the lesions of LS were classified into early, established, and advanced. RESULT: LS was found in 32 cases, whereas 11 cases showed nonspecific inflammation and fibrosis. The upper dermal homogenization (n = 29), dermoepidermal cleft (n = 28), and mid dermal band (n = 27) were the commonest histopathological changes. The established and advanced changes were confined to the inner preputial surface (n = 31), and the outer preputial skin surface was unaffected in all the cases. A peculiar histopathological evolution pattern was seen with established or advanced lesions, early lesion, and normal histology on the inner preputial surface, mucocutaneous junction, and outer preputial skin, respectively. CONCLUSIONS: LS is a common cause of childhood phimosis. It shows a peculiar histopathological evolution that mandates the thorough analysis of inner mucosal surface.

A rare cause of wheezing in an infant: Esophageal duplication cyst.

Esophageal duplication cyst (EDC) is classified as a subgroup of foregut duplication cyst. They are very rare and predominantly detected in children. We present an unusual cause of wheezing in a 2-month-old infant. The diagnosis of EDC was suspected by bronchoscopy, provisionally confirmed by magnetic resonance imaging, and followed by successful surgical excision of the cyst. We conclude that foregut duplication cyst of the esophagus is very rare, and must be considered in the differential diagnosis of persistent wheezing in infants who do not respond to conventional treatment.

Myxoid liposarcoma diagnosed on fine needle aspiration cytology: There is more to it than meets the eye.

ABSTRACT: Liposarcoma is a rare mesenchymal neoplasm commonly involving deep soft tissues and the retroperitoneum. Among the various types of liposarcoma, myxoid liposarcoma is the most frequently encountered in adolescents and young adults, with a predilection for lower extremities. Fine needle aspiration allows easy assessment and rapid on-site evaluation for distinguishing benign from malignant lipomatous lesions. Here, we present a case of myxoid liposarcoma in the calf region of a 19-year-old boy, diagnosed via fine needle aspiration cytology, and subsequently confirmed by histopathological examination after surgical resection. The intention behind this case report is to highlight the cytological features of myxoid liposarcoma and to improve understanding of this tumor entity, aiming to prevent misdiagnosis by inexperienced pathologists when evaluating cytology specimens.

Isolated intracholecystic metastasis of renal cell carcinoma: A report of a rare case.

Renal cell carcinomas are known for their unforeseeable metastatic pattern. They are known to have high metastatic potential, thus commonly associated with synchronous or metachronous metastatic presentation. At the time of diagnosis, approximately one-third of patients present with metastatic disease. We present a case of synchronous metastasis of clear cell carcinoma to the gallbladder in a 54-year-old male within two months after radical nephrectomy.

Effect of Laparoscopic Sleeve Gastrectomy on Quality of Life.

PURPOSE: The clinical benefits of bariatric surgery are well-established, but the impact of bariatric surgery on psychosocial outcomes such as health-related quality of life (HRQL) is less clear. The aim of this study is to assess the Quality of life (QOL) as a whole and in separate domains in post-bariatric surgery patients. METHODOLOGY: A single unit cross-sectional analysis of a prospective study is done on QOL in 51 patients undergoing laparoscopic sleeve gastrectomy at tertiary hospital. QOL was assessed by WHOQOL-BREF (World Health Organisation Quality of Life questionnaire -Brief version) questionnaire and Global Quality of Life Scale in each patient. Scores were calculated on a 0-100 scale and results compared. RESULTS: The median scores given by patients before surgery were 14, 21, 42, 40 and 12.5 for each of the parameters physical, psychological, social, environmental and overall well-being respectively. The median scores for after surgery were 86, 87, 91, 88 and 87.5 respectively. The difference was significant (p value 0.001). Global QOL after surgery, calculated year wise, showed QOL scores of 90, 100, 95 and 80 in patients with 1 year, 2 years, 3 years and 4 years of follow-up without any significant difference (p value 0.502). CONCLUSION: Through this study, we emphasize the need for the selection of a standardised scale by international organisations to compare the different studies. By proving the significant differences in the QOL of patients who underwent LSG [laparoscopic sleeve gastrectomy], we suggest to consider the Quality of Life as one of the criteria to consider a patient for bariatric surgery.

Delayed referral for orchidopexy: Scrutinising the causes.

Orchidopexy for undescended testis is recommended at a younger age than heretofore; our study aimed to assess delays, and their causes, by retrospective analysis of data from a single tertiary care centre over one year (2015-2016). Almost 80% of children were brought after 1 year of age, mostly because of delayed referral by primary physicians (60%), or missed diagnosis by parents or primary physicians (20%). Misconception about the risk of surgery below 1 year was significant (15%). A timely referral is encouraged.

Atypical scrotal masses: Surgical surprise.

We present three unusual cases of atypical scrotal lesions in children. The first was a firm left scrotal mass with the testis indistinguishable. The second with apparently a large hydrocoele, which was a cyst, and the third with multiple nodular lesions, pushing the left testis into the right hemiscrotum. These turned out to be a pigmented neuroectodermal tumour, a lymphatic malformation and neurofibromas respectively. Paediatric surgeons should be aware of such surgical surprises.

Comparison of clinical outcome and anal manometry following laparoscopic-assisted anorectoplasty and posterior sagittal anorectoplasty in patients with high and intermediate anorectal malformation: A randomised controlled trial.

Introduction: High and intermediate types of anorectal malformations (ARMs) may be managed by either open posterior sagittal anorectoplasty (PSARP) or by laparoscopic-assisted anorectoplasty (LAARP). Most of the literature favours one approach over the other based on retrospective analysis. We performed this study with the aim to compare the short-term outcomes of both procedures. Materials and Methods: All paediatric patients with high and intermediate ARM were enrolled and randomised into two groups: open PSARP group and LAARP group. Outcome parameters such as faecal continence using Kelly's scoring system, anal manometric parameters and post-operative complications were compared between the groups. Results: A total of 16 patients were included with equal distribution in the open PSARP and LAARP group. Patient's variables were comparable in both the groups. Five patients developed immediate post-operative complications, three in the LAARP and two in the open group. The mean Kelly's score was 3.63 ± 1.6 versus 2.57 ± 1.9 (P = 0.132) for LAARP and PSARP group, respectively. The mean resting pressure was 34.71 ± 6.26 cm of H2O and 35 ± 6.16 cm of H2O (P = 0.384) in LAARP and open group, respectively. Rectoanal inhibitory reflex was demonstrated in 6/7 patients in LAARP group and 5/7 patients in open group. Conclusion: Faecal continence in patients undergoing either of the procedure is comparable. However, wound-related complications are lesser in LAARP procedure.

Catheter test: A reliable alternative to assess adequacy of bladder management in patients of PUV in developing countries.

INTRODUCTION: Following posterior urethral valve ablation, persisting bladder dysfunction can lead to lack of improvement or worsening of hydronephrosis and as well, to lack of improvement or even worsening renal function. On the other hand, managing bladder dysfunction offers the possibility of lessening both the degree of hydronephrosis and the level of serum creatinine. As we have no available urodynamics we feel impelled to find other means of diagnosing and managing abnormal bladder dynamics in these boys. We wanted to determine if a short course of continuous bladder drainage would be an effective means of lowering abnormal serum creatinine levels. Based on an assumption that most bladders following valve ablation, especially in those boys with a persistently elevated serum creatinine post valve ablation, have some form of dysfunction, we wanted to determine if a short course of continuous catheter drainage could improve these abnormal serum creatinine levels. METHODS: The study was conducted over a 3year period and included all children up to 3 years of age who had higher age-appropriate nadir serum creatinine, 3 months post valve ablation. Patients were followed up at 1 week, 2 month and 3-month post valve ablation. On third follow-up visit patients with an elevated serum creatinine level were subjected to continuous bladder drainage (catheter test) for 5 days and serum creatinine level was recorded at the end of 5th day. Enforced bladder management which included dose titration of anti-muscaranic drugs and clean intermittent catheterization (CIC) with or without nocturnal catheter drainage (NOC) was instituted for next 3 months. Serum creatinine was determined at the end of the 3 month period and compared with prior values. RESULTS: A total of 21 patients were included in the study. The mean age of the children at the time of valve ablation was 6.97 ± 4.38 months. There was a significant fall in serum creatinine from 0.64 ± 0.12 mg/dl pre 5 days of drainage to 0.48 ± 0.10 at the end of the 5 day period (p = 0.024) along with a significant rise in GFR (p = 0.001). Following 3 months of enforced bladder management, there was a very mild rise in creatinine to 0.51, a normal age adjusted value. DISCUSSION: The study demonstrates the importance of early diagnosis of bladder dysfunction in boys with an elevated serum creatinine levels post valve ablation. Elevated nadir serum creatinine 3 months post ablation seems to be a useful indicator of possible bladder dysfunction; lowering of serum creatinine following a 5 day trial of continuous bladder drainage confirms this diagnosis. However, the lack of availability of urodynamic testing leaves the specific type of bladder dysfunction undiagnosed. CONCLUSION: Lowering of serum creatinine following a 5-day trial of continuous bladder drainage can be used as a rough indicator of existing bladder dysfunction in boys with a history of PUV and should be used in countries without urodynamic equipment as an indicator for at least a 3 month trial of enforced bladder management.

Cushing syndrome in an infant due to cortisol secreting adrenal pheochromocytoma: a rare association.

Adrenocortical tumors are the most common cause of endogenous Cushing syndrome in infancy and early childhood. Cushing syndrome resulting from ectopic adrenocorticotrophic hormone (ACTH) producing tumor has been infrequently reported in adults. Cortisol secreting pheochromocytoma is rarely reported in literature. We report an eleven month old child presenting to us as Cushing syndrome with hypertension due to left adrenal tumor. He was treated with antihypertensives and left adrenalectomy was done under perioperative glucocorticoid coverage. Diagnosis of pheochromocytoma was made only after histopathology. Despite the rare association of Cushing syndrome and pheochromocytoma, preoperative diagnosis of pheochromocytoma is required for appropriate perioperative medical and anaesthetic management to prevent life threatening complications.

Gastric teratoma with predominant nephroblastic elements.

Gastric teratoma is an uncommon neoplasm in infancy. Most of the gastric teratomas described have mature elements while some have immature elements. Occurrence of nephroblastic rests in gastric teratoma has not been described in available literature earlier.

Spigelian hernia in children: low versus classical.

PURPOSE: Pediatric spigelian hernias are very rare. They are often missed or misdiagnosed. A series of cases with spigelian hernia, presented to a tertiary care center are presented here with emphasis on different anatomy of spigelian hernias with cryptorchidism and those without associated cryptorchidism. MATERIALS AND METHODS: Over a period of seven years, nine cases of spigelian hernia presented to our tertiary care center. Male:female ratio was 3:1.There was a preponderance of right sided hernias. Three patients had associated cryptorchidism. One patient had associated lumbar hernia. All three patients with cryptorchidism had low spigelian hernia while others had classical spigelian hernia. CONCLUSION: There is a likelihood of anatomical variation in SH associated with UDT and those without UDT. Understanding this anatomy may help in correct scrotal placement of testis. TYPE OF STUDY: Prospective Observational. LEVEL OF EVIDENCE: 4.

Aberrant Pancreatic Tissue in a Mediastinal Enteric Duplication Cyst: A Rarity with Review of Literature.

Mediastinal enteric duplication cysts are a rare congenital malformation encountered mainly in neonates and infants. It is a distinct entity within the family of foregut duplication cysts. It can present with respiratory distress due to mass effect and hence surgical excision is the preferred treatment. Histologically, it is characterised by a double layered smooth muscle wall with intestinal lining epithelium. We report a case of mediastinal enteric duplication cyst with aberrant pancreatic tissue in a neonate due to its rarity and early presentation. A neonate presented with respiratory distress and a cystic mass in the right posterior mediastinum. The lesion was excised and on histopathological analysis the diagnosis of mediastinal enteric duplication cyst was made. Also, aberrant pancreatic tissue which has been reported rarely was noted in this case. We discuss this case and review similar cases reported in literature.

Zinc as an adjunct treatment for reducing case fatality due to clinical severe infection in young infants: study protocol for a randomized controlled trial.

BACKGROUND: An estimated 2.7 of the 5.9 million deaths in children under 5 years of age occur in the neonatal period. Severe infections contribute to almost a quarter of these deaths. Mortality due to severe infections in developing country settings is substantial despite antibiotic therapy. Effective interventions that can be added to standard therapy for severe infections are required to reduce case fatality. METHODS/DESIGN: This is a double-blind randomized placebo-controlled parallel group superiority trial to investigate the effect of zinc administered orally as an adjunct to standard therapy to infants aged 3 days up to 2 months (59 days) hospitalized with clinical severe infection, that will be undertaken in seven hospitals in Delhi, India and Kathmandu, Nepal. In a 1:1 ratio, we will randomly assign young infants to receive 10 mg of elemental zinc or placebo orally in addition to the standard therapy for a total of 14 days. The primary outcomes hospital case fatality, which is death due to any cause and at any time after enrolment while hospitalized for the illness episode, and extended case fatality, which encompasses the period until 12 weeks after enrolment. DISCUSSION: A previous study showed a beneficial effect of zinc in reducing the risk of treatment failure, as well as a non-significant effect on case fatality. This study was not powered to detect an effect on case fatality, which this current study is. If the results are consistent with this earlier trial, we would have provided strong evidence for recommending zinc as an adjunct to standard therapy for clinical severe infection in young infants. TRIAL REGISTRATION: Universal Trial Number: U1111-1187-6479, Clinical Trials Registry - India: CTRI/2017/02/007966 : Registered on February 27, 2017.

Mature Teratoma in a Supernumerary Ovary in a Child: Report of the First Case.

BACKGROUND: Supernumerary ovary (ie, ovarian ectopia having no anatomic connection with the normally placed ovaries) is a rare gynecologic condition. To the best of our knowledge, only 1 pediatric case of supernumerary ovary has been reported to date in the English literature. CASE: A 4-year-old girl was assessed for foul-smelling vestibular discharge and was found to have a fistulous tract with opening near the vaginal orifice. Fistuloscopy revealed hair in the lumen of the tract. Computed tomography scan showed a retroperitoneal mass in continuation of the tract. Excision of the mass revealed a mature teratoma in a retroperitoneal supernumerary ovary. SUMMARY AND CONCLUSION: Supernumerary ovary, a gynecologic rarity, is even more uncommon in children. Hence, a thorough clinical-radiological-pathological correlation is mandatory to diagnose extragonadal ovarian tumors arising in supernumerary ovaries.

References in Indian Pediatrics: authors need to be accurate.

To determine the accuracy of references published in Indian Pediatrics, we reviewed the reference lists appended to the original articles published in Indian Pediatrics during the year 2002 (volume 39) for citation and quotation accuracy. A total of 176 references out of 322 cited in 17 original articles could be retrieved from available resources. Errors of citation were found in 69 (39.27 percent) references while errors of quotation were found in 15 (8.6 percent) references. The most common errors were those in the name of authors and title of the article. Contributors should make serious efforts to check the accuracy of the references cited in their manuscripts.