A Novel Association of Colonic Duplication with Mayer-Rokitansky-Kuster-Hauser Syndrome in an Adolescent Girl.

The association is defined as two or more malformations with unclear relationships, which do not fit the criteria of a syndrome. This case report presents a rare finding of tubular colonic duplication in association with Mayer-Rokitansky-Küster-Hauser syndrome in an adolescent girl. The patient presented with gastrointestinal bleeding and abdominal pain, necessitating surgical intervention. The successful excision of the duplicated segment highlights the importance of prompt diagnosis and treatment in such cases.

A Pilot Survey of Indian Stakeholders: Parents, Doctors, and Grown-Up Patients of Disorders of Sexual Differentiation on Management Decisions and Associated Gender Dysphoria.

Background and Aims: Of late, there are many legal representations from select quarters to halt all medical interventions in children with differences of sex development (DSD). In this survey on management decisions in DSD, we distil the views of Indian stakeholders: parents, physicians, and grown-up patients with DSD on their management decisions to identify decisional satisfaction or gender dysphoria. Methods: The survey domains included the patient demographics, final diagnosis, decision on the sex of rearing, surgical interventions, opinion of the stakeholders on the preferred age of sex assignment, final sex of rearing, and agreement/disagreement about sex assignment (gender dysphoria). Results: A total of 106 responses were recorded (66% parents, 34% grown-up patients aged 12-50 years). Among parents, 65/70 (95%) preferred the sex to be assigned soon after birth. All grown-up patients preferred sex to be assigned soon after birth. Regarding decisions on surgery, 74% of physicians and 75% of the grown-up patients felt parents should be allowed to decide interventions. Among Indian parents, 90% felt they should have the right to decide surgery in the best interest of their child for a safe social upbringing. Overall, gender dysphoria among Indian DSD patients was <1% (1/103, 0.97%). Conclusions: The predominant preference and opinion of major Indian stakeholders (physicians, parents, and grown-up DSD patients) support the existing approach toward DSD management, including early sex assignment and necessary medical intervention.

Role of Urinary Biomarkers (Transforming Growth Factor ß1, Neutrophil Gelatinase-Associated Lipocalin, and Cystatin C) as a Prognostic Factor of Renal Outcome in the Posterior Urethral Valve.

Background: The urinary biomarker response precedes the appearance of any renal structural or functional derangement. Transforming growth factor-ß1 (TGF-ß1), neutrophil gelatinase associated lipocalin (NGAL), and Cystatin C (CysC) can act as the early prognostic markers in posterior urethral valve (PUV) patients. Aim: To compare the urinary levels of TGF-ß1, NGAL, and CysC between PUV cases and age matched controls and to correlate these with renal structural and functional parameters. Materials and Methods: This prospective study included children with PUV diagnosed using the standard investigations and an equal number of age-matched controls with nonurological problems. For the study subjects, the urinary samples were collected at three different time points (pre- and postoperatively at 3 and 6 months), whereas for controls, only single-voided samples were studied. The urinary levels of TGF-ß1, NGAL, and CysC were estimated by the standardized techniques using the ELISA kits. Statistical methods were used to drive the comparisons between cases and controls. Results: Fifteen children with a median age of 10 (5-48) months were enrolled in each of the two groups. The mean uTGF-ß1 in the case group was significantly higher at all three time points (43.20 ± 6.13 pg/ml, 43.33 ± 11.89 pg/ml and 40.71 ± 9.01 pg/ml) as compared to the control group (29.12 ± 8.31 pg/ml) (P ≤ 0.001). The median uNGAL in the case group was also higher (17.78 ng/ml, 2.35 ng/ml and 2.536 ng/ml) as compared to the control group (1.31 ng/ml). However, the difference was significant only preoperatively (P = 0.02). The median uCysC in case group was similarly higher (0.347 µg/ml, 0.439 µg/ml, and 0.382 µg/ml) than the control group (0.243 µg/ml) (P > 0.05). Serum creatinine in the case group (0.49 mg/dl) showed no significant rise above that of control (0.24 mg/dl). A cutoff value of uTGF-ß1 = 36.55 pg/ml (P < 0.001), uNGAL = 0.879 ng/ml (P = 0.02), and uCysC = 0.25 µg/ml (P = 0.22) was found to be associated with renal damage in PUV. A significant correlation was found between uNGAL and S. creatinine at 3 months (r = 0.43, P = 0.017) and 6 months (r = 0.47, P = 0.08). Conclusion: The elevated uTGF-ß1, a decline in uNGAL and an increase in uCysC suggests ongoing inflammation, improvement in hydronephrosis and a prolonged proximal tubular dysfunction in PUV patients, respectively.

Ileal Trichobezoar Presenting with Acute Appendicitis and Intestinal Obstruction: A Rare Case Report and Review of Literature.

Trichobezoar is a rare diagnosis among pediatric patients highlighting underlying psychiatric illness. Gastric bezoar with a long tail extending into small bowel may present with varied presentation including small bowel obstruction. Isolated small bowel trichobezoar is rare making diagnosis difficult highlighted in the index case.

Foreign Body Ingestion in Children: An Experience of 99 Cases in a Tertiary Care Center in Delhi.

Background: Accidental ingestion of foreign bodies in children is critical, as the inability to effectively communicate can potentially lead to devastating consequences. We aimed to determine the epidemiology of foreign body ingestion and variability according to age, gender, type, and location of foreign body, and describe its management. Aim and Objective: The aim was to study the various types of foreign body ingestions in children admitted to pediatric surgery and their management. Materials and Methods: A retrospective study was conducted from January 2020 to June 2022 on children under the age of 12 years with a confirmed diagnosis of foreign body ingestion. Patients were clinically and radiologically assessed, after which standard protocols were followed wherein patients were followed by either observation or emergent management. Emergent management included removal of the foreign body by either endoscopy or surgery. Comparisons among multiple age groups, gender, type of foreign body, location of foreign body, and their management were analyzed. Results: Out of 99 subjects in our study, there were 76 boys and 23 girls. The median age of presentation was 5 years. Most children were asymptomatic at presentation. The most frequently ingested foreign body was a coin in all age groups. The majority of the foreign bodies were suspected to be in the small bowel. The foreign bodies that had crossed the duodenojejunal flexure (n = 74, 74.7%) were managed conservatively with the observation of a variable period of a minimum of 24 h and a maximum of 48 h. 21 cases were managed by endoscopic removal, while three cases required surgical intervention. Conclusions: Overall, the most common gastrointestinal foreign body was a coin in all age groups. Button battery is the most worrisome foreign body; however, depending on its position, it can be managed conservatively. Upper GI foreign bodies can be safely removed endoscopically. Parental counseling is very important for the prevention of ingestion of foreign bodies.

Pediatric Age-adjusted Shock Index (SIPA): From Injury to Outcome in Blunt Abdominal Trauma.

Purpose: The Shock Index Pediatric Age-Adjusted (SIPA) score is a useful tool for identifying pediatric trauma patients at a risk of poor outcomes and for triaging. We are studying the relationship between elevated SIPA score and specific outcomes in pediatric trauma patients. Materials and Methods: A retrospective study was conducted in which case records of 58 pediatric patients with blunt abdominal trauma were evaluated and tabulated for their SIPA scores only at the time of their initial presentation and categorized into two groups - normal SIPA and elevated SIPA. The primary outcomes were need for blood transfusion, need for any intervention, and need for emergency surgery, and the secondary outcomes were need for computed tomography (CT) scan, need for a ventilator, intensive care unit (ICU) stay, length of hospital stay, and mortality. Statistical methods were applied to find a relationship between elevated SIPA score and the primary and secondary outcomes. Results: An elevated SIPA score was noted in 27 (46%) patients. There was a significant relationship between elevated SIPA scores and patients needing blood transfusion (68.75%, n = 11) and length of hospital stay (10.48 ± 7.54 days). A significant relationship between elevated SIPA score and need for emergency surgery (54.54%, n = 6), need for a CT scan (56%, n = 14), and ICU stay (50%, n = 2) was not found. Conclusion: We have seen in our study that elevated SIPA scores at presentation are significantly related to need for blood transfusion and length of hospital stay. In more than half of the patients, elevated SIPA was associated with need for emergency surgery and requirement of CT scan, but it was statistically not significant. Therefore, assessment of this parameter can help in identifying such poor outcomes.

Role of Ultrasonography and Inflammatory Markers in Predicting Complicated Appendicitis.

Aim: The aim is to compare the diagnostic accuracy of laboratory investigations and ultrasonography (USG) in distinguishing complicated appendicitis (C-AA) from uncomplicated appendicitis (UC-AA). Materials and Methods: Forty-six children who underwent appendicectomy at our center between November 2018 and July 2020 were included. Based on intraoperative findings, they were divided into two groups - complicated (perforated, gangrenous, or associated with fecal peritonitis; n = 18) and UC-AA (n = 28). USG findings and inflammatory markers were compared in both groups at admission. Results: At admission, the mean values for total leukocyte count (TLC) (16090.56 vs. 11739.29 per mm3), high sensitivity C-reactive protein (hsCRP) (35.8 vs. 31.62 mg/L), and procalcitonin (PCT) (3.83 vs. 1.41 ng/mL) were significantly higher in C-AA. Visualization of a blind tubular aperistaltic structure was the only sonographic sign showing statistical significance - significantly lower in C-AA (50% vs. 90%). Independent predictors of C-AA were - duration of symptoms >48 h (odds ratio [OR] 6.3), free fluid/loculated collection in right iliac fossa (OR 3.75), TLC >11000/mm3 (OR 3.6), hsCRP >35 mg/L (OR 6.0), PCT >0.6 ng/mL (OR 4.02), and nonvisualization of appendix on USG (OR 8.33). Biochemical factors were sensitive (89%) and specific (55%) in differentiating C-AA from UC-AA but the addition of sonological parameters significantly improved the specificity of predicting complicated AA to 61% (P = 0.0036). Conclusion: Combining laboratory data with sonological findings significantly improves the predictive value for differentiating C-AA from UC-AA and can help decide operative approach and prognosticating.

Triple Diversion Technique in Complete Duodenal Transaction Following Blunt Trauma Abdomen: A Time-Tested Method in a Very Rare Injury.

Duodenal injury following blunt abdominal trauma is extremely rare in children and many times, it has delayed presentation, leading to increased morbidity and mortality. A patient with complete duodenal transaction is a surgical challenge and management involves the time of presentation and extent of visceral damage. A 10-year-old boy was brought with features of bowel perforation after road traffic accident and underwent emergency laparotomy which revealed complete transaction of duodenum at D1 and D2 and pyloroduodenal junction extending toward lesser curvature. Primary closure of pyloroduodenal junction and D1-D2 was done with omental patch along with triple tube decompression (cholecystostomy, gastrostomy, and jejunostomy). The patient had an uneventful recovery. Primary closure of disturbed ends with triple diversion is a safe approach in young children with complete duodenal transaction in absence of gross peritoneal contamination and early presentation.

Laparoscopic versus open appendicectomy for complicated appendicitis in children: A systematic review and meta-analysis.

BACKGROUND: Laparoscopic appendectomy (LA) is the preferred approach in uncomplicated appendicitis. However, in patients with complicated appendicitis (CA), the best approach is still unclear though laparoscopy is being increasingly preferred over open appendicectomy (OA) nowadays. AIM: To comprehensively review the current literature and compare the associations of LA and OA concerning various postoperative outcomes in order to determine the best approach for children with CA. METHODS: The PRISMA guidelines were adhered to and an electronic database search was extensively performed. Data analysis, including subgroup analysis of randomized-control trials, was performed using RevMan 5.3. Methodological and statistical heterogeneity, as well as publication bias of the included studies, were assessed. RESULTS: Four randomized controlled trials (266 LA versus 354 OA) and thirty-six case-controlled trials (2580 LA versus 3043 OA) were included in the analysis. Compared to OA, LA has a shorter length of stay, a lower rate of surgical site infection as well as a significantly lower overall complication rate. The rates of intraabdominal abscess formation, post-operative fever, pneumonia and ileus are similar in the two groups. So are the rates of readmissions and reoperations. LA was also shown to have a shorter time taken to oral intake and a lesser requirement of analgesics as well as intravenous antibiotics. Operative time for OA was found to be significantly shorter than that for LA. CONCLUSION: This meta-analysis objectively demonstrates that laparoscopy has a better overall complication profile compared to OA and should be the procedure of choice in children with complicated appendicitis.

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.

Anesthetic considerations in patients with cystic pulmonary adenomatoid malformations.

Congenital pulmonary adenomatoid malformation (CPAM) is a rare entity. The authors searched the US National Library of Medicine Database, EMBASE, Google Scholar, PubMed Central for anesthetic management in CPAM. The search was performed using the terms: congenital cystic adenomatoid malformation, congenital pulmonary adenomatoid malformation, CCAM, CPAM, anesthetic management. The prognosis of CPAM depends on timely diagnosis, presence of hydrops, degree of hypoplasia of remaining lung, and the size of the lesion. Symptomatic patients must be treated surgically and lobectomy is considered the gold standard. Anesthetic management of such cases is challenging as it involves thoracotomy or thoracoscopic lobectomy or cystectomy and can lead to sudden hemodynamic Collapse. Early extubation should be considered to avoid iatrogenic ventilator-induced bronchial stump dehiscence resulting from positive pressure ventilation.

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.

Intranatal Torsion of Polydactyly: A Rare Event.

Polydactyly is one of the most common anomalies of hand and/or foot. Postnatal torsion of pedunculated polydactyly is a well known complication but intranatal torsion has been infrequently described in published literature. Here, we describe a case of pedunculated ulnar polydactyly which was gangrenous at birth due to intranatal torsion. Controversies surrounding the management of narrow pedicled pedunculated polydactyly by traditional method of suture ligation at base are also discussed.

Angiomatosis: A Rare Vascular Proliferation of Head and Neck Region.

Angiomatosis is a diffuse vascular lesion which clinically mimics hemangioma or vascular malformation. It usually involves multiple tissues and is histopathologically characterised by proliferation of vessels of varying calibre intimately admixed with large amount of adipose tissue. Its surgical removal is very difficult because of its infiltrative nature. Therefore, a precise histopathological diagnosis is of utmost importance. It is usually seen in females in the first two decades and commonly involves lower extremities. Angiomatosis of head and neck region is very rare. Here we present a rare case of angiomatosis of the lower face involving right cheek and lip in a 4-year-old boy clinically diagnosed as hemangioma. Histopathological differential diagnosis of angiomatosis is also discussed.

Malignant urothelial carcinoma of urinary bladder in a young child: a rare case report.

We present a rare case report of a malignant, high-grade, urothelial bladder carcinoma in a 4-year-old boy. The tumor recurred quickly after resection. The recurrence was treated with partial cystectomy plus MVAC (methotrexate, vinblastine, Adriamycin, cisplatin). This is the first reported use of the MVAC regimen for a pediatric bladder tumor.

Bedside neonatal intensive care unit surgery- myth or reality!

Neonatal transport is associated with complications, more so in sick and unstable neonates who need immediate emergency surgery. To circumvent these problems, surgery in Neonatal intensive care unit (NICU) is proposed for these neonates. This article reviews the literature regarding feasibility of this novel concept and based on the generated evidence, suggest the NICU planners to always include infrastructure for this. Also neonatal surgical team can be developed that could be transported.