Association of Wilms Tumor with Crossed Fused Renal Ectopia in Children: A Case Report.

Introduction: Wilms tumor is the most common renal malignancy in children. The occurrence of Wilms tumor with various congenital genitourinary anomalies has been reported, particularly in horseshoe kidneys, hypospadias, disorder of sexual development, and double collecting system. However, Wilms tumor with crossed renal ectopia is a rare finding. Case Description: We are reporting a case report of Wilms tumor in a 3-year-old girl who presented with a huge left flank mass with cross-fused renal ectopia. After the initial workup and triphasic computed tomography scan of the chest, abdomen, and pelvis for confirmation of diagnosis and metastasis, the patient underwent image-guided tissue biopsy, followed by neoadjuvant chemotherapy, left radical nephrectomy with separation of fused right ectopic renal moiety, and adjuvant chemoradiation. Practical Implication: This report shows an association of Wilms tumor with cross-fused renal ectopia, a rare combination. Unusual findings on imaging with unilateral flank mass should be considered as part of the differential diagnosis for this rare finding. Adjuvant chemotherapy and modern imaging helped delineate the anatomy and ease the surgery for safe resection, improving the overall outcome.

Emergency Angioembolization for Life-Threatening Hemorrhage in Wilms Tumor.

Introduction: Renal artery embolization has been used in a palliative fashion for symptomatic relief of hematuria or flank pain in unresectable renal cell carcinoma in adults. There is limited data on the use of embolization for actively bleeding and unresectable tumors in the oncological pediatric population. Case Description: A previously healthy 5-year-old boy with no significant past medical or surgical history presented to the clinic with gradually worsening abdominal distension associated with occasional abdominal pain, gross hematuria, and lethargy for four months. Diagnostic investigations showed an 18-cm left-sided metastatic (pulmonary) renal tumor (Wilms), which was deemed unresectable on imaging. Treatment was planned according to the SIOP-RTSG protocol. However, he became hemodynamically and vitally unstable with acute, sudden distension of the abdomen on the left side after the first cycle of chemotherapy. Imaging showed active bleeding from an inferior branch of the left renal artery. Selective angioembolization was done, and chemotherapy was reinitiated with a patent left main renal artery. Following the fourth cycle of chemotherapy, he developed hemodynamic instability and abdominal pain; imaging revealed the resolution of pulmonary nodules and bleeding from the left renal artery (main); this was again embolized, and the patient was stabilized. The patient was operated on after optimization, and a complete resection of the mass was done with negative margins. On six months follow-up, he is well. Practical Implications: To the best of our knowledge, this is the first case where angioembolization has been done in conjunction with neoadjuvant chemotherapy to downsize a Wilms tumor to achieve favorable outcomes. Continued research efforts are necessary to optimize strategies and improve the prognosis for pediatric patients, and this case is one of the prime examples.

Testicular tumours in children: a single-centre experience.

Introduction: Testicular tumours in childhood have diverse characteristics for different age ranges. This study aimed to describe the pattern, presentation and outcomes of primary testicular tumours in a paediatric population. Methods: A retrospective study was conducted from January 2010 to December 2020 on children (≤18 years) with a diagnosis of primary testicular tumour. Baseline demographics, clinical characteristics, pathology, treatment and outcomes of these patients were analysed. The data were entered into IBM SPSS Statistics version 20.0. Chi-square test and Fisher's exact test were applied to find the statistical significance, which was set at P value ≤ 0.05. Results: The study included 115 males, with 85 (73.9%) patients in the prepubertal age range with a mean age of 2.53 ± 2.06 years and 30 (26.1%) patients in the postpubertal group with a mean age of 15.73 ± 1.25 years. Yolk sac tumour was the most common (62.6%) histological subtype. Majority (46.1%) of patients had stage I disease on presentation, while 29.6% had stage IV disease. All patients underwent upfront high inguinal radical orchiectomy, which was followed by platinum-based adjuvant chemotherapy in 67% of the patients. The five-year event-free survival and overall survival for all patients were 75% and 91%, respectively. Conclusion: Primary testicular tumours follow a bimodal age distribution pattern. Majority of patients can be cured with platinum-based chemotherapy despite having advanced disease at presentation.

Tracheo-Cutaneous Fistula: A Rare Presentation Due To A Forgotten Rubber Band In Neck Of A Young Girl.

The presence of foreign bodies in a chronic wound is a well-known phenomenon. The common locations are soft tissues of the wrist and neck, gastro-intestinal foreign objects, foreign objects in the ear and in the Uro-genital tract. aero digestive tract and chest. Retained foreign bodies in soft tissues become reason for formation of sinus which can be superimposed by infection. Most of foreign bodies in soft tissues remain un-detected, becoming the reason for delayed diagnosis. Some of the foreign bodies are inert and stay in the body tissues for prolonged time without causing any deleterious effect but few of them erode into surrounding tissues and cause prolonged illness. Tracheo-cutaneous fistula secondary to a foreign body in a child is rarely reported. In this case, the patient had retained foreign body in her neck for almost eight years which remained un diagnosed while being treated for various types of skin disease.

Review of Perioperative Care Pathway for Children With Renal Tumors.

Introduction Wilms tumor is the most common (90%) renal tumor in children. With the recent advances survival rate approaches 90%. This study was designed to identify factors associated with early recovery and hospital discharge, a step forward in the future development of early recovery after surgery (ERAS) protocol in children.  Methods A retrospective cohort study was conducted from May 2020 to February 2022 among children up to the age of 18-years with a diagnosis of primary malignant renal tumors who underwent radical nephrectomy. Baseline parameters (preoperative), intraoperative, and postoperative components were analyzed. A univariable and multivariable logistic regression model was used to identify the factors leading to early discharge. The data were entered into SPSS version 20 (IBM Inc., Armonk, USA), and a p-value ≤0.05 was statistically significant. Results A total of 44 patients with malignant renal tumors were managed with a mean age at diagnosis of 4.06±3.00 years. Twenty-six (59.1%) were male and 18 (40.9%) were female. All the patients received neoadjuvant chemotherapy. Wilms tumor was the most common pathology found in 41 (93.1%) patients; 19 (43.0 %) had stage I, 21 (47.7%) stage II, and four (9.1%) stage III disease. Thirty-four (77.3%) patients had a nasogastric tube placed postoperatively. Median blood loss (BL) was 40 ml (range: 5-250), and the median operative time was two hours (range: 1-4). The median postoperative day to mobilization was one day (range: 1-3), to clear liquids was two days (range: 1-3), and advanced to regular diet was three days (range: 2-5). The median postoperative day of discharge (from surgery to discharge) was four days (range: 2-7), with 31 (70.5%) patients discharged early. Conclusion Our findings indicate that early removal of tube, mobilization, and re-feedings were significantly associated with the early hospital discharge, while the other analyzed factors were not statistically significant. Furthermore, our findings are important in the future development and implementation of ERAS protocol in pediatric oncological resections.

A study of ovarian lesions in pre-menarche girls.

OBJECTIVE: To analyze various clinical presentations and the surgical management of ovarian pathologies in pre-menarche girls. DESIGN: Case series. PLACE AND DURATION OF STUDY: Surgical Unit B, National Institute of Child Health, Karachi, from September 2002 to August 2004. PATIENTS AND METHODS: Case records of all the pre-menarche girls child having an ovarian pathology and managed during the study period were reviewed. Age of the patients, presenting symptoms, investigations performed, surgical approaches, operative findings and final histological diagnosis were noted. Ovarian lesions were classified according to WHO criteria.System proposed by the Children's Cancer Group and the Paediatric Oncology Group was used for staging of tumors. Following resection, patients with malignant tumors were followed-up by oncologist. RESULTS: There were 18 patients with ovarian lesions. Age range of patients was from day 1 to 13 years. Mass in abdomen was the most common presenting symptom (60% patients ) followed by pain (50%). Right ovary was involved in 12 (55%) cases and left in 6 (45%). Six patients had non-tumorous cysts, 3 had benign tumors and 9 were with malignant lesions. Of the non-tumorous cysts, 3 patients had simple follicular cysts and 2 were hemorrhagic cysts. There were 2 patients with benign teratoma and one with dermoid cyst. Four patients had malignant teratoma, 4 dysgerminoma and one yolk sac tumor. Malignant tumors were all of stage IA according to Paediatric Oncology Group staging. CONCLUSION: Ovarian tumors are rare in paediatric age group especially in pre-menarche girls. Except one, all malignant tumors were found in patients above 7 years of age. Benign (tumorous and non-tumorous) and malignant lesions occurred with equal frequency in pre-menarche girls in this study.

Complications / problems of colostomy in infants and children.

OBJECTIVE: To determine the frequency of complications/problems occurring with construction of colostomy in infants and children. DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: Surgical Unit B, National Institute of Child Health, Karachi, from September 2001 to March 2003. PATIENTS AND METHODS: All the patients admitted in the unit who required colostomy as part of their management were included in the study. The patients who were operated upon previously or operated elsewhere and referred after having colostomy, were excluded. Problems associated with colostomy construction like skin excoriation and chronic blood loss were also recorded. Chi-square test of proportion was used to determine the p-value. RESULTS: There were 121 patients. Most of the patients were operated due to anorectal malformations (n=71) and Hirschsprung's disease (41). Complications/problems related to colostomy occurred in 67.7% patients. The most common problem was skin excoriation. Second in rating was chronic blood loss from stoma. Prolapse of stoma was more common in transverse loop colostomies. Divided colostomies had higher number of complications as compared to loop colostomies, similarly, transverse colostomies had high number of complications but in both the cases difference was not statistically significant. Four (3.3%) patients died. CONCLUSION: Construction of colostomy in paediatric patients carries high frequency of complications/problems and requires careful technique. The role of stoma care clinic and enterostomal therapist can be instrumental in preventing problems associated with colostomy.

Ileal duplication cyst causing recurrent abdominal pain and melena.

Alimentary tract duplications are rare congenital anomalies. The presentation depends on their anatomical location, size and other characteristics. The most common variety is small bowel cystic duplication. We report a case of an eight years old girl who presented with recurrent abdominal pain and melena. Radioisotope technetium scan showed increased uptake of tracer in right lower abdomen and a diagnosis of Meckel's diverticulum made. At surgery a cystic, communicating, ileal duplication found which was resected along with adjacent gut. It is thus reiterated that while investigating children with recurrent abdominal pain and melena, gut duplications must be included in the differential diagnosis.