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.

Tracheoesophageal Fistula with Bilateral Facial Cleft: A Rare Occurrence.

Commissural or lateral facial cleft (macrosomia), classified at Tessier number 7 craniofacial clefts, is a rare congenital anomaly usually associated with deformities of other structures developed from the first and second branchial arches. It affects the esthetics and functional aspect of the oral cavity. Bilateral transverse cleft occurring alone is uncommon and it's with tracheoesophageal fistula (TEF) has not been reported to the best of our knowledge. We report a case of esophageal atresia (EA) and TEF with macrosomia. EA was repaired, and the patient was discharged on full feeds. He is awaiting cleft repair.

Pediatric PseudoTumoral Hepatic Tuberculosis. A Great Mimicker!!

Pediatric liver masses are rare and difficult to treat. Common liver masses in children include hepatoblastoma, hemangiomas, liver abscesses, and hydatid disease. Isolated liver tuberculosis (TB) is rare in children and can have variable clinical presentations. We report a child with isolated liver TB masquerading as a liver tumor.

Correlation of Urinary Biomarkers (Interleukin-6, Transforming growth factor-ß, E-Cadherin, and MCP-1) with Conventional Parameters of Disease Progression in Patients of Posterior Urethral Valves: A Comparative Analysis.

Aims: Posterior urethral valves (PUV) are the leading cause of end-stage renal disease in boys. The study aimed to look at the ongoing renal damage and profibrotic activity by measuring the levels of Interleukin-6 (IL-6), Transforming growth factor-ß (TGF-ß), E-cadherin, and Monocyte Chemoattractant Protein-1 (MCP-1) and observing trends in subsequent follow-ups and at the same time correlating them with the established parameters of disease progression. Materials and Methods: This prospective study included 36 consecutive patients of PUV, managed over a period of 18 months. IL-6, TGF-ß, E-cadherin, and MCP-1 were measured in urine samples at the time of admission, pre-fulguration and 3 months' and 9 months' post fulguration. The observed values were correlated with the conventional parameters used in clinical practice. Results: All the biomarkers showed statistically significant trends when these values were compared on admission, postoptimization and 3 months' and 9 months' postfulguration. None of the biomarkers showed a significant correlation with renal function tests. E-Cadherin and TGF-ß showed a positive and a negative correlation with ultrasonography (USG) kidney, ureter, and bladder (KUB) respectively. E-Cadherin showed a positive correlation, whereas IL-6 and TGFß showed negative correlation respectively with micturating cystourethrogram (MCUG). IL-6 showed statistically a significant negative correlation with dimercapto succinic acid (DMSA). MCP-1 did not show any significant correlation with USG KUB, MCUG and DMSA. Conclusion: This study concludes that E-Cadherin, IL-6, TGF-ß can be promising urinary biomarkers for early detection of the ongoing renal damage in patients of PUV following valve fulguration. MCP-1 may have more complex interactions, with inflammatory markers; which warrants further research.

A Fix for a Scalp Varix! A Rare Case of Cirsoid Aneurysm in a Child.

Cirsoid aneurysm (CA) is a rare arteriovenous fistula of the scalp. There exists scant literature on the incidence and approach to CA in children. We describe a case of CA in a 7-year-old boy which was diagnosed by angiography and managed with angiographic embolization followed by surgical excision.

Oral Atenolol versus Propranolol in the Treatment of Infantile Hemangioma: A Systematic Review and Meta-Analysis.

Background: Infantile hemangioma (IH) is the most common benign vascular tumor of infancy. Propranolol is considered first-line therapy for IH. However, it is associated with side effects. Therefore, there was a need for alternative therapy. Atenolol, a selective b1-blocker may be free from such side effects. Hence, the present study aims to develop a more accurate estimate of the safety and efficacy of atenolol compared to propranolol in the treatment of IH. Methodology: A search of various literature databases (PubMed, Embase, Ovid, Scopus, Cochrane Central, CINAHL, Web of Science, and Google Scholar) was done to identify studies which compared propranolol versus atenolol in the treatment of IH. The combined odds ratio along with corresponding 95% confidence intervals (CIs) were evaluated using a fixed-effects model. Results: A total of 300 articles were screened of which five studies including 116 patients in atenolol arm and 138 patients in the propranolol arm were analyzed. Atenolol was comparable to propranolol in terms of efficacy as no significant difference was seen between both the treatment arms in terms of hemangioma activity score (mean difference 0.25 [95% CI;‒0.21, 0.71]) and complete response (odds ratio [OR] =0.43; 95% CI; 0.17, 1.11; P = 0.08,). Atenolol therapy was better than propranolol in terms of safety, i.e., serious/potentially serious side effect, (OR = 0.11; 95% CI; 0.02, 0.51; P = 0.005) and wheezing/bronchial hyperreactivity (OR = 0.11; 95% CI; 0.02, 0.51; P = 0.005). Conclusion: The present meta-analysis provides evidence that atenolol has got a comparable efficacy and better safety profile with propranolol.

Recurrent lupus enteritis in a child-chase the calm before the storm.

Systemic lupus erythematosus (SLE) is a complex multisystemic autoimmune disease. Lupus enteritis (LE), one of the less commonly described manifestations of childhood SLE, presents with relatively nonspecific clinical and laboratory features. In addition, recurrent episodes of LE occurring in temporal proximity are rare in children. Presence of disease activity at other sites (which may not be seen universally) supports the diagnosis of LE in an appropriate setting. Because of its potential role to cause ischemic complications, early recognition and prompt treatment are necessary for a good outcome. Herein, we describe a child with recurrent LE with an interval of about 3 months between the first and the second episode. The first episode correlated with systemic disease activity and bowel thickening was noted on abdominal ultrasonography. This episode was successfully managed with intravenous methylprednisolone pulse therapy. Conversely, the second episode was not associated with significant clinical and laboratory evidence of disease activity at other sites and the initial abdominal ultrasonography was non-contributory. Diagnostic and therapeutic delays, hence, led to the development of fatal complications. We highlight that a high index of suspicion of LE and a timely aggressive treatment is imperative for optimal outcomes even in rare pediatric cases of recurrent LE that may have normal imaging findings initially and may not be associated with systemic lupus erythematosus disease activity index (SLEDAI).

Fungaemia due to rare yeasts in paediatric intensive care units: A prospective study.

BACKGROUND: Considering the emergence of fungaemia due to rare yeasts at our centre, we performed a systematic epidemiologic study on fungaemia due to rare yeast. OBJECTIVES: We undertook the present prospective observational study to explore the epidemiological features and clinical characteristics of fungaemia due to rare yeasts in paediatric ICUs at our centre. METHODS: The successive yeasts isolated from blood at our PICUs during December 2017 through March 2019 were identified by molecular methods. Fungaemia due to yeasts other than C. albicans, C. tropicalis, C. glabrata, C. krusei and C. parapsilosis was categorised as rare yeasts. Antifungal susceptibility testing of the yeast isolates was performed as per clinical and laboratory standards institute (CLSI) guidelines. We also compared different clinical parameters of fungaemia due to common versus rare yeasts, and rare yeasts in neonates versus non-neonates. RESULTS: During the study period, 212 yeast isolates were obtained from 159 patients at PICUs of our hospital, and 127 isolates from 98 patients (61.6%) were categorised as rare yeasts. Neonates acquired fungaemia significantly earlier after ICU admission than non-neonates (median:4 vs 6 days; p = .005). Regarding epidemiology study of rare yeast fungaemia, Wickerhamomyces anomalus (43.8%) and Candida utilis (40.8%) were common isolates; surgical intervention and gastrointestinal disease were significantly associated; overall, azole, echinocandin and amphotericin B resistance was at 9.1%, 1.02% and 1.02%, respectively; overall mortality was 65.3%. CONCLUSIONS: The emergence of rare yeasts especially W. anomalus and C. utilis causing fungaemia in our children demands urgent attention to control the spread.

Extrapulmonary Pleuropulmonary Blastoma in a 3-Year-Old Child: A Case Report and Review of Literature.

Pleuropulmonary blastomas (PPBs) are very rare, highly aggressive, dysembryonic neoplasms of thoracopulmonary mesenchyme. These have been reported in the pediatric population and account for only 0.5%-1% of all primary malignant lung cancers. They normally arise from lung tissue, however rarely the parietal pleura may be the tissue of origin (extra pulmonary PPB) which are extremely rare. Common age of presentation is three to 4 years. The prognosis is poor with distant metastasis to central nervous system and bone with survival rate of approximately 42.9% at 5 years. They are managed by aggressive multimodal therapies including surgery and chemotherapy. We report a case of a 3-year-old male child with Type 2 PPB of the left hemithorax, managed by surgical excision of the mass and adjuvant chemotherapy.

Growth and Development Assessment of Children (1-5 Years) Operated for Tracheoesophageal Fistula/Esophageal Atresia: A Case Control study.

INTRODUCTION: Among children, esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is one of the major and common congenital anomalies. It is a life-threatening emergency and at birth may be associated with three C's coughing, choking, and cyanosis. It requires surgical interventions in the early neonatal period. The postsurgical period is associated with poor growth which can be developmental outcomes particularly in the first 5 years of life and attributed to postsurgical complications. The aim of the study is to assess and compare the growth and development of the children (1-5 years) operated for TEF/EA attending Pediatric Surgery OPD/admitted inwards at APC, PGIMER, Chandigarh versus healthy controls. MATERIALS AND METHODS: A case-control study was conducted on age-matched 40 children aged between 1 and 5 years operated for TEF/EA and healthy controls. The sampling technique for cases was total enumeration and for controls was purposive sampling. Tools used were socio-demographic sheets of children, clinical profile of children, Trivandrum Development Screening chart, and Vineland Social Maturity Scale for Indian adaptation. RESULTS: Majority 33 (82.5%) of children had distal TEF and more than two-third 28 (70%) have undergone primary repair. More than one-third 14 (35%) had a respiratory infection, 12 (30%) anastomosis leakage and 6 (15%) had Gastroesophageal reflux (GER) as one of the early and late postoperative complications. More than one-fourth 11 (27.5%) of TEF/EA operated children had less weight, 11 (30%) had less height and 16 (40%) had less weight for height for their reference age. A significant difference was found for height for age, weight for height, and social maturity among children who had TEF repair as compared to their healthy counterparts. CONCLUSION: Growth monitoring reflected (more than one-fourth of children were underweight and stunted while more than one-third were wasted) and showed development delay in TEF/EA operated children as compared to healthy controls.

Antenatally Detected Multiple Mediastinal Enterogenous Cysts in a Newborn Presenting with Severe Cardio-Respiratory Compromise and Early Life-Saving Management.

Posterior mediastinal enterogenous cyst is a rare entity in neonate. The neonate can present with severe cardio-respiratory compromise in the form of respiratory distress, shock, cardiac failure or arrhythmia soon after birth which may require immediate surgical intervention. Antenatal screening can demonstrate the cystic mass in fetus early and can help in quick postnatal management. Multidisciplinary management with aspiration of the cysts was life-saving in the present case.

Bladder Mass Masquerading as Eosinophilic Cystitis in a Child: When to Think Beyond Malignancy?

Eosinophilic cystitis is a rare inflammatory disease in the pediatric population with varied presentations. Diagnosis requires a high index of suspicion and cystoscopy with biopsy of the bladder mass. There are no standard treatment guidelines, however, these patients usually respond with medical management, but recurrence is a possibility. We present a case of eosinophilic cystitis in a 6-year-old boy who presented with lower urinary tract symptoms, gross hematuria, and bladder mass.

Modified Duhamel's Two-Staged Procedure for Hirschsprung's Disease: Further Modifications for Improved Outcomes.

AIMS AND OBJECTIVES: We studied the short- and long-term outcomes and quality of life (QOL) in patients undergoing a two-staged modified Duhamel's procedure for Hirschsprung's disease. MATERIALS AND METHODS: Patients who had undergone this modified procedure, with initial Hartmann's procedure based on contrast enema, followed by bowel preparation and low colo-anal anastomosis below the dentate line were included. The patient who underwent this procedure over 10 years with a minimum 2-year follow-up were analysed based on an interview-based questionnaire. RESULTS: Of the 152 patients, 69 responded. Mean age at the time of interview was 7.72 ± 3.04 years with mean follow-up of 4.9 years (2-11 years). Perineal excoriation and soiling was present in 60.9% and 36.2% of patients initially which reduced to 0 and 4.3% by the end of 2 years. In the first 6 months, postoperative period, 15.9% of patients had constipation and 78.2% had altered stool consistency. Good fecal continence score was present in 97.1% of patients in the long term. About 95.4% had good QOL scores. There was no mortality in this series. CONCLUSIONS: Although short-term outcomes showed altered bowel function, soiling, and perineal excoriation, this improved significantly in the long term, with good QOL scores in the majority.

Intraoperative Dilemmas in Polyorchidism: To Pex or not to Pex!!

Supernumerary testis or polyorchidism is a rare congenital anomaly. It is often associated with processus vaginalis anomalies and with increased risk of malignancy and infertility. The approach to management has changed over time, with improvements in imaging techniques allowing surveillance to replace surgical excision or exploration and biopsy. In this study, two patients were managed with orchidopexy and have had a close follow-up of 2 years.

Pleomorphic Lipoma of the Neck in an Infant: A Rare Clinical Entity.

Lipomas are rare in the pediatric age group. A 10-month-old male child presented with an asymptomatic neck mass which was evaluated and excised completely. Histopathology was consistent with pleomorphic lipoma, not previously reported in children.

Factors affecting quality of life in early childhood in patients with congenital hydrocephalus.

BACKGROUND: Predicting the outcome of congenital hydrocephalus in early infancy and childhood is difficult. Various consequences of hydrocephalus like ophthalmic, audiometric, musculoskeletal, and the developmental abnormalities play a complex role. We analyzed the quality of life of these patients in early life. METHODS: A prospective multispecialty assessment of the patients with congenital hydrocephalus, who had undergone ventriculoperitoneal shunt in the Pediatric Surgery unit of a tertiary care hospital, was carried out and analyzed. RESULTS: There were 24 boys and 6 girls. Eighty-three percent were operated before the age of 3 months. Mean age at follow up was 6 years (3-9 years). Shunt complications were seen in 53% (16) of the patients, out of which 68.75% had shunt blocks. In 70% (21) of the patients, the ventricle to hemisphere ratio (VHR) was between 51 and 70%, 20% (6) had a VHR of more than 70% and only 3 patients had a VHR between 40-50%. Audiological and ophthalmic problems were seen in 20 and 66% of the children, respectively. Musculoskeletal abnormalities were present in 36% of the patients. Developmental profile showed 44% being normal, 30% retarded, and 26% were borderline cases. The schooling had started in 56.6%, out of which only one child went to a special school. CONCLUSIONS: A multitude of problems associated with congenital hydrocephalus should be diligently diagnosed, aggressively followed and intervened with in the early years of life. Preschool management well before the peer interaction should optimize the social integration and improve the quality of life in these patients.

Cytomorphologic and immunocytochemical characterization of pediatric pleuropulmonary blastoma with a comprehensive review of the literature.

INTRODUCTION: Pleuropulmonary blastoma (PPB) is a rare, aggressive, primary intrathoracic malignancy typically seen in infancy and early childhood. Accurate distinction from congenital cystic lung lesions is crucial due to significant prognostic and therapeutic differences. Cytologic features have rarely been described. Establishing a cytodiagnosis is challenging owing to its rarity, lack of awareness, and multiple morphologic mimics. MATERIALS AND METHODS: This was a retrospective study conducted over 8 years. The histopathology and cytopathology databases were searched for all pediatric PPB cases. The corresponding cytologic samples were reviewed to identify characteristic features that can help distinguish PPB from its mimics. RESULTS: There was a total of six cases of pediatric PPB reported during the study period. Of these, four (66.7%) presented as intrathoracic, and two (33.3%) as pleural-based masses. Cytology smears showed discretely scattered and perivascular arrangements of round-oval tumor cells with background eosinophilic stromal material. The tumor cells were mildly pleomorphic (n = 3) with round nuclei, fine chromatin, inconspicuous nucleoli, and scanty cytoplasm; however, three cases showed marked anaplasia, and one each showed necrosis and rhabdoid differentiation. On immunocytochemistry (4/6), these were positive for vimentin and desmin and negative for WT1, chromogranin, SALL4, cytokeratin, CD45, and CD99. FISH (1/6) did not show N-Myc amplification. CONCLUSIONS: Knowledge of the characteristic cytomorphological and immunocytochemical features of PPB is vital to establish a prompt and accurate cytodiagnosis with appropriate clinicoradiologic correlation.