Canal of nuck hernia with uterus and ovary presenting as inguinal swelling in a female infant.

Canal of nuck hernia is rarely reported in pediatric population. We report one such case of a canal of nuck hernia in a 2-month-old girl containing uterus, ovary and small bowel diagnosed on ultrasonography, and which was later confirmed and treated surgically.

A higher tumor volume and undernutrition at diagnosis adversely affect the survival of children with Wilms tumor: A study of 200 patients.

BACKGROUND: Distinct prognostic factors for Wilms tumor (WT) in low- and middle-income countries need identification. METHODS: Retrospective study of patients with WT managed by the International Society of Pediatric Oncology (SIOP) approach for over 11 years (2005-2016) at a single center in Chandigarh, India. RESULTS: The study included 200 patients (median age: 33.5 months). The tumor stage (SIOP) distribution included stage I (30%), II (36%), III (14%), IV (17%), and V (3%). The histology-risk groups were low (8%), intermediate (84%), and high risk (9%). At diagnosis, 68 out of 190 (36%) patients were underweight. The median tumor volume at diagnosis was 481 ml (interquartile ratio [IQR]: 306.9, 686.8, n = 146). Following neoadjuvant chemotherapy, it reduced to 110 ml (IQR: 151.2, 222, n = 77). Treatment was abandoned in 20.5% of the patients. Treatment-related mortality occurred in 13 of 179 (7.2%) patients. Relapse occurred in 26 of 158 (16.5%) patients. The 3-year overall survival (OS) and event-free survival (EFS) of patients who completed therapy were 78.3 and 72%, respectively. The stage (p = .013) and histology (p = .023) influenced OS. A lower OS in stage II (75.4%) versus stage III disease (83.7%) suggested understaging. Patients with a higher tumor volume at diagnosis (p = .005; odds ratio [OR]: 0.99; 95% confidence interval [CI]: 0.99-1.00) or a lower weight-for-age z-score (p = .002; OR: 1.68; 95% CI: 1.21-2.33) had an increased risk of death or relapse. CONCLUSIONS: The 3-year OS and EFS of children who completed therapy were 78.3 and 72%, respectively. A higher tumor volume and lower weight-for-age z-score at diagnosis were identified as distinct adverse prognostic factors. A likely suboptimal lymph node assessment (intraoperative and histopathology) contributed to the understaging of stage III to II disease and reduced survival.

Screening of renal anomalies in first-degree relatives of children diagnosed with non-syndromic congenital anomalies of kidney and urinary tract.

BACKGROUND: Non-syndromic congenital anomalies of kidney and urinary tract (CAKUT) are usually sporadic in nature but familial clustering of cases have been observed suggesting a genetic predisposition to this condition. We aimed to determine the frequency and pattern of renal anomalies in first-degree relatives of children with non-syndromic CAKUT. METHODS: We screened all the first-degree relatives of children with CAKUT. A total of 149 first-degree relatives, belonging to 62 families were screened with ultrasonography. RESULTS: A renal anomaly was detected in 9 out of the 62 families. Two of these nine families had identical anomalies (child and a parent) indicating single-gene disorders with possible autosomal dominant inheritance, while the rest of families had a non-identical anomaly. The anomalies detected in the first-degree relatives were renal hypodysplasia (n = 2), multicystic dysplastic kidney (n = 3), pelviureteric junction obstruction (n = 2) and mild hydronephrosis (n = 2). The incidence of a sonographically detected anatomic renal anomaly in first-degree relatives of children with CAKUT was found to be 6.0%. Familial cystic kidney disease was found in two out of the 4 families with cystic kidney disease. CONCLUSION: Significant renal anomalies were identified in first-degree relatives of children with non-syndromic CAKUT and hence, attempts must be made to screen the family members of children with non-syndromic CAKUT.

Prescription practice and clinical utility of chest radiographs in a pediatric intensive care unit: a prospective observational study.

BACKGROUND: Chest radiograph (CXR) prescribing pattern and practice vary widely among pediatric intensive care units (PICU). 'On demand' approach is increasingly recommended as against daily 'routine' CXRs; however, the real-world practice is largely unknown. METHODS: This was a prospective observational study performed in children younger than 12 years admitted to PICU of a tertiary care teaching hospital in India. Data were collected on all consecutive CXRs performed between December 2016 and April 2017. The primary outcome was to assess the factors that were associated with higher chest radiograph prescriptions in PICU. Secondary outcomes were to study the indications, association with mechanical ventilation, image quality and avoidable radiation exposure. RESULTS: Of 303 children admitted during the study period, 159 underwent a total of 524 CXRs in PICU. Median (IQR) age of the study cohort was 2 (0.6-5) years. More than two thirds [n = 115, 72.3%] were mechanically ventilated. Most CXRs (n = 449, 85.7%) were performed on mechanically ventilated patients, amounting to a median (IQR) of 3 (2-5) radiographs per ventilated patient. With increasing duration of ventilation, the number of CXRs proportionately increased in the first two weeks of mechanical ventilation. In non-ventilated children, about two thirds (68%) underwent only one CXR. Majority of the prescriptions were on demand (n = 461, 88%). Most common indications were peri-procedure prescriptions (37%) followed by evaluation for respiratory disease status (24%). About 40% CXRs resulted in interventions; adjustment in ventilator settings (13.5%) was the most frequent intervention. In 26% (n = 138) of radiographs, image quality required improvement. One or more additional body part exposure other than chest and upper abdomen were noted 336 (64%) images. Children with > 3 CXR had higher PRISM III score, more often mechanically ventilated, had higher number of indwelling devices [mean (SD) 2.6 (1.2) vs. 1.7 (1.0)] and stayed longer in PICU [median (IQR) 11(7.5-18.5) vs. 6 (3-9)]. CONCLUSION: On demand prescription was the prevalent practice in our PICU. Most non-ventilated children underwent only one CXR while duration of PICU stay and the number of devices determined the number of CXRs in mechanically ventilated children. Quality improvement strategies should concentrate on the process of acquisition of images and limiting the radiation exposure to unwanted body parts.

Flexible Fibreoptic Bronchoscopy and Bronchoalveolar Lavage for Confirmation of Pulmonary Hydatid Disease in Children: A Case Series.

Hydatid disease of the lungs is common in endemic regions. It can be suspected clinically by non-specific respiratory symptoms in children living in endemic regions, especially when they are close to sheep or dogs. Chest imaging X-ray or computed tomography may show characteristic cysts in some cases, but typical findings are absent in many children. Hydatid serology may contribute to the diagnosis, but does not have sufficient sensitivity for pulmonary cysts. Thus, there is no confirmatory diagnostic test, other than surgical excision and histopathologic examination. Hence, there is a need for more reliable diagnostic tests. We present a series of children, both with and without suspected pulmonary hydatid, wherein flexible fibreoptic bronchoscopy (FFOB) performed under conscious sedation, revealed hydatid membranes in the airways. Bronchoalveolar lavage (BAL) analysis revealed hydatid in most of them. Thus the diagnosis could be confirmed even before surgical excision of cysts was performed. We propose that FFOB with BAL could be useful to confirm the diagnosis of pulmonary hydatid in children. This will be particularly helpful in children without characteristic radiological or serological findings. To the best of our knowledge, this is a completely novel approach to the condition with potential to alter the diagnostic paradigm Lay summary Hydatid disease of the lungs is commonly encountered in endemic regions. However, there is no confirmatory diagnostic test for pulmonary hydatid cyst, other than surgical excision and histopathologic examination. Imaging including chest X-ray and computed tomography may not be typical, especially in complicated cysts and hydatid serology does not have a satisfactory sensitivity for diagnosing lung cysts. Thus, there is a need for more reliable diagnostic tests. We present a series of children, both with and without suspected pulmonary hydatid, wherein flexible fibreoptic bronchoscopy (FFOB) under conscious sedation, revealed hydatid membranes in the airways. Bronchoalveolar lavage (BAL) analysis confirmed hydatid in most of them. We propose FFOB with BAL as a useful diagnostic modality to confirm pulmonary hydatid in children, prior to surgical excision. To the best of our knowledge, this is a completely novel approach to the condition with potential to alter the diagnostic paradigm.

Survival in patients with high-risk neuroblastoma treated without autologous stem cell transplant or dinutuximab beta.

The majority of patients with high-risk neuroblastoma (HR-NB) in low- and middle-income countries (LMIC) do not have access to autologous stem cell transplant (ASCT) and dinutuximab. Consolidation with nonmyeloablative chemotherapy is not well-defined, and the outcomes are variable. We report a single-center outcome of patients with HR-NB, treated with nonmyeloablative consolidation. A tabulated compilation of similar reports is included. A retrospective chart review of patients with HR-NB was performed from January 2009 till June 2016. Patients were treated on the backbone of HR-NBL1/SIOPEN protocol. Treatment included induction with rapid-COJEC, surgery, followed by consolidation. Consolidation involved 4 cycles of topotecan, vincristine, and doxorubicin (TVD) instead of ASCT. Infusion of vincristine and doxorubicin were modified for ease and to enable administration in the clinic. Subsequent treatment included radiotherapy to the primary tumor and differentiation therapy with isotretinoin. Over 7½ years, 28 patients with HR-NB were treated. Two (7%) patients had therapy-related mortality. A relapse or disease progression occurred in 11 (39%) patients at a median duration of 17 months (IQR: 5, 18). Treatment abandonment was observed in 4 (14%) patients. The median follow-up of disease-free patients was 49 months (IQR: 45, 79). Patients with relapse were not treated further. A 4-year EFS of 29.3% was observed when 4-cycles of TVD were administered instead of ASCT in patients with HR-NB. The study and the review will aid decision-making for care of patients in LMIC while considering the options of treatment for HR-NB if access to ACST and dinutuximab is lacking.

Autologous stem cell transplant for high-risk neuroblastoma: Achieving cure with low-cost adaptations.

BACKGROUND: The majority of patients in low- and middle-income countries (LMIC) are unable to receive optimal therapy, including autologous stem cell transplant (ASCT) for high-risk neuroblastoma. Management is intensive and multidisciplinary; survival is often poor. We report a single-center outcome of high-risk neuroblastoma, with adaptations optimized for LMIC. PROCEDURE: The study was retrospective. Patients were treated on the backbone of the high-risk neuroblastoma study-1 of SIOP-Europe (HR-NBL1/SIOPEN) protocol with ASCT. Adaptations incorporated to decrease cost, requirement for inpatient admission, infections, and faster engraftment included (a) optional outpatient administration for rapid-COJEC, (b) two sessions of stem-cell apheresis, (c) storing stem cells at 2-6°C without cryopreservation for up to 7 days, (d) no central lines, (e) no antibacterial/antifungal/antiviral prophylaxis, (f) omitting formal assessment of cardiac/renal/pulmonary functions before ASCT, and (g) administration of pegylated granulocyte colony-stimulating factor on Day +4. RESULTS: Over 5 years 9 months, 35 patients with high-risk neuroblastoma were treated. Rapid-COJEC was administered over a median duration of 80 days (interquartile range: 77, 83). Conditioning regimen included melphalan (n = 7), oral busulfan-melphalan (Bu/Mel; n = 6), or intravenous Bu/Mel (n = 22). The median viability of stem cells stored for 6 days (n = 28) was 93% (range: 88-99). Two (5.7%) patients had ASCT-related mortality. The 3-year overall and event-free survival was 41% and 39%, respectively. A relapse occurred in 20 (57%) patients. Treatment abandonment was observed in one (3%) patient. CONCLUSIONS: Administration of therapy in a disciplined time frame along with low-cost adaptations enables to manage high-risk neuroblastoma with low abandonment and an encouraging survival in LMIC. Stem cells can be stored safely without cryopreservation for up to 7 days.

Diagnostic accuracy of 3-T lung magnetic resonance imaging in human immunodeficiency virus-positive children.

BACKGROUND: More than 70% of human immunodeficiency virus (HIV)-positive children sustain respiratory diseases in their lifetime. Imaging plays an important role in establishing early and correct diagnosis. OBJECTIVE: To evaluate the diagnostic accuracy of 3-Tesla (T) thorax MRI in HIV-positive children, using chest CT as the gold standard. MATERIALS AND METHODS: We included 25 children with confirmed HIV-positive status and pulmonary complaints who were referred for chest CT. All children had 3-T thorax MRI using T2-W turbo spin-echo sequence, steady-state free precession gradient echo sequence, T2-W turbo spin-echo MultiVane XD sequence, and T1-weighted modified Dixon sequences. We evaluated the images for various pulmonary and mediastinal findings and calculated the sensitivity and specificity of 3-T thoracic MRI. RESULTS: Sensitivity of 3-T MRI was 100% for detecting nodules >4 mm (95% confidence interval [CI] 66.3-100%), pleural effusion (CI 29.2-100%) and lymphadenopathy (CI 81.5-100%). It demonstrated a specificity of 100% for nodules >4 mm (CI 79.4-100%), pleural effusion (CI 84.6-100%) and lymphadenopathy (CI 59-100%). For consolidation/collapse, sensitivity and specificity were 93.8% (CI 69.8-99.8%) and 88.9% (CI 51.8-99.7%), respectively. The sensitivity and specificity for detecting bronchiectasis were 75% (CI 42.8-94.5%) and 100% (CI 75.3-100%), respectively, while for ground-glass opacity, sensitivity and specificity were 75% (CI 34.9-96.8%) and 94.1% (CI 71.3-99.9%), respectively. Nodules <4 mm were not well detected on MRI, with sensitivity of 35% (CI 15.4-59.2%). CONCLUSION: Thoracic MRI at 3 T demonstrates a high sensitivity and specificity for detecting nodules >4 mm, effusion and lymphadenopathy in HIV-positive children.

Challenges in the management of localized Ewing sarcoma in a developing country.

Survival in pediatric Ewing sarcoma (ES) lags in low- and middle-income countries (LMICs). This study analyzed factors contributing to a lower outcome in an LMIC center. A retrospective case review of children with localized ES treated from January 2011 till December 2017 was performed. Neoadjuvant chemotherapy with alternating cycles of vincristine, doxorubicin, cyclophosphamide; and ifosfamide, etoposide was administered 3-weekly for 48 weeks. Reassessment was planned for week 12, followed by local therapy (surgery/radiotherapy or both) tailed by adjuvant chemotherapy. Forty-eight patients with mean age 8 years (range: 0.7-14) were evaluated. Extremity and central axis tumors were seen in 25 (52%) and 23 (48%) patients. Three patients died of neutropenic sepsis and five abandoned therapy. Local therapy included primary surgery, radiotherapy and a combination of surgery and radiotherapy in 7 (16%), 20 (45%) and 17 (39%) patients. The 3-year event-free survival (EFS) and disease-free survival (DFS) for the cohort were 47.7 ± 11% and 57.6 ± 11.2%. Time to local therapy >16 weeks was associated with inferior DFS vs. local therapy administered within 16 weeks [46.6 ± 12.4 vs. 63.9 ± 19.4, p=.046]. Older age, axial site, large size and incomplete surgical resection did not predict relapse/progression. Patients who received wide local excision, as local therapy, had 100% DFS. Coordinated efforts to ensure timely therapy can improve outcome in pediatric ES. Abandonment and treatment-related mortality (TRM) are additional challenges that need to be tackled in LMICs.

Comparison of oral midazolam with intranasal dexmedetomidine premedication for children undergoing CT imaging: a randomized, double-blind, and controlled study.

BACKGROUND: Children undergoing computerized tomography (CT) frequently require sedation to allay their anxiety, and prevent motion artifacts and stress of intravenous (IV) cannulation. AIMS: The aim of this trial was to compare the effectiveness of oral midazolam and intranasal dexmedetomidine as sole premedicants in children for carrying out both IV cannulation as well as CT scanning, without the need for additional IV sedatives. METHODS: Fifty-nine children, aged 1-6 years, scheduled to undergo CT imaging under sedation were randomized to receive either 0.5 mg·kg-1 oral midazolam (group M) or 2.5 mcg·kg-1 intranasal dexmedetomidine (group D). After 20-30 min, intravenous cannulation was performed and response to its placement was graded using the Groningen Distress Rating Scale (GDRS). After cannulation, children were transferred on the CT table, and assessed using the Ramsay sedation score (RSS). CT imaging was performed without any further sedative if the RSS was ≥4. If there was movement or decrease in sedation depth (RSS ≤ 3), ketamine 1 mg·kg-1 IV was given as an initial dose, followed by subsequent doses of 0.5 mg·kg-1 IV if required. RESULTS: A Significantly higher proportion of children in group D (67%) achieved RSS ≥ 4 as compared to group M (24%) (P-0.002). The risk ratio (95% CI) was 2.76 (1.38-5.52). Significantly lower GDRS scores were noted in group D (1(1-2)) as compared to group M (2(1-2)) at the time of venipuncture (P = 0.04). CONCLUSION: In the doses and time intervals used in our study, intranasal dexmedetomidine (2.5 µg·kg-1 ) was found to be superior to oral midazolam (0.5 mg·kg-1 ) for producing satisfactory sedation for CT imaging.

Rapid lung MRI in children with pulmonary infections: Time to change our diagnostic algorithms.

BACKGROUND: To determine the diagnostic utility of a new rapid MRI protocol, as compared with computed tomography (CT) for the detection of various pulmonary and mediastinal abnormalities in children with suspected pulmonary infections. METHODS: Seventy-five children (age range of 5 to 15 years) with clinically suspected pulmonary infections were enrolled in this prospective study, which was approved by the institutional ethics committee. All patients underwent thoracic MRI (1.5T) and CT (64 detector) scan within 48 h of each other. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of MRI were evaluated with CT as a standard of reference. Inter-observer agreement was measured with the kappa coefficient. RESULTS: MRI with a new rapid MRI protocol demonstrated sensitivity, specificity, PPV, and NPV of 100% for detecting pulmonary consolidation, nodules (>3 mm), cyst/cavity, hyperinflation, pleural effusion, and lymph nodes. The kappa-test showed almost perfect agreement between MRI and multidetector CT (MDCT) in detecting thoracic abnormalities (k = 0.9). No statistically significant difference was observed between MRI and MDCT for detecting thoracic abnormalities by the McNemar test (P = 0.125). CONCLUSION: Rapid lung MRI was found to be comparable to MDCT for detecting thoracic abnormalities in pediatric patients with clinically suspected pulmonary infections. It has a great potential as the first line cross-sectional imaging modality of choice in this patient population. However, further studies will be helpful for confirmation of our findings.

Comparison of intravenous urography and magnetic resonance urography in preoperative evaluation of pelvi-ureteric junction obstruction in children.

AIMS: To compare intravenous urography (IVU) and magnetic resonance urography (MRU) in the preoperative evaluation of pelvi-ureteric junction obstruction (PUJO) in children. MATERIALS AND METHODS: A total of 35 children up to 10 years of age in whom unilateral or bilateral PUJO were suspected on ultrasonography were enrolled in this prospective study. All children underwent IVU and MRU, and the findings were compared. RESULTS: Of the 70 kidneys evaluated, 14 (20%) were not visualized on IVU because of nonexcretion of contrast, whereas all the 70 (100%) kidneys were visualized on MRU. On IVU, nephrogram was not visualized in 66 (94.2%) of the 70 kidneys, whereas MRU showed prompt and homogeneous nephrogram in 68 (97.1%) of the 70 kidneys. No evidence of PUJO was seen in 31 (44.2%) kidneys on both IVU and MRU. IVU showed PUJO in 26 (37.1%) kidneys, whereas MRU showed it in 38 (54.2%) kidneys. MRU detected two duplex systems that were missed on IVU. A focal renal lesion and two incidental extra renal abnormalities were detected on MRU, which were not visualized on IVU. CONCLUSION: MRU is better than IVU, especially in case of poorly functioning kidneys which are not visualized on IVU. MRU also provides anatomic details of the ureter and vessels with better evaluation of renal parenchyma. It also has an additional advantage of detecting incidental extra renal abnormalities, if present.

Intima-media complex thickness: preliminary workup of comparative evaluation of abdominal aorta and carotid artery of small-for-gestation-age term newborns and normal size term newborns.

BACKGROUND: Increased intima-media thickness (IMT) has shown to be a good predictor of increased incidence of cardiovascular disease. The use of noninvasive measurement of abdominal aortic intima-media thickness (aIMT) and carotid artery intima-media thickness (cIMT) is an attractive modality to further explore and define possible intrauterine factors that may be associated with increased risk of atherosclerosis. PURPOSE: The aim of this study was to compare intima-media thickness of abdominal aorta and carotid artery in small-for-gestation-age (SGA) term newborns with appropriate for gestation age (AGA or normal sized) term newborns. MATERIAL AND METHODS: We measured the intima-media thickness of the abdominal aorta (aIMT) and carotid artery (cIMT) by high resolution ultrasonography of 50 SGA and 50 AGA term newborns. RESULTS: Mean aIMT and cIMT were significantly greaterin the SGA term newborns group as compared to AGA term newborns (0.54 +/- 0.06 mm and 0.44 +/- 0.04 mm in SGA term newborns vs. 0.50 +/- 0.04 mm and 0.40 +/- 0.04 mm in AGA term newborns; P < 0.008 and P < 0.001, respectively). The significance was even more apparent after adjustment for birthweight. A negative correlation of aIMT and cIMT was seen with birthweight, Ponderal index, length and head circumference. CONCLUSION: SGA term newborns have significantly increased aortic and carotid intima-media thickness as compared to AGA term newborns. This might be associated with higher risk for atherosclerosis. Longitudinal studies are required to further enhance the possible correlation between birthweight and intima-media thickness in SGA babies.