Magnetic-Assisted Capsule Endoscopy in Children With Crohn Disease: Feasibility and Impact on Gastric Transit Time.

OBJECTIVES: Standard capsule endoscopy (CE) is ineffective for upper gastrointestinal (GI) tract examination because it does not allow operator-controlled navigation of the capsule. Magnetically assisted capsule endoscopy (MACE) may offer a solution to these problems. This pilot study is aimed to evaluate the feasibility of MACE system in pediatric Crohn disease (CD) and if magnetic steering could enhance capsule gastric emptying when compared with standard CE. METHODS: Pediatric CD patients already studied by standard small bowel CE were enrolled. All participants swallowed a magnetically assisted CE and an external magnetic field navigator was used to guide the capsule through the upper GI tract. Maneuverability, completeness of the MACE examination, differences in the esophageal transit time (ETT), gastric transit time (GTT), and pyloric transit time (PTT) between standard CE and MACE were assessed. RESULTS: Ten patients [mean age 11.4 years (range 6-15); 60% male] were enrolled. Maneuverability was defined as good and fair in 60% and 40% of participants, respectively. Completeness of MACE examination was 95%, 65%, and 92.5% in the esophagus, proximal, and distal stomach, respectively. Transpyloric passage of the capsule under magnetic control was successfully performed in 80% of patients. Magnetic intervention significantly increased ETT ( P < 0.001) and reduced GTT and PTT ( P = 0.002). No significant adverse events occurred. CONCLUSIONS: MACE is a safe and feasible technique in children. Magnetic steering enhances capsule gastric emptying and facilitates capsule transpyloric passage when compared with standard CE.

Abdominal Recurrence after Robotic Nephron-Sparing Surgery for Wilms Tumor in an Adult Patient.

A 31-year-old man was referred to an adult urologist for a renal polar mass that measured 7.2 cm in maximum diameter. Robotic assisted complete tumor excision for suspicious renal cell carcinoma was carried out, preserving the rest of the left kidney. Histopathology showed a Wilms tumor (WT) with positive margins. No postoperative therapy was made, and the patient shortly presented an abdominal recurrence. The patient was referred to our pediatric oncology unit; he received preoperative chemotherapy, followed by surgery (completion nephrectomy and removal of neoplastic deposits in the omentum and parietal peritoneum), postoperative chemotherapy, and abdomen radiotherapy. He is well at the 5-year follow-up. Peritoneal dissemination after laparoscopic nephron-sparing surgery (NSS) in a child with a 10-cm WT was previously reported. We suggest open NSS for large WT may be safer than laparoscopic or robotic NSS because carbon dioxide pneumoperitoneum and traumatic handling of tumor may predispose to tumor cell migration. An abdominal WT relapse in adults can be salvaged by multimodal therapy recommended by current pediatric WT guidelines.

Zero-ischemia testis-sparing surgery for probably benign pediatric testicular tumors.

Permanent ischemia-induced testicular damage may occur as early as 30 min in prepupertal rats. With the goal of potentially enhancing testicular function and fertility preservation, we performed testis-sparing surgery (TSS) without ischemia for testicular lesions in select children with negative markers and high likelihood of benignity on ultrasonography. Preliminary experience suggests that off-clamp TSS should be more liberally encouraged, especially in infants and prepubertal children, given their particularly vulnerable spermatic cord elements.

Evaluation of Nephron-Sparing Surgery as Potential Risk Factor for Relapse in Unilateral Wilms Tumor.

BACKGROUND: The aim of the study was to assess the prognostic significance of nephron-sparing surgery (NSS) without tumor size limits as a risk factor for relapse in children with unilateral Wilms Tumor (WT). METHODS: A 28-y retrospective single-center review was performed. Prognostic relevance of age, gender, stage, histology, nephrectomy (N), and NSS was analyzed. RESULTS: Sixty-nine cases (42 females and 27 males) with WT, off-therapy from 21 to 325 mo after chemotherapy mainly based on the International Society of Pediatric Oncology trials, were treated at our institution. Five cases were excluded (three children with synchronous bilateral WT and two adults with unilateral WT). Of 64 children with unilateral WT, 51 underwent N and 13 NSS without tumor size limits. Indeed, two-thirds of children who underwent NSS presented with a tumor diameter >4 cm. Overall, nine patients (14%) had a relapse (male-to-female ratio = 1:8). Initial surgery was N in eight cases and NSS in another one. Relapse rates in N and NSS groups were 15.7% and 7.7% (P = nonsignificant), respectively; the relapse rates in N and NSS groups were 8.6% and 7.7% (P = nonsignificant) for stages I-II unilateral WT cohort, respectively. On univariate analysis, factors correlated with probability of relapse were unfavorable histology (P < 0.002) and stage III disease (P < 0.01). CONCLUSIONS: In unilateral WT, NSS, whenever feasible, does not seem to increase the risk of recurrence. A multicenter prospective trial is required to carefully evaluate this risk.

The DATE Association: A Separate Entity or a Further Extension of the VACTERL Association?

BACKGROUND: The combination of esophageal atresia, congenital duodenal obstruction, and anorectal malformation has seldom been reported. We describe the largest series of patients with such association, which we summed up with the mnemonic acronym DATE [D-duodenal obstruction, A-anorectal malformation (ARM), and TE-tracheoesophageal fistula with esophageal atresia]. METHODS: This was a multicenter retrospective review of 13 patients recruited from 8 institutions over a nearly 5-decade period (1968-2017). Information gathered included type of DATE malformations, other associated anomalies, type and timing of surgery, and clinical outcomes. RESULTS: The DATE association consisted of type C esophageal atresia (13), complete (9) or incomplete (4) congenital duodenal obstruction (CDO), and high or intermediate (8) or low (5) ARM. Eight patients had at least one additional component feature of VACTERL association. A total of 6 patients died. Overall, 9 patients achieved complete restoration of gastrointestinal continuity, 7 of whom are alive at a median follow-up of 4 y (range, 1 to 9). Survivors received a median of 6 major operations (range, 4 to 14) to overcome their anomalies and surgical complications. Two incomplete duodenal obstructions were initially overlooked. All survivors with high or intermediate ARM defects required some form of bowel management to keep them clean. CONCLUSIONS: The DATE association is a low-frequency entity, often occurring among the wider spectrum of VACTERL association. Functional outcomes largely depend on the severity of ARM or other major associated malformations. Awareness of the DATE association may avoid untoward diagnostic delays of subtler component features of the spectrum, such as an incomplete CDO.

Renal function up to the 5th decade of life after nephrectomy in childhood: A literature review.

The aim of the present study was to find out if in children ablation of 50% of renal mass may be associated with a progressive renal damage (Brenner's hypothesis). We collected 1035 adult or adolescent survivors who underwent nephrectomy for unilateral oncological or non-oncological causes during childhood. Stratification of all survivors for age revealed that the number of subjects with blood hypertension and/or renal dysfunction (glomerular filtration rate < 90 mL/min per 1.73 m2 ) to be significantly higher in survivors ≥30 years old in comparison with younger patients. Available data on long-term renal function after nephrectomy during childhood support the Brenner's hypothesis.

Noninvasive Therapy for Osteoid Osteoma: A Prospective Developmental Study with MR Imaging-guided High-Intensity Focused Ultrasound.

Purpose To demonstrate that magnetic resonance (MR) imaging-guided high-intensity focused ultrasound (HIFU) is a safe, effective, and durable treatment option for the management of osteoid osteoma in children and young adults. Materials and Methods This prospective study was institutional review board approved and is registered with clinicaltrials.gov (identification number NCT02302651). Written informed consent was obtained from patients or their parents. Patients who had both clinical and radiologic findings that were diagnostic for nonvertebral osteoid osteoma and no contraindications to MR imaging-guided HIFU were enrolled between June 2010 and June 2013. The feasibility, safety, and clinical effectiveness of MR imaging-guided HIFU were considered primary outcomes; tumor control at imaging was considered a secondary outcome. Analyses were conducted on a per-protocol basis. Results Forty-five of the 50 recruited patients underwent MR imaging-guided HIFU. All patients were discharged without treatment-related complications. The median visual analog scale (VAS) pain score (scale, 0-10) decreased from 8 before treatment to 0 at 1-week and 1- , 6- , 12- , 24- , and 36-month follow-up. Similarly, median VAS scores for the degree to which pain interfered with sleep and physical and daily activities decreased to 0 within the 1st month after treatment and remained stable at subsequent follow-up. Overall, 39 (87%) of the 45 patients achieved and maintained a VAS score of 0 during the 3-year observation period. Quality of life, as assessed by using Functional Assessment of Cancer Therapy-Bone Pain (FACT-BP) scores (scale, 0-60 points), improved, with the median FACT-BP score of 28 (points) before treatment increasing to a median score of 55 at 1-week follow-up and 60 at 6-month and subsequent follow-ups. At (final) 36-month follow-up, MR imaging depicted an absence of residual nidus vascularity in 32 (76%) of the 42 patients who were treated with MR imaging-guided HIFU only. Conclusion The durable clinical efficacy and safety of MR imaging-guided HIFU were demonstrated. These features are evidence of the potential of MR imaging-guided HIFU to be part of a routine strategy for the treatment of osteoid osteoma. © RSNA, 2017.

Plaque-Like Myofibroblastic Tumor of Infancy: A New Case Report and Literature Review.

Plaque-like myofibroblastic tumor of infancy is a rare entity, with only a few reports described in the literature. Herein we present a new case of a nodular plaque-like lesion of the left lower back in an 18-month-old boy. The lesion might initially be thought to be a dermatofibroma, but the overall characteristics suggested the diagnosis of plaque-like myofibroblastic tumor of infancy. We also provide a summary of previous reports in the literature about this exceptional tumor.

Bi-parametric magnetic resonance imaging applied to obstetrics.

Foetal magnetic resonance imaging (MRI) plays an increasingly important role in the diagnosis of foetal abnormalities. Over the years, we have successfully applied bi-parametric MRI (bp-MRI) to the following obstetric conditions: (1) neurologic vascular diseases; (2) assessment of lung parenchyma maturation; (3) renal pathologies, such as polycystic kidney, suspected renal infarction, unilateral or bilateral renal agenesis; (4) placental pathologies, as twin-twin transfusion syndrome or placenta accreta; (5) benignant and malignant congenital tumours or cysts of the liver, such as haemangioendothelioma, hepatoblastoma or metastatic neuroblastoma, of the kidney (e.g. mesoblastic nephroma) and of the retroperitoneum, such as teratoma. The information derived from bp-MRI, and concerned with water motions in different tissues, improved the morphologic details provided by conventional foetal MRI. It has the potential to increase the value of MRI in the assessment of a wide range of foetal pathologies, particularly in renal diseases, allowing an adequate management decision and therapy.

The THUNDERBEAT system for tissue dissection and vascular control in laparoscopic splenectomy.

The advent of new energy sources for hemostasis has greatly facilitated advanced laparoscopic procedures. We describe a straightforward technique of laparoscopic splenectomy (LS) accomplished using the THUNDERBEAT™ system (TS) (Olympus Medical Systems Corp., Tokyo, Japan) as the sole means of tissue dissection and hemostasis in two patients aged 19 and 6 years, respectively. The specimens were removed intact via a Pfannenstiel incision. Total operative time was 165 and 150 min, and length of hospital stay was three and 4 d, respectively. The TS is an appealing and reliable alternative to currently available energy devices, allowing fast dissection and secure hemostasis during laparoscopic splenectomy.

Role of magnetic resonance imaging in the prenatal diagnosis of gastrointestinal fetal anomalies.

PURPOSE: This study was done to evaluate the role of fetal magnetic resonance imaging (MRI) in the study of gastrointestinal malformations in comparison to prenatal ultrasound (US). MATERIALS AND METHODS: A prospective (2010-2012) study of 38 fetal MRI scans was performed on 38 fetuses between 24 and 38 weeks of gestation. All the fetuses had a US diagnosis of gastrointestinal anomalies. T2-weighted HASTE, T1-weighted fast gradient echo, TrueFISP and diffusion-weighted images of the fetal abdomen were obtained on a 1.5-Tesla magnet. All fetal MRI diagnoses were compared with postnatal US findings, autopsy or surgical reports. RESULTS: Fetal MRI was able to confirm the sonographic findings in nine of 38 fetuses (23.7%), to provide additional information in 23 of 38 fetuses (60.6%), to exclude the US diagnosis in five cases (5.2%) and to change it in two cases (5.2%). It was not able to characterize a case of gastric duplication and a case of abdominal cystic lymphangioma (5.2%). CONCLUSIONS: Fetal MRI can be used as a complementary imaging modality to US in prenatal evaluation of gastrointestinal anomalies and can be considered a valuable tool not only for confirming or excluding but also for providing additional information to fetal ultrasonographic findings.

Effective compression bandage for repair of a complicated radial artery pseudoaneurysm.

We describe a 10-week-old hemophilic infant who developed a large-sized pseudoaneurysm of the radial artery after arterial puncture. Based on our previous experience, compression bandage was elected as first-line treatment. The clinical course was complicated by skin ulceration. However, compression bandage was continued, and complete thrombosis of the pseudoaneurysm occurred after 4 weeks of treatment, preserving arterial patency. Bedside clot evacuation and enzymatic debridement promoted rapid wound healing with negligible scar formation. Compression bandage is a safe and valuable measure for repair of radial artery pseudoaneurysm, even in patients with clotting disorders. Professionals are encouraged to consider the benefit of such an artery-sparing treatment option.

Renal function adaptation up to the fifth decade after treatment of children with unilateral renal tumor: a cross-sectional and longitudinal study.

BACKGROUND: Mild-to-moderate renal function loss may be an independent risk factor for cardiovascular disease and overall mortality. As in adults with renal carcinoma nephrectomy is associated with an high risk for moderate renal function loss, we aimed to assess the renal function adaptation over a long period of time in children with unilateral renal tumor (URT). PROCEDURE: Seventy-two children who underwent surgery for URT were enrolled in this study. Glomerular filtration rate was estimated (eGFR) with the Modification of Diet in Renal Study or the Schwartz equation, as appropriate for the age. RESULTS: Twelve patients treated by nephron-sparing surgery (Group A) and 42 treated by nephrectomy (Group B) had an age between 2 and 30 years; 18 patients treated by nephrectomy had an age between 33 and 51 years (Group C). At cross-sectional follow-up 8% patients of Group A, 42% of Group B and 78% of Group C presented a mild-to-moderate renal function. The longitudinal data stratified by post-operative intervals showed that patients of Group C presented a significant progressive decrease in mean ± standard deviation eGFR (88.1 ± 22.6 during the third decade after surgery vs. 66.6 ± 15.6 ml/min/1.73 m(2) during the fifth decade after surgery; P = 0.02). The longitudinal data stratified by age showed that patients with an age between 45 and 54 years presented a mean eGFR significantly lower than that expected for the physiological renal function decline with aging (P = 0.001). CONCLUSION: Aging is associated with a mild-to-moderate renal function loss in many adult patients following nephrectomy during childhood for URT.

The Role of Manual Detorsion in Pediatric Testicular Torsion During the Global COVID-19 Pandemic: Experience From 2 Centres.

OBJECTIVE: To evaluate the role of emergency manual detorsion as first line management for testicular torsion in the context of the COVID-19 pandemic. METHODS: This retrospective observational study includes 90 pediatric patients ≤14 years old with diagnosis of testicular torsion made at 2 tertiary centers between October 2020 and June 2022. Variables examined included age, presentation delay, surgical wait time, number of attempts at manual testicular detorsion, and manual testicular detorsion success. All patients finally underwent surgery, including contralateral testicular fixation. Outcomes included predictors of successful manual detorsion, testicular findings at surgery, and operation time. RESULTS: Mean (SD) age at diagnosis was 11.51 (2.64) years. Mean presentation delay was 11.76 (13.79) hours. Detorsion was attempted in 72 (80%) patients, resulting successful in 58 (80.5%). Surgical wait time after successful manual detorsion was 22.85 (16.94) hours. On multivariable analysis, successful manual detorsion was associated with a presentation delay<6 hours (odds ratios [OR] 0.154, 95% confidence intervals (CI) 0.036-0.655, P = 0.01) and absence of scrotal edema (OR 0.171, 95% CI 0.038-0.769, P = 0.02). Vice versa, a heterogeneous echo-texture (OR 0.57, 95% CI 0.007-0.461, P = 0.007) and absent blood flow on Doppler ultrasound scan (OR 0.256, 95% CI 0.067-0.971, P = 0.045) were significantly associated with the likelihood of manual detorsion failure. CONCLUSION: In our experience, manual detorsion provided safe and effective emergency treatment for pediatric testicular torsion, especially in absence of edema and when presentation delay is <6 hours. This maneuver should be more widely attempted immediately after diagnosis as temporizing rescue.

Early Surgery Versus Watchful Waiting Strategy for Infantile Abdominoscrotal Hydrocele.

BACKGROUND: Infantile abdominoscrotal hydrocele is generally managed with early surgery. However, whether these patients actually benefit from an initial watchful waiting strategy is yet to be determined. METHODS: This single-center, comparative observational analysis included 36 consecutive patients (9 bilateral cases) diagnosed between January 1998 and December 2019. Initial 16 patients (21 lesions) underwent surgical repair shortly after diagnosis (Group A) at a mean (SD) age of 8 (4) months. Remaining 20 patients (24 lesions) underwent initial nonoperative management (Group B). Overall follow-up was 82.74 (63.84) months. RESULTS: Six lesions of Group B spontaneously regressed at a mean age of 23.8 (7.8) months. Remaining 18 hydroceles underwent surgical management at a mean age of 18.7 (12.6) months. Early postoperative morbidity was high (43%) in both groups (Clavien-Dindo grade I-II). During follow-up, there were 1 recurrent hydrocele and 1 testicular atrophy, both events occurring in Group A patients. Group B patients required a higher need for concomitant ipsilateral orchidopexy (61% versus 14%; p = 0.001), and developed a higher rate of ipsilateral inguinal hernia (39% versus 5%; p = 0.01), occurring within 8.3 (3.53) months of surgery. On multivariable analysis, nonoperative management was associated with unplanned second surgery (odds ratio 5.5, 95% CI 1.25-24.17, p = 0.02), regardless of the type of hydrocelectomy performed. CONCLUSIONS: Nonoperative management provides effective spontaneous resolution in about 25% of infantile abdominoscrotal hydrocele. Clinicians must balance the potential benefits of such strategy against the impact of pressure induced detrimental effects on the success of future surgery when dealing with longstanding lesions. LEVEL OF EVIDENCE: III.

Female splenogonadal fusion: A PLEA for conservative management.

Splenogonadal fusion in female patients is seldom reported. We describe a 6-month-old girl who represents the youngest living female with splenogonadal fusion reported to date. The lesion was diagnosed as an incidental finding during screening abdominal ultrasonography performed for a vulvar infantile hemangioma. A tail-like structure with splenic echotexture connecting a normally located spleen and the left ovary was detected and better characterized by MRI. We also reviewed the pertinent literature on managing this usually asymptomatic condition, especially in female patients. Greater professionals' awareness of this benign anomaly is paramount to avoid the unnecessary removal of an otherwise normal gonad.