Neuroblastoma presenting like a Wilms' tumor with thrombus in inferior vena cava and pulmonary metastases: a case series.

Neuroblastomas and Wilms' tumors are frequent pediatric solid tumors. The first is frequently detected in the adrenal gland and the second develops in the kidneys. The extension through the vena cava and the lung metastases are frequent in Wilms' tumors and are rarely seen in neuroblastoma. We present the cases of three children with abdominal tumors with thrombus in the inferior vena cava and pulmonary metastases demonstrating a stage 4 neuroblastoma. The three male patients were between 23 to 48 months old. They presented an abdominal mass, near the superior pole of the kidney. Thrombus of the vena cava was showed on imaging studies in all cases and pulmonary metastases were always found. Catecholamine metabolites were present in the first case and negative in the two others. Two out of three patients had a radical nephrectomy. The pathological analysis always found a poorly differentiated or undifferentiated neuroblastoma without MYCN amplification and confirmed the vein tumoral thrombus in the second case. The evolution of the first two patients was unfavorable and the third is alive. Invasion of the inferior vena cava and pulmonary metastases in children with neuroblastoma is uncommon and can modify the surgical management.

A study of calretinin in Hirschsprung pathology, particularly in total colonic aganglionosis.

INTRODUCTION: Calretinin, a calcium-binding protein, has been reported to be an important new marker in Hirschsprung's disease (HD). The aim is to study the diagnostic value of Calretinin in total colonic aganglionosis (TA), prematurity, and superficial biopsy when nerve hyperplasia may not be accessed by ACE activity. METHODS: Records of patients diagnosed with HD at our institution from 1985 to 2010 were studied and patients with TA identified. We examined tissue samples from those TA, partial colectomies for HD, biopsies for suspicion of HD, and rectal tissue from aborted fetuses. Immunohistochemical analysis of Calretinin was compared with ACE gold standard method in all cases. RESULTS: In the majority of the cases, the diagnosis was ascertained by ACE activity and Calretinin staining. However, in 9 cases, the diagnosis was possible with Calretinin staining but not with ACE: in 4 TA because of the absence of nerve hyperplasia, and in 5 cases because the biopsies were too superficial to examine the nerve hyperplasia. In addition, Calretinin was expressed in the gut as early as 22 gestational weeks. CONCLUSION: The use of Calretinin staining may be superior to ACE activity, particularly in the context of TA, superficial biopsies, and prematurity, allowing earlier diagnosis.

Incidental finding of synchronous bilateral ductal carcinoma in situ associated with gynecomastia in a 15-year-old obese boy: case report and review of the literature.

Carcinoma of the breast is rarely encountered in the male population and is even less prevalent in the pediatric male population. Studies have suggested an association between male breast carcinoma and gynecomastia, but conflicting results have been shown. Only 3 cases of carcinoma in situ associated with bilateral gynecomastia during puberty have been described in the literature. Here, we present the case of a 15-year-old boy with bilateral gynecomastia who was found to have synchronous bilateral ductal carcinoma in situ.

Predictive indicators for bowel injury in pediatric patients who present with a positive seat belt sign after motor vehicle collision.

PURPOSE: Abdominal wall bruising (AWB) is a frequent finding in children wearing seat belts involved in motor vehicle collision (MVC) and is highly suspicious but not indicative of intestinal injury. The aim of this study was to find objective clinical and radiologic predictors for the need of an abdominal exploration in these children. MATERIALS AND METHODS: A retrospective chart review of children admitted from 1998 and 2008 with AWB after MVC was conducted. Demographics, vital signs, physical examinations, radiologic investigations, associated injuries, management, and outcome were extracted. Univariate and multivariate statistical analyses were done. RESULTS: Fifty-three children with a median age of 9 years (range, 3-16 years) were included. Forty-four patients (83%) had abdominal pain on arrival, and 25 (47%) had free intraabdominal fluid on ultrasound/scan. Intraabdominal injuries were noted in 29 patients (55%), and the most common were mesenteric or bowel injuries (25%), splenic injuries (13%), and hepatic injuries (8%). Ten patients (19%) needed therapeutic laparotomy, and all were victims from collision involving 2 moving vehicles, had abdominal pain, free intraabdominal fluid, and tachycardia. Five patients (50%) operated on had lumbar fracture compared to only 4 patients (9%) in the nonoperative group. Pulse rate higher than 120 (P = .048), lumbar fracture (P = .008), and free intraabdominal fluid (P

Endoscopic retrograde cholangiopancreatography is useful and safe in children.

PURPOSE: Endoscopic retrograde cholangiopancreatography (ERCP) is a recognized diagnostic and therapeutic tool in the adult population. Its use in children has been more common in the last years. There are little data on safety and usefulness of that procedure in children. The aim of this study was to review the experience with ERCP in a tertiary university center dedicated to children. METHOD: We conducted a retrospective chart review of patients seen at the Centre Hospitalier Universitaire Ste-Justine (Montreal, Quebec, Canada) who had undergone an ERCP between September 1990 and July 2007. Data on demographics, diagnosis, anesthesia type, treatments, and complications were collected. RESULTS: Thirty-eight ERCPs were performed on 29 patients. There were 21 girls (72%), and median age at time of procedure was 10.3 years old (range, 3-17 years). Most had only one procedure performed. Two children had 2 interventions, and 1 child with papillary stenosis had 8 interventions linked to stent treatment. The ampulla was cannulated, and the procedure was successfully completed in 97% (37/38) of cases. General anesthesia and sedation were performed in 74% and 26% of procedures, respectively. Indications for ERCP were 29 recurrent or chronic pancreatitis (76%), 8 common bile duct obstructions (21%), and 1 choledochal cyst (3%). Endoscopic treatment was done in 29% of cases. The complication rate was 13.5%, and 4 clinical acute pancreatitis resolved with conservatory treatment. No severe pancreatitis, perforation, or bleeding was noted. Of the patients, 79% had their follow-up at the Centre Hospitalier Universitaire Ste-Justine for a median length of 43 months (range, 1-53 months). CONCLUSION: Endoscopic retrograde cholangiopancreatography is used as a diagnostic and therapeutic procedure in children with a complication rate similar to that seen in adults. The need for general anesthesia is much more frequent with children. When performed by well-trained endoscopists, ERCP is useful and safe in children.

Timing and surgical management of neonatal testicular torsions.

BACKGROUND: The timing and surgical management of neonatal testicular torsions (NTTs) remain controversial, varying from immediate orchiectomy with empirical contralateral orchiopexy to expectant management with resulting atrophy of the affected testicle. The goal of the present study is to review the management of this entity at our institution. MATERIALS AND METHODS: A retrospective study of all patients with NTT from 1989 to 2007 was undertaken. The age, clinical presentation, investigation, management, and short- and long-term outcomes were noted. RESULTS: Forty-four patients were included. Most presented with a firm testicular mass, scrotal discoloration, and hydrocele (42), whereas a few presented with testicular atrophy (2). The median age at presentation was 1 day of age (range, 0-84 days), with NTT occurring on the right side in 22 patients and the left side in 20. Two patients (5%) had bilateral torsion at presentation. In 33 patients, the diagnosis was confirmed by Doppler ultrasonography, whereas 11 patients did not undergo any additional investigation. Surgical management included ipsilateral orchiectomy and contralateral orchiopexy (IOCO) (27), orchiopexy of the contralateral testis (CO) (7), bilateral orchiopexy (4), orchiectomy of the ipsilateral testis (1), orchiopexy of the ipsilateral testis (2), and observation (1). The 2 bilateral torsions underwent bilateral orchiectomy (2). The median age at surgery was 25 days (range, 1-912 days). Postoperative complications occurred in 8 patients (18%), mainly in those with IOCO (4) and CO (4) operated before 12 days of age, and included recurrent hydrocele (3), wound infection (2), urinary tract infection (1), and others (2). Upon follow-up, patients who underwent CO developed ipsilateral testicular atrophy (6). No patients were readmitted for recurrence of torsion or other complications. CONCLUSION: At our institution, the most frequent management of unilateral neonatal testicular torsions is IOCO or CO, but this carries an 18% complication rate, particularly if surgery is performed early. There seems to be no advantage to early intervention, and the need for orchiectomy is debatable because torsion leads to ipsilateral testicular atrophy. Contralateral orchiopexy done to decrease the incidence of bilateral asynchronous torsion should, at the very least, be deferred until the risks of anesthesia and surgery are improved, given its rarity. Given the fact that most patients underwent IOCO or CO, we cannot conclude which strategy is the best for neonatal testicular torsions. A prospective study is welcomed.

Mechanisms of active laryngeal closure during noninvasive intermittent positive pressure ventilation in nonsedated lambs.

The present study stems from our recent demonstration (Moreau-Bussiere F, Samson N, St-Hilaire M, Reix P, Lafond JR, Nsegbe E, Praud JP. J Appl Physiol 102: 2149-2157, 2007) that a progressive increase in nasal intermittent positive pressure ventilation (nIPPV) leads to active glottal closure in nonsedated, newborn lambs. The aim of the study was to determine whether the mechanisms involved in this glottal narrowing during nIPPV originate from upper airway receptors and/or from bronchopulmonary receptors. Two groups of newborn lambs were chronically instrumented for polysomnographic recording: the first group of five lambs underwent a two-step bilateral thoracic vagotomy using video-assisted thoracoscopic surgery (bilateral vagotomy group), while the second group, composed of six lambs, underwent chronic laryngotracheal separation (isolated upper airway group). A few days later, polysomnographic recordings were performed to assess glottal muscle electromyography during step increases in nIPPV (volume control mode). Results show that active glottal narrowing does not develop when nIPPV is applied on the upper airways only, and that this narrowing is prevented by bilateral vagotomy when nIPPV is applied on intact airways. In conclusion, active glottal narrowing in response to increasing nIPPV originates from bronchopulmonary receptors.

Origins of the inhibiting effects of nasal CPAP on nonnutritive swallowing in newborn lambs.

The present study investigated the mechanism by which continuous positive airway pressure (CPAP) suppresses nonnutritive swallowing (NNS) during quiet sleep (QS) in newborn lambs. Eighteen full-term lambs were chronically instrumented and evenly distributed into three separate groups to determine the extent to which modulation of NNS may be attributed to stimulation of upper airway and/or bronchopulmonary mechanoreceptors. Six lambs were tracheotomized, six other lambs underwent a two-step bilateral intrathoracic vagotomy, and the remaining six lambs underwent chronic laryngotracheal separation (isolated upper airway group). Forty-eight hours after surgery, each nonsedated lamb underwent polysomnographic recordings on three consecutive days. States of alertness, NNS and respiratory movements were recorded. Results demonstrate that a CPAP of 6 cmH(2)O inhibited NNS during QS while administered directly on the lower airways and that bivagotomy prevented this inhibition. However, application of CPAP on the upper airways only also inhibited NNS during QS. Finally, the application of a CPAP of 6 cmH(2)O had no systematic effect on NNS-breathing coordination (assessed by the respiratory phase preceding and following NNS). In conclusion, our results suggest that bronchopulmonary receptors are implicated in the inhibiting effects of nasal CPAP of 6 cmH(2)O on NNS in all our experimental conditions, whereas upper airway receptors are only implicated in certain conditions.

Clinical algorithm and resource use in the management of children with minor head trauma.

PURPOSE: There are no clear guidelines for the management of minor head injury, including the use of skull x-rays and computed tomography (CT) scans of the head. This is reflected in clinical practice by a wide variability in imaging study use and by the fact that some patients are discharged home from the emergency room (ER), whereas others are admitted to the hospital with or without a period of observation before admission. To address this issue, we proposed and applied a new protocol for minor head injury at our institution. METHODS: Between January 2004 and December 2005, 417 patients presented to the emergency department at our institution with minor head injury. All of them had fallen from less than 1 m. Every chart was retrospectively evaluated, and pertinent data were extracted. RESULTS: The mean age of the patients was 9.8 months (2 weeks to 32 months). One hundred fifty-three had a skull x-ray, and 13 had a CT scan of the head. Of the 153 patients who had a skull x-ray, only 15 had a skull fracture. Of these 15 patients, 3 also had a CT scan of the head that confirmed the diagnosis of skull fracture. Of the 13 CT scans that were done, only these 3 were positive. Eleven patients were kept in the ER for 6 hours for close observation, and 5 of these were eventually admitted. Overall, 8 patients were admitted to the hospital for observation. Of these 8 patients, 7 had a skull x-ray, from which 5 were positive. Only 2 of the admitted patients had a CT scan, and they were both positive for a skull fracture. One of the CT also demonstrated a subdural hematoma along with subarachnoid hemorrhage. These 2 patients also had a positive skull x-ray. None of the patients that were admitted had headaches or neurologic impairments. The mean age of the patients admitted was 3.8 months (2 weeks to 12 months). The mean hospital stay was 1.2 days (1-3 days). CONCLUSION: Only 10% of the skull x-rays and CT scans were positive for a skull fracture, which led to an admission in half of these patients. The other half was mainly discharged from ER after being observed. Several patients underwent a skull x-ray that we feel was not necessary in the management of their minor head injury. For those who had a head CT scan, only one revealed additional information and none of them had an impact on the final management. Observation in the ER could have been reasonable for most cases.

Vacuum-assisted closure for wound management in the pediatric population.

BACKGROUND/PURPOSE: Wound management in children has traditionally consisted of daily dressings. Although vacuum-assisted closure (VAC) is well described in the adult literature, there are few reports about children. We reviewed our experience with VAC. METHODS: A retrospective review from 2003 to 2005 revealed that 16 children underwent VAC. Variables analyzed included demographics, diagnosis, duration and characteristics of VAC, wound closure, recurrence, complications, and cost analysis. RESULTS: Sixteen children received VAC therapy at an average age of 12.1 years (range, 1 month-18 years). Indications included tissue loss after pilonidal sinus excision (n = 8, primary = 5, recurrent = 3) after wound dehiscence of the abdomen (3), the sternum (2), the back (1), the leg (1), and after chronic postoperative perineal fistula. Average length of VAC use was 23 days, with an average pressure of 104 mm Hg. Wound closure occurred in 15 of 16 patients. Patients with primary pilonidal disease obtained wound closure by 45 days, whereas those with recurrent disease required 72 days. Children with wound dehiscence healed by 28 days. Recurrent sinuses developed in all 3 patients with known recurrent pilonidal disease. Pain in 1 patient required cessation of VAC therapy after 7 days. Follow-up after wound closure averaged 8 months. CONCLUSIONS: Vacuum-assisted closure is well tolerated in our pediatric population and offers many advantages including fewer dressing changes and an earlier return to daily activities.

Spontaneous pneumomediastinum: are we overinvestigating?

BACKGROUND: Spontaneous pneumomediastinum (SPM) is uncommon in pediatrics. Because of the growing concern about the risks of radiation in children, the authors analyzed whether an extensive radiologic workup influences management and outcome. METHODS: In a retrospective study from 1991 to 2003, 53 patients were diagnosed with SPM. Charts were reviewed for demographics, predisposing factors, presentation, investigation, and evolution. Pneumomediastinum occurring in the neonatal period or related to either pneumothorax, barotrauma, or trauma were excluded. RESULTS: Of 53 cases, 26 (49%) were bronchospasm related, 11 (21%) had respiratory tract infections, and 8 (15%) were idiopathic. Four (7.5%) were caused by inhaled foreign bodies while other causes accounted for the remaining 7.5%. No esophageal perforations were identified. Presentations included dyspnea (64%), subcutaneous emphysema (60%), cough (45%), cervical or chest pain (42%), and Hamman's sign (19%). Postero-anterior chest x-rays (CXR) were diagnostic in all cases except one. Mean number of CXR per hospitalization was 3. Only 3 patients subsequently had pneumothorax, and none required pleural drainage. Of the 8 patients with idiopathic SPM, 5 underwent a barium swallow, and 2 had a chest CT scan; results of all were normal. CONCLUSIONS: More than 70% of SPMs were related to bronchospasm or respiratory tract infections. Idiopathic SPMs deserve more attention because of the concern about esophageal perforation, although most investigations will be negative. SPM usually is a self-limited condition, and prognosis is related to the underlying disorder. Consequently, with clinical improvement, aggressive investigation and follow-up x-ray rarely is warranted.

Asynchronous bilateral ovarian torsion.

BACKGROUND: Pediatric ovarian torsion (OT) is a serious condition, especially in cases of asynchronous bilateral ovarian torsion (ABOT). The authors sought to evaluate the predisposing factors for ABOT and to evaluate the most appropriate treatment for ovarian torsion. METHODS: The authors retrospectively reviewed the charts of patients with ovarian torsion between 1980 and 2002. Data collected included age at presentation, type and duration of symptoms, ultrasound scan findings, interval to surgery, procedures, pathology report, and follow-up. RESULTS: Seventy-six patients had adnexal torsion confirmed at surgery, 4 of whom had ABOT. The mean age was 10 years. The mean duration of complaints before hospitalisation and interval to surgery were 56 and 33 hours, respectively. Thirty-five patients had simple tubo-ovarian torsion (46%), including all the patients with ABOT (11.4%), and 41 had an ovarian pathology (54%). All patients with ABOT underwent salpingo-oophorectomy at the first episode. They presented earlier for the second episode and had a shorter interval to surgery where detorsion with oophoropexy was performed. Follow-up ultrasound scan showed perfusion and follicles in the remaining ovary. CONCLUSIONS: The diagnosis of ovarian torsion often is delayed, especially when a solid tumor is suspected. Conservative management should be strongly considered when there is no underlying ovarian pathology. Furthermore, oophoropexy of the ipsilateral and contralateral ovary should be considered to prevent a potentially devastating recurrence.

[Anatomic variations of the spermatic vein and endovascular treatment of left varicoceles: a pediatric series]. / Variantes anatomiques de la veine spermatique lors du traitement endovasculaire des varicocèles gauches: une série pédiatrique.

OBJECTIVE: To report on the high incidence of anatomical variants of the origin and course of the internal spermatic vein (ISV) discovered at the time of percutaneous embolization of left varicoceles in a pediatric population. METHODS: We reviewed retrospectively the 65 cases of left varicocele treated by percutaneous embolization (grade II and III) in our institution between 1990 and 2000. The course of the left renal vein (LRV), the origin of the ISV, and the number of ISVs and their pathway were recorded in all cases, according to the Bähren classification. RESULTS: In 37/65 (57%), the ISV was single and arose from a normal LRV (type I). The following variants were encountered: type V--circumaortic LRV 9/65 (14%); type IVb--intrarenal origin of ISV 8/65 (12%); type II--multiple ISV 5/65 (8%); and pelvic collaterals 6/65 (9%). CONCLUSION: Venous anatomical variants are frequently encountered (43%) at the time of left varicocele embolization in children. Such variants often impose some adjustments to the technique of embolization and, at times, hamper the procedure.