Presacral Medulloepithelioma: Case Report and Literature Review.

Medulloepithelioma is a rare early childhood tumor typically presenting in the intraocular region and neuroaxis. We report a rare case of a 2-year-old girl that presented with a peripheral medulloepithelioma in the presacral region. Examination of the tumor revealed that it lacked amplification of the 19q13.42 locus yet was positive for LIN28A. The patient was treated with intensive and high-dose chemotherapy as per 99703 protocol followed by complete surgical resection of the tumor and rapamycin maintenance and remains disease-free 5 years postinitial diagnosis. Ten previous cases were reported, including 5 patients who were alive disease free at the time of the publication. Optimal management of this rare condition is still controversial, particularly with regard to the respective role of chemotherapy and radiation.

Lung tattooing combined with immediate video-assisted thoracoscopic resection (IVATR) as a single procedure in a hybrid room: our institutional experience in a pediatric population.

BACKGROUND: Analysis of small pulmonary nodules in children poses an important diagnostic and therapeutic challenge for clinicians. OBJECTIVE: To review our experience of lung tattooing with immediate video-assisted thoracoscopic resection (IVATR) performed as a single procedure in a hybrid room for technical difficulties, complications and diagnostic yield of the procedure. MATERIAL AND METHODS: Retrospective analysis of 31 children (16 boys, 15 girls) who underwent lung tattooing of various lesions from January 2001 to July 2011. Data were collected from the Interventional Radiology database, Electronic Patient Chart (EPC) and PACS. RESULTS: A total of 34 lesions were treated in 31 children. Tattooing was performed on lung lesions with median size 3 mm and median depth 2 mm from pleura. Technical success was 91.1% and diagnostic yield was 100%. In seven children, it was combined with other interventional radiologic procedures. The median procedure time for lung tattooing and IVATR was 197 min. CONCLUSION: Lung tattooing with IVATR as a single procedure in a hybrid room is safe and effective in children with several inherent advantages, including avoiding the need to move the child from the interventional radiology suite to the operating room.

The very large recurrent postoperative scrotal hydrocele after pediatric inguinal hernia repair: a rare problem.

PURPOSE: To report the rare problem of a very large recurrent postoperative scrotal hydrocele after pediatric inguinal hernia repair. METHODS: From July 1969 to December 2007 inclusive, the charts of five boys mean age 8 years (range 7 months-16 years) with very large recurrent postoperative scrotal hydroceles were reviewed (Research Ethics Board approval 1000007698). RESULTS: In each case, there was a very large recurrent postoperative symptomatic scrotal hydrocele (3 right, 1 left, 1 bilateral). Their ages at the time of these recurrences were between 11 and 16 years; they were repaired within 2-18 months. The first four were repaired via a groin incision on finding no recurrent inguinal hernia. The last two were repaired through a scrotal approach because a groin ultrasound showed no recurrent inguinal hernia. There was 1 second recurrence which was successfully repaired. A followup of 1(1/2)-9 years revealed no recurrent hydrocele. CONCLUSIONS: The incidence of a very large recurrent symptomatic postoperative scrotal hydrocele after repair of a pediatric inguinal hernia is 0.06% and usually occurs in older boys. If ultrasonography of the groin is normal (since a recurrent inguinal hernia seems unlikely in such cases), this repair can usually be done via a scrotal approach.

Struggles, strengths, and strategies: an ethnographic study exploring the experiences of adolescents living with an ostomy.

BACKGROUND: Adolescents with IBD requiring ostomy surgery experience perioperative needs that may exceed those of patients experiencing other major abdominal surgery 1. This procedure requires ongoing and vigilant daily care and management. Gastrointestinal symptoms and complications impose psychological and social stresses on young patients 2, and the procedure results in body image changes and daily regimens of self-care. This study aimed to explore adolescents' experiences and quality of life following ostomy surgery. METHODS: Ethnographic interviews and a subsequent focus group were conducted with 20 adolescents with an ostomy or j-pouch being treated at the Hospital for Sick Children in Toronto, Canada. Interviews were transcribed verbatim and subjected to theme generation. RESULTS: Findings suggest that adolescents are profoundly affected by their ostomy. Adolescents convey strength as well as adjustment struggles. Identified impacts include body intrusion and body image changes, decreased independence, secrecy about the ostomy, adjustment over time, challenges for the family, and strategies for constructively moving forward. CONCLUSION: Implications address the importance of ensuring meaningful opportunities to understand and reframe the stresses of illness. An ongoing clinical challenge involves the promotion of a healthy self-esteem and psychosocial adjustment for these adolescents and their families. Finding effective ways to minimize stress and embarrassment and reframe personal shame, constitute important clinical priorities. Opportunities for peer support and family dialogue may assist in clarifying worries and easing the burden carried by these young persons. Flexible and adequately funded resources are advocated in fostering quality of life.

Necrotizing enterocolitis: assessment of bowel viability with color doppler US.

PURPOSE: To determine whether absence of bowel wall perfusion at color Doppler ultrasonography (US) is indicative of bowel necrosis in neonates with necrotizing enterocolitis (NEC). MATERIALS AND METHODS: This study was approved by the research ethics board, and informed consent was obtained. Sixty-two neonates enrolled in the prospective study underwent US of the bowel wall. Neonates were divided into two groups. Group A included 30 control subjects with gestational ages (GAs) ranging from 24 to 41 weeks. Group B included 32 neonates with GAs ranging from 24 to 40 weeks who were clinically proved to have or suspected of having NEC. All neonates in group B underwent abdominal radiography. Normative values were calculated in group A. In group B, the sensitivities and specificities of color Doppler US and abdominal radiography for detection of bowel necrosis were computed by using the modified Bell staging criteria for NEC as the reference standard. RESULTS: Two neonates were excluded from group B; thus, a total of 60 neonates were included in the study. In group A, bowel wall thickness ranged from 1.1 to 2.6 mm. Bowel wall perfusion was detected with color Doppler US in all 30 neonates. Color Doppler signals ranged from one to nine dots per square centimeter. Twenty-two of 30 neonates in group B received a diagnosis of NEC. Mild to moderate NEC was diagnosed in 12 neonates. Color Doppler US depicted an isolated segment of bowel-absent blood flow in two neonates; this finding was confirmed with laparotomy. In 10 neonates with severe NEC, color Doppler US depicted isolated or multiple segments of bowel with absent perfusion. Pneumoperitoneum was present in only four neonates. The remaining eight neonates at risk for NEC had no evidence of loops without perfusion at color Doppler US. The sensitivity of free air at abdominal radiography as a positive sign for severe NEC with necrotic bowel was 40% compared with the 100% sensitivity of absence of flow at color Doppler US (P = .03). CONCLUSION: Color Doppler US is more accurate than abdominal radiography in depicting bowel necrosis in NEC.

Retroperitoneal infantile fibrosarcoma: clinical, molecular, and therapeutic aspects of an unusual tumor.

The authors describe a patient with a large retroperitoneal infantile fibrosarcoma that responded well to preoperative chemotherapy, which subsequently facilitated the complete surgical resection of the mass. The patient had an unusual site of metastasis presumed to be to a regional lymph node. The histology on initial core biopsies was not classic but showed a round cell, myxoid pattern. The presence of the t(12;15)(p13;q25) and the fusion transcript ETV6-NTRK3 by RTPCR facilitated the diagnosis.

Tissue plasminogen activator for the treatment of intraabdominal abscesses in a neonate.

Fibrinolytic agents have been used successfully in the management of loculated empyema; however, their use in the treatment of intraabdominal abscesses is limited. The authors describe the case of a 4-week-old girl with intraabdominal abscesses secondary to intestinal perforation that were not amenable to percutaneous drainage, but were managed successfully with intracavitary administration of tissue-plasminogen activator. This case represents the first report in a human, in which tissue-plasminogen activator was used to facilitate percutaneous drainage of an intraabdominal abscess. It is also the first time a fibrinolytic agent has been used for this purpose in a child.