Introduction

Most underlying diseases for abdominal pain in children are not dangerous. However some require rapid diagnosis and treatment, such as acute ovarian torsion or appendicitis. Since reaching a diagnosis can be difficult, and delayed treatment of potentially dangerous diseases might have significant consequences, exploratory laparoscopy is a diagnostic and therapeutic option for patients who have unclear and potentially hazardous abdominal diseases. Here we describe a case where the anomaly could not be identified using a laparoscopy in an adolescent girl with acute abdomen.

Case presentation

A 13-year old postmenarchal caucasian female presented with an acute abdomen. Emergency sonography could not exclude ovarian torsion. Accurate diagnosis and treatment were achieved only after an initial laparoscopy followed by a laparotomy and after a magnetic resonance imaging scan a further laparotomy. The underlying disease was hematometra of the right uterine horn in a uterus didelphys in conjunction with an imperforate right cervix.

Conclusion

This report demonstrates that the usual approach for patients with acute abdominal pain may not be sufficient in emergency situations.

Purpose

To determine the response rate to oral capsular fenretinide in children with recurrent or biopsy proven refractory high-risk neuroblastoma.

Experimental Design

Patients received 7 days of fenretinide: 2475 mg/m2/day divided TID (<18 years) or 1800 mg/m2/day divided BID (≥18 years) every 21 days for a maximum of 30 courses. Patients with stable or responding disease after course 30 could request additional compassionate courses. Best response by course 8 was evaluated in Stratum 1 (measurable disease on CT/MRI +/− bone marrow and/or MIBG avid sites) and Stratum 2 (bone marrow and/or MIBG avid sites only).

Results

Sixty-two eligible patients, median age 5 years (range 0.6–19.9), were treated in Stratum 1 (n=38) and Stratum 2 (n=24). One partial response (PR) was seen in Stratum 2 (n=24 evaluable). No responses were seen in Stratum 1 (n=35 evaluable). Prolonged stable disease (SD) was seen in 7 patients in Stratum 1 and 6 patients in Stratum 2 for 4–45+ (median 15) courses. Median time to progression was 40 days (range 17–506) for Stratum 1 and 48 days (range 17–892) for Stratum 2. Mean 4-HPR steady state trough plasma concentrations were 7.25 µM (coefficient of variation 40–56%) at day 7 course 1. Toxicities were mild and reversible.

Conclusions

Although neither stratum met protocol criteria for efficacy, 1 PR + 13 prolonged SD occurred in 14/59 (24%) of evaluable patients. Low bioavailability may have limited fenretinide activity. Novel fenretinide formulations with improved bioavailability are currently in pediatric Phase I studies.

Background

In large bowel intussusceptions, several reports described US signs which are associated with a lower likelihood of reducibility by hydrostatic or pneumatic enema. US may demonstrate echogenic dots or lines (foci) in the bowel wall, which may indicate ischemic bowel.

Objective

To determine the presence of echogenic intramural and subserosal foci and trapped gas in large bowel intussusceptions and to correlate with the reducibility.

Material and methods

Between 2001 and 2008, 74 consecutive US examinations of large bowel intussusception were retrospectively evaluated by 2 pediatric radiologists for intramural, subserosal echogenic foci (gas) or trapped gas in the intussusception. The sonographic findings were correlated with the hydrostatic or pneumatic reducibility.

Results

In 73 US examinations with large bowel intussusception, 56 (76%) were reducible and 17 (23%) were not reducible. Eight out of ten (80%) patients with intramural gas, six out of eleven (56%) with subserosal gas and nine out of fourteen (64%) with intramural and/or subserosal gas had non reducible intussusceptions. Univariate analysis of the above predictors found intramural gas and/or subserosal gas significantly influential. Multivariate analysis however proved only intramural gas to be the significant predictor, in presence of subserosal gas, of fluoroscopic reducibility

Conclusion

Having sonographically detected intramural gas in large bowel intussusception, significantly decreases the chance of intussusception reduction