Consult and procedure incidence outcomes following establishment of a fertility preservation program for children with cancer.

PURPOSE: Fertility is a quality of life outcome adversely affected by cancer therapy. Many childhood cancer patients, however, are not offered options to preserve their fertility. Providers acknowledge difficulty discussing impaired fertility to patients due to lack of knowledge of available options. Our objective was to review the impact of a pediatric multidisciplinary fertility preservation program on providers' fertility preservation counseling and discussion of options. METHODS: A retrospective medical chart review was conducted for pediatric cancer patients prior to and following program establishment. Fertility preservation discussions, consults, and incidence were noted. Following filtering and stratification, 198 and 237 patients were seen prior to and following program establishment, respectively. RESULTS: Following program establishment, provider-patient discussions of impaired fertility (p = 0.007), fertility preservation consults (p = 0.01), and incidence of fertility preservation procedures (p < 0.001) increased among patients. Furthermore, the number of patients who received fertility preservation consults after receiving gonadotoxic treatment decreased (p < 0.001). This trend was particularly noted in pre-pubertal and female patients, for whom fertility preservation options are limited without an established program. CONCLUSION: The establishment of a formal program greatly improved access to fertility preservation consults and procedures in children with cancer.

Is there a role for fetal interventions in gastroschisis management? - An updated comprehensive review.

We conducted a comprehensive evidence-based review on the epidemiology and current standard of care of gastroschisis management as well as the pathophysiology, rationale and feasibility of fetal therapy as a viable alternative. Gastroschisis is a periumbilical abdominal wall defect characterized by abdominal viscera herniation in utero. It affects 4 in 10 000 live births, but the prevalence has steadily increased in recent years. Gastroschisis is typically diagnosed on routine second-trimester ultrasound. The overall prognosis is favorable, but complex gastroschisis, which accounts for about 10% to 15% of cases, is associated with a higher mortality, significant disease burden and higher healthcare costs due to long- and short-term complications. The current standard of care has yet to be established but generally involves continued fetal surveillance and multidisciplinary perinatal care. Postnatal surgical repair is achieved with primary closure, staged silo closure or sutureless repair. Experimental animal studies have demonstrated the feasibility of in utero closure, antiinflammatory therapy and prenatal regenerative therapy. However, reports of early preterm delivery and amnioinfusion trials have failed to show any benefit in humans. Further experimental studies and human trials are necessary to demonstrate the potential benefit of fetal therapy in gastroschisis.

Fetoscopic Therapy for Severe Pulmonary Hypoplasia in Congenital Diaphragmatic Hernia: A First in Prenatal Regenerative Medicine at Mayo Clinic.

OBJECTIVE: To introduce the prenatal regenerative medicine service at Mayo Clinic for fetal endoscopic tracheal occlusion (FETO) care for severe congenital diaphragmatic hernia (CDH). PATIENTS AND METHODS: Two cases of prenatal management of severe CDH with FETO between January and August 2017 are reported. Per protocol, FETO was offered for life-threatening severe CDH at between 26 and 29 weeks' gestation. Regenerative outcome end point was fetal lung growth. Gestational age at procedure and maternal and perinatal outcomes were additional monitored parameters. RESULTS: Diagnosis by ultrasonography of severe CDH was based on extremely reduced lung size (observed-to-expected lung area to head circumference ratio [o/e-LHR], eg, o/e-LHR of 20.3% for fetus 1 and 23.0% for fetus 2) along with greater than one-third of the liver herniated into the chest in both fetuses. Both patients underwent successful FETO at 28 weeks. At the time of intervention, no maternal or fetal complications were observed. Postintervention, fetal lung growth was observed in both fetuses, reaching an o/e-LHR of 62.7% at 36 weeks in fetus 1 and 52.4% at 32 weeks in fetus 2. The balloons were removed successfully at 35 weeks and 4 days by ultrasound-guided puncture in the first patient and at 32 weeks and 3 days by ex utero intrapartum therapy-to-airway procedure in the second patient. Postnatal management followed standard of care with patch CDH therapy. At discharge, one patient was breathing normally, whereas the other required minimal nasal cannula oxygen support. CONCLUSION: The successful launch of the first fetoscopic therapy for CDH at Mayo Clinic reveals its feasibility and safety, with early signs of benefit documented by fetal lung growth and reversal of severe pulmonary hypoplasia. TRIAL REGISTRATION: clinicaltrials.gov Identifier: G170062.

Adenocarcinomas of the jejunum and ileum: a 25-year experience.

Adenocarcinomas of the jejunum and ileum are rare gastrointestinal malignancies. Because few large published experiences exist, we reviewed patients with jejunal and ileal adenocarcinoma treated at our institution over the last 25 years. Between January 1976 and December 2001, 77 patients had an operation for a jejunal or ileal adenocarcinoma. Records were retrospectively reviewed for patient, tumor, and treatment variables. Factors affecting disease recurrence and patient survival were investigated. Fifty-two of the adenocarcinomas (67%) occurred in the jejunum and 25 occurred in the ileum (33%). Mean patient age was 63 +/- 14 years. Segmental bowel resection was performed in 50 patients (65%) with curative intent. Palliative operative procedures including resection or bypass were performed in 27 patients (35%). One (1%) patient had stage I, 18 (23%) stage II, 19 (25%) stage III, and 39 (51%) stage IV adenocarcinoma at diagnosis. Postoperatively, 12 patients had palliative and 18 adjuvant chemotherapy (n = 30), radiation therapy (n = 1), or combination treatment (n = 7). Median patient survival was 19 months. Sixty-six percent of patients who had a curative operation had a tumor relapse. Tumor stage had a highly significant effect (P < 0.0001) on median survival (72 months for stage I and II, 30 months for stage III, and 9 months for stage IV disease). In multivariate analysis of patients having curative treatment, tumor recurrence (P < 0.0001), stage (P < 0.0002), and weight loss (P < 0.001) were significant negative prognostic indicators. Most patients with adenocarcinoma of the jejunum or ileum present with advanced disease. Tumor stage, disease recurrence, and weight loss predicted patient outcome following a curative operation. Early recognition of these tumors requires a high index of suspicion.