Fetal therapy: state of the art. (25/166)

OBJECTIVE: To review our experience with the use of sonography in evaluating potential candidates for in utero fetal therapy performed at The Center for Fetal Diagnosis and Treatment at The Children's Hospital of Philadelphia. METHODS: This review article was designed to discuss open hysterotomy for the 4 fetal surgical procedures that have been performed at our institution. The procedures included surgical repair of myelomeningocele, resection of sacrococcygeal teratoma in fetuses with nonimmune hydrops, resection of an enlarging congenital cystic adenomatoid malformation that is not amenable to thoracoamniotic shunting, and tracheal clip occlusion for severe left congenital diaphragmatic hernia. RESULTS: For each surgical procedure, the use of sonography in the prenatal diagnosis of the congenital anomaly was detailed, as were indications for surgery and surgical procedures, postoperative monitoring and finally delivery, postnatal treatment, and long-term follow-up. Three of the procedures have been reasonably successful with rather dramatic results in some cases such that these techniques are still being performed. The 1 exception was open hysterotomy for the tracheal clip procedure for congenital diaphragmatic hernia, which has been abandoned. CONCLUSIONS: Fetal therapy is a rapidly evolving specialty, which is being practiced at several centers in this country. Sonography is an integral part of this specialty practice and has been used extensively in the diagnosis of some congenital anomalies that have debilitating or lethal consequences for the fetus. Technologic improvements in both sonography and magnetic resonance imaging have assisted tremendously in the many advances herein reported in the diagnosis and treatment of the above-described 4 congenital anomalies.  (+info)

Metastatic endodermal sinus tumor: CT appearances. (26/166)

A one-week-old boy had undergone resection of a sacrococcygeal benign cystic teratoma. At the age of 12 months, he had a serum alpha-fetoprotein level of 139,000 IU/ml and a recurrent pelvic mass which was removed, and the microscopic examination revealed endodermal sinus tumor. Postoperatively, massively enlarged inguinal lymph nodes and abdominal distention developed. Computerized tomography displayed enlarged inguinal lymph nodes, metastatic lesions in the liver, and a pelvic recurrent mass. He received BEP (bleomycin, etoposide, cisplatin) chemotherapy regimen, and a complete remission was achieved with a normal serum alpha-fetoprotein. Close follow-up and serum alpha-fetoprotein monitoring are mandatory after the resection of a sacrococcygeal teratoma.  (+info)

Effect of butanolic fraction of Desmodium adscendens on the anococcygeus of the rat. (27/166)

The chemical composition of plants can vary according to factors such as soil and time of collection. Desmodium adscendens (Sw.) D.C. var. adscendens (Papillionaceae) is a plant employed in the treatment of asthma in Ghana, Africa. Studies have shown that butanolic extract inhibits contraction of the ileum and trachea in guinea pigs. In Mato Grosso, this plant is used only in the treatment of ovarian inflammation. The objective of this work was to verify if the plant found in Mato Grosso also relaxes smooth muscle and to understand better its action. The cumulative application of the butanolic fraction relaxed the contraction maintained in the isolated anococcygeus of a rat, induced by high potassium, but not that induced by phenylephrine. Relaxation was not altered by methylene blue. The butanolic fraction reduced in a concentration-dependent way the maximum response of concentration-response curve to calcium in the anococcygeus muscle. The results suggest that the butanolic fraction acts, at least partly, through the blockade of voltage-sensitive Ca+2 channels.  (+info)

Prognostic value of tumor size, metastases, extension into bone, and increased tumor marker in children with malignant sacrococcygeal germ cell tumors: a prospective evaluation of 71 patients treated in the German cooperative protocols Maligne Keimzelltumoren (MAKEI) 83/86 and MAKEI 89. (28/166)

PURPOSE: To evaluate the prognostic value of metastases, extension into bone, and alpha-fetoprotein (AFP) elevation in children with malignant sacrococcygeal germ cell tumors (GCTs) prospectively collected in two cooperative Maligne Keimzelltumoren (MAKEI) protocols (83/86 and 89). PATIENTS AND METHODS: Between October 1983 and October 1995, 76 of 210 registered patients with sacrococcygeal primaries presented either with pure yolk sac tumor, embryonal carcinoma (EC), or yolk sac tumor and EC mixed with immature and mature teratoma elements. Stages T1 and T2 disease were diagnosed in 15 and 61 children, respectively, 41 patients had metastases, and 35 children presented with extension into bone. At diagnosis, 22 children had an AFP elevation of less than 10,000 ng/mL. Thirty-six children showed an AFP level between 10,000 and 100,000 ng/mL, and 12 patients had values of greater than 100,000 ng/mL. Five patients died of complication during treatment and were excluded from further evaluation. Seventy-one patients could be analyzed. RESULTS: The 5-year relapse-free survival rate (RFS, Kaplan-Meier) was 0.76 +/- 0.03 (54 of 71 patients; median observation time, 54 months after diagnosis). The RFS of patients with and without metastases was different, but not significantly so (0.71 v 0.82). The outcome of patients with extension into bone (n = 31) and without this extension (n = 40) was 0.71 versus 0.80 (RFS, 5 years). Above-normal AFP level had no prognostic significance (P =.52). CONCLUSION: In children with malignant sacrococcygeal GCTs treated with an intensive, short-interval, platinum-based regimen, the stage, extent of metastases, extension into bone, and AFP level had no prognostic significance.  (+info)

Laparoscopic presacral neurolysis for endometriosis-related pelvic pain. (29/166)

BACKGROUND: Some patients with endometriosis are candidates for sympathectomy of the superior hypogastric plexus. The objective of this paper is to describe our technique of laparoscopic presacral neurolysis for sympathectomy and to report 1 year results of the first 15 cases. METHODS: To achieve this objective in a prospective observational study of 1 year follow-up; we performed laparoscopic presacral chemical neurolysis with phenol in 15 patients with pelvic pain and minimal-moderate endometriosis. The main outcome measures were: the impact of treatment on pelvic symptom resolution, non-opioid analgesic consumption during menses, sexual performance and observed complications and side effects during 1 year follow-up. RESULTS: We noted a significant reduction in total pelvic symptom score as compared with baseline mean (SD) of 9.04 (1.2). The mean difference [95% confidence interval (CI)] of reduction was 5.7 (4.9-6.5), 5.8 (5.0-6.6) and 5.8 (4.9-6.6) from the baseline at the 3rd, 6th and 12th postoperative month (P < 0.001). We observed a significant improvement in Sabbatberg Sexual Rating Scale as compared with baseline mean (SD) of 30.9 (4.3). The mean difference (95% CI) of increase was 33.4 (30.3-36.4), 33.2 (30.1-36.2) and 33.2 (30.1-36.3) from the baseline at the 3rd, 6th and 12th postoperative month. We observed a significant reduction in analgesic consumption during menses in terms of total naproxen sodium tablets as compared with baseline mean (SD) of 8.9 (1.1). The mean difference (95% CI) of reduction in the total number of naproxen sodium 250 mg tablets was 6.5 (5.5-7.5), 6.7 (5.7-7.7) and 6.6 (5.6-7.6) from the baseline at the 3rd, 6th and 12th postoperative month. The most common side effect was constipation. CONCLUSION: Laparoscopic presacral neurolysis is feasible and simple. More data is needed to support its efficacy and safety.  (+info)

Venous pulsation in the fetal left portal branch: the effect of pulse and flow direction. (30/166)

OBJECTIVE: To determine whether the waveform in the left portal branch is reciprocal to the waveform found in the ductus venosus and umbilical vein due to difference in pulse direction compared to flow. METHODS: Ten fetuses (gestational age, 18-33 weeks), six with intrauterine growth restriction, three with non-immune hydrops and one with sacrococcygeal teratoma, were examined using ultrasound imaging and pulsed Doppler. Techniques were adjusted to record simultaneously the waveform from neighboring sections of the veins, relate wave components to each other and determine degree of pulsatility. The corresponding vessel diameters were determined. ANOVA with t-test or Wilcoxon signed rank test was used to compare paired measurements. RESULTS: Pulsation in the left portal branch was noted in all fetuses. The pulsatility index was higher than in the umbilical vein (P = 0.005) and the diameter smaller (P = 0.001). In the left portal branch the atrial contraction wave appeared as a velocity peak while there was a nadir during ventricular systole. Simultaneous recordings showed that the waveform was reciprocal to that found in the ductus venosus and umbilical vein. In three cases an augmented pulsatility represented a pendulation of blood in the left portal branch with time-averaged velocity near zero. CONCLUSIONS: The velocity waveform recorded in the left portal vein is an inverse image of that in the ductus venosus, proving that pulse wave and blood flow run in the same direction in the left portal vein. Low compliance (i.e. small diameter) is probably a main reason for the high incidence of pulsation in this vein. Time-averaged velocity near zero recorded in three fetuses indicates that this area acts also as a watershed.  (+info)

Coccygeal chordoma in a dog. (31/166)

A 4-cm diameter spherical mass was removed from the region of the second coccygeal vertebra of a 6-year-old female Doberman Pinscher dog. The mass had been present for 7 months and was described as multilobular and smooth. Histologically, the neoplasm consisted of small spindle-shaped cells surrounding lobules of large vacuolated polygonal cells. Mucin was present within most lobules. The large polygonal cells stained positively with antibodies against vimentin, cytokeratin, and S-100 protein. Although the spindle-shaped cells stained positively with antibodies against vimentin, they only occasionally contained cytokeratin. The histology and immunohistochemistry are consistent with that reported for chordoma. Although 4 chordomas have previously been reported in dogs, this is the first case in which immunohistochemistry has been used to support the histological diagnosis. Neither recurrence nor metastasis was reported 10 months after surgical excision.  (+info)

Extradural myxopapillary ependymoma with sacral osteolysis--a case report. (32/166)

Extradural ependymomas arising from filum terminale externa in the sacrococcygeal region are very rare. Since 1937, out of 58 cases reported in English literature, only 13 cases of tumor located presacrally in the retrorectal space, have been reported. The authors report a 38 yrs old male with a primary ependymoma of the sacrococcygeal region causing extensive sacral destruction.  (+info)