Primary malignant melanoma arising in a cystic necrotic ovarian teratoma.

Cystic ovarian teratomas are common tumours. Malignant melanoma developing in a teratoma, however, is an extremely rare diagnosis. To date, only 24 cases have been reported in the literature. A 56-year-old patient underwent laparotomy for an ovarian tumour. Histopathologic examination revealed a primary malignant melanoma within a cystic necrotic teratoma, possibly arising from uveal cells. An extraovarian primary melanoma could not been found. After 7 months of operation metastasis to the vertebral column occurred, 2 months later liver metastases were diagnosed. The patient died of the disease 14 months after initial diagnosis.

Treatment and postretention changes in dental arch width dimensions--a long-term evaluation of influencing cofactors.

The aim of the present long-term follow-up study of orthodontically treated patients was to analyze postretention changes in arch width dimension and to isolate factors that may serve as predictors of long-term prognosis. Pretreatment, end-of-treatment, and postretention (at least 10 years) models of 226 cases with different malocclusions were used to measure intercanine and intermolar width, arch length, sum of the mesiodistal dimension of the incisors, irregularity index, crowding, molar and canine relationship, overjet, and overbite. To assess the influence of initial and end-of-treatment alignment, kind of treatment (extraction versus nonextraction) and the amount of expansion in postretention stability, the sample was divided into different subgroups. The findings indicate that postretention arch width relapse occurred more frequently in the upper intermolar (25.8%) and lower intercanine region (23.9%) than in the lower intermolar (19.0%) and upper intercanine (13.8%) region. Pretreatment and posttreatment alignment as well as the kind of treatment and the amount of expansion were found to be influencing factors. The study concludes by proposing a reassessment of the definition of stability. The influence of the pretreatment anomaly, kind of treatment, amount of expansion, and posttreatment alignment on long-term stability should be recognized. Patients should be apprised of treatment limitations before treatments.

How to deal with the drop-out in clinical follow-up studies: results of a long-term follow-up study of orthodontically treated patients.

The drop-out in a long-term follow-up study of former orthodontically treated patients was analyzed to avoid biased results. Since structural conformity of the responders (n = 299) and the potential study population (n = 1464) cannot be presumed on the basis of the clinical results, roentgenologic results, and questionnaires, analysis of the participating patients, the follow-up method was used. By means of questionnaires sent to all nonresponders, a comparison of responding and nonresponding patient characteristics was completed to find a presumed selectivity bias. The comparison of the participating patients (n = 299) and the total sample revealed that the responders live closer to the health center, had a longer treatment time, and a shorter posttreatment interval. Compared with nonresponders (n = 266), the responders were satisfied with the treatment result and had a higher dental Intelligence Quotient (IQ, concerning tooth and jaw position). Although a definite answer to the question of validity of the examined subsample was difficult to give, the description and evaluation of the parameters that might be reasons for missing at random respectively, not missing at random was recommended.

Post-retention crowding and incisor irregularity: a long-term follow-up evaluation of stability and relapse.

The aim of the present long-term follow-up study of orthodontically-treated patients (mean: 15.7 years) was to analyse post-retention changes and to reveal factors which may play a role as predictors for long-term prognosis. Pretreatment, end-of-treatment, and post-retention models of 226 cases with all types of anomaly were used to measure intercanine and intermolar width, arch length, and sum of the mesiodistal dimension of the incisors, Irregularity Index, crowding, molar and canine relationship, overjet, and overbite. In order to assess the influence of sex, initial and end-of-treatment alignment, type of therapy, amount of tooth movement, and presence of third molars on the extent of post-retention changes, the total sample was divided into subgroups. Findings indicated that post-retention crowding and incisor irregularity increased more frequently in the mandible than in the maxilla. Pretreatment variables such as increased mesiodistal incisor dimension, severe crowding and incisor irregularity, arch length deficiency, arch constriction, and increased overbite as well as post-treatment spacing, arch expansion, increased arch length, and residual Class II or III molar relationships were found to be associated factors in the process of post-retention increase of crowding and incisor irregularity. 'Overexpansion' was found to be a factor in mandibular incisor relapse.

[A retrospective evaluation of the treatment of skeletal Class-II with removable appliances]. / Retrospektive Bewertung von Behandlungen der skelettalen Klasse II mit herausnehmbaren Geräten.

Pre- and post-treatment lateral radiographs of 120 former patients with skeletal class II, who had been treated with either an Andresen/Häupl type activator or bite jumping plates, were selected for this study. The evaluation results showed that bite jumping plates had a significant inhibition on the forward downward growth and the anterior rotation of the maxilla. The increase of the SNB-angle was significantly higher and there was also more posterior rotation of the maxilla in those patients, who had been treated with an activator. A two-way analysis of variance was carried out and it was possible to show that the independent treatment factors explained only 1/5 to 1/3 of the overall variance of the sample. The differences among the patients seem to depend more on individual growth patterns than just on the type of removable appliance.

Pulmonary hypertensive vasculopathy--no indication for corrective operation of isolated ventricular septum defect in babyhood.

The frequency of surgical closure of a ventricular septum defect (VSD) in the first year of life has risen from 10% to 30% in the last seven years in West Germany. Whereas there was a decrease of mortality in older children, mortality has stagnated at 8% for correction at age 12 months or less (early correction). Development of an irreversible pulmonary hypertensive vasculopathy (PHVP) and recurrent heart failure with growth disorders are given as reasons for operation in the first year of life. In the last six years, we have operated on 31 infants for isolated VSD with pulmonary hypertension (PH) and closed the VSD. In approximately equal preoperative pressure in the two ventricles with a pressure ratio of 0.88 +/- 0.16, there was an immediate fall in pressure in the right ventricle with pressure ratios of 0.43 +/- 0.24. Three patients (9.7%) died perioperatively. In the same period, a primary VSD closure was carried out in 120 children even after over 12 months with three deaths (2.5%). Pulmonary tissue removed in this operation did not show any PHVP in four patients (13%), grades 0 to I and I to II in 10 patients each (total 33%) and a PHVP grade II to III in six patients (20%). This PHVP is capable of full regression. An irreversible PHVP does not develop up to the end of the first year of life in isolated VSD with PH, so that the correction can be safely postponed to the beginning of the second year of life provided that recurrent heart failure with growth disorders does not compel earlier correction.(ABSTRACT TRUNCATED AT 250 WORDS)

[Hemodynamics and histology of lung blood vessels in 191 patients with atrioventricular canal]. / Hämodynamik und Histologie der Lungengefässe bei 191 patienten mit atrio-ventrikulärem Kanal.

The correlation between hemodynamic and histological changes of the small lung arteries was examined in 191 patients with atrio-ventricular canal. Lung biopsies were taken during palliative or corrective surgery. The histological findings were classified according to the Heath and Edwards-grading. The haemodynamic data were obtained within 3-4 months before operation (pulmonary/systemic ratios of systolic pressure, flow, and resistance as well as mean pulmonary artery pressure). The results indicated that the term atrioventricular canal must be divided into three different subgroups with regard of the development of HPVD: I. The incomplete atrio-ventricular canal (IAVC) showed a strong correlation between pulmonary hypertension and histological changes. II. The VSD-III (endocushion defect) developes HPVD in the first month of life similar to the complete av-canal. In about all children of this subgroup a serve pulmonary hypertension was found, therefore a correlation to the variety of histological findings could not be established. III. The CAVC was the largest subgroup of our patients. These patients had relatively the highest incidence of high-grade histological alterations. In 87% of the children with CACV we found histologically changes of the structure of the small arteries of the lungs mostly a hypertrophy of the media, but also alterations of the intima as proliferation of fibrosis. 88% of the children within the subgroup CAVC also hat a pulmonary hypertension with a systolic pressure ratio above 0.8.(ABSTRACT TRUNCATED AT 250 WORDS)

Lung diseases after bone marrow transplantation. Results of a clinical, radiological, histological, immunological and lung function study.

The case histories of 72 subsequently treated patients - 44 with acute leukemia, 10 with chronic myeloid leukemia, 16 with severe aplastic anemia and 2 with neuroblastoma - were analyzed after bone marrow transplantation (BMT) with respect to pulmonary diseases. Thirty-eight patients suffered from a total of 51 pulmonary complications, which led to death in 20. Of 13 patients, 3 died of bacterial pneumonia, all of them during granulocytopenia; 2 of 6 patients died of fungal pneumonia and 2 out of 3 of a mixed bacterial-mycotic infection. Adult respiratory distress syndrome (ARDS) led to death in 2 patients. A granulocyte count under 500/microliter correlated significantly (P less than 0.002) with the fatal outcome of bacterial, fungal and ARDS pneumonia as well as with bronchitis. Viral pneumonia led to death in 8 of 9 patients; in each there was a significant correlation (P less than 0.05) with graft-versus-host disease (GvHD). Patients with repeated episodes of pulmonary illness had significantly more chronic GvHD (P less than 0.05); several of these patients displayed a reduction in helper T cells and an increase in suppressor T cells in the peripheral blood. The natural killer (NK) cells were reduced and the percentage of activated NK cell level lay between 6% and 69%. B-cells were absent or deficient. These findings explain in part the absence of specific antibody reactivity. Five of these patients also contracted GvHD-associated obstructive bronchiolitis, which did not respond to therapy. Pulmonary infiltrates of unknown origin (including idiopathic interstitial pneumonia) occurred in 8 of the patients (11.1%), with a fatal outcome in 3 patients. Significant changes (P less than 0.05) in lung function after BMT appeared in the form of reduced vital capacity (VC) increased residual volume (RV) and an increase in RV expressed as the percentage of total lung capacity. Pulmonary diseases were the most common complication and cause of death in our patients after BMT.

[Anemic infarct of the liver following blunt abdominal trauma]. / Anämischer Leberinfarkt nach stumpfem Bauchtrauma.

Particularly in childhood, ischaemias of the liver are very rare. This is due to numerous collateral vessels and a dual blood supply of the liver. We, therefore, report on a 2-year old boy who suffered from an anaemic infarction of the whole left lobe of the liver after a fall. The clinical course and findings, including histology, are described. After resection of the left lobe the boy was free of symptoms.

[Echocardiographic diagnosis and successful resection of a left ventricular rhabdomyoma in the neonatal period]. / Echokardiographische Diagnose und erfolgreiche Resektion eines linksventrikulären Rhabdomyoms im Neugeborenenalter.

Among the rare heart tumours in childhood rhabdomyomas are mostly found in the neonatal period. They cause in 50% left- or right-sided obstruction. By means of 2D-echocardiography extent and relation to neighbouring structures can clearly be disclosed. Because of the severe natural history - mortality in the first year of life between 60 and 78% - successful operation was performed in a 2,5 kg weighing neonate.

[Hemodynamics and histology of the lung in 175 ventricular septal defects]. / Hämodynamik und Histologie der Lunge bei 175 Ventrikelseptumdefekten.

Lung biopsies obtained during operation from 175 patients with isolated or combined ventricular septal defect were examined histologically. The degree of the pulmonary hypertensive vasculopathy was classified according to the Heath and Edwards-grading. These morphological findings were compared to the hemodynamic data (pulmonary/systemic ratios of systolic pressure, resistance, and flow as well as mean pulmonary artery pressure). Pressure and resistance showed a good correlation to the histological changes. The combination of both variables, however, did not further improve the prediction of the severity of the vascular lesions. No relation was found between flow and pulmonary vasculopathy. Higher grades of the vasculopathy were associated with systolic pressure ratios exceeding at least 0.65, in most patients with even marked higher ratios. On the other hand an elevation of pulmonary blood pressure of pulmonary arterial resistance does not mean a more severe vasculopathy implicitly, since there were some patients with a considerable hemodynamic hypertension without at least severe histological changes. We conclude that patients with a systolic pulmonary pressure not exceeding about 75% of systemic pressure have a very low risk of a morphologically fixed hypertension. Therefore an urgent operation of such patients to prevent fixation of the pulmonary hypertension seems not to be necessary.

Malignant ovarian tumours during childhood.

Malignant ovarian tumours in childhood are rare. Germinal cell tumours are the most common tumours and are extremely malignant, with the exception of dysgerminomas. The possibilities of therapy have improved considerably in the last 3 to 4 years, thanks to combined chemo- and radiotherapy following surgical resection of the tumour. Exact staging at the beginning of therapy is very important. A second operation, primary or as a second look procedure, is recommended after emergency operations. Therapy should be carried out in pediatric oncology centres. Because of the invasive measures, medical and psychological care are necessary after the conclusion of therapy. The rarity of these tumours should prevail upon us to organize national and international therapeutic studies.

[Clinico-histologic-morphometric correlations in pulmonary arteries among patients with heart malformations ]. / Klinisch-histologisch-morphometrische Korrelationen an Lungenarterien bei angeborenen Herzfehlern.

To improve the evaluation of grades of pulmonary vascular lesions in congenital heart malformations, we studied the extent to which there is a measurable relationship between pressure conditions in the pulmonary circulation and the area of the media in small and medium-sized pulmonary arteries, and the possibility of a correlation between the subjective evaluation of grades of hypertensive pulmonary vascular lesions according to Heath and Edwards (1958). Material and methods. The lungs in 68 children (mean age, 22.7 months) with congenital malformations of the heart or great vessels resulting in pulmonary hypertension were examined. The lungs were fixed in a 4% formalin solution passed via the trachea under a constant filling pressure of 150 cm H2O. Peripheral as well as central tissue was removed from all lobes of the lungs; the specimens were stained with Elastica-van Gieson. The extent of hypertensive pulmonary vasculopathy was staged, without knowledge of the pressure conditions, according to the grading system developed by Heath and Edwards. The diameter and the area of all muscular arteries with diameters smaller or larger than 100 micron were measured using a semiautomatic measurement device for quantitative morphometric analysis (MOP/AM 01). Cardiac catheter values were available for all cases. The quotient of systolic pressures in the pulmonary artery and the aorta was taken as the measure of hemodynamic conditions in the pulmonary circulation. The Pearson-Bravais correlation coefficient (r) was computed from the respective area quotient and the corresponding pressure values. In addition, the coefficient of determination (r2) and regression functions were determined. Results. A linear correlation (r = 0.70) exists between the pressure quotient (Psyst. pulm. art./ Psyst. aorta) and the vessel area quotient (media area/total area). The correlation is expressed by the following functions: x = 1.89 y - 0.08 y = 0.26 x + 0.24 Using the Heath and Edwards grading, the following frequencies were obtained: Grade 0: 21, Grade I: 7, Grade II: 16, Grade III: 15, Grade IV: 6, and 3 children were undeterminable. No relationship exists between the grades and certain heart malformations. In spite of the fact that hypertensive vasculopathy becomes progressively more severe as the child grows older, we found four cases of Grade IV in children under the age of one. On the whole, a comparison of the measured area quotients and the Heath and Edwards grading showed a good correlation. Although we considered only pressure quotients and no other hemodynamic parameters, conclusions can be drawn about the operability of congenital heart malformations based on these findings. Using measured pressure values, the morphologic state of the pulmonary arteries can be approximately evaluated.

Morphologic aspects of Goldblatt hypertension in a newborn infant.

Hypertension (210 mm Hg), hyponatremia, hypochloremia, and hypokalemia were observed in an 11-day-old infant. After electrolyte repletion, blood pressure rose to 320 mm Hg in spite of administration of antihypertensive drugs. The child died of a massive cerebral hemorrhage on the 17th day of life. At autopsy, the left renal artery was partially thrombosed and the left kidney partially infarcted. The glomeruli in the preserved cortical areas were of normal size; the juxtaglomerular apparatuses markedly enlarged. The blood vessels were inconspicuous. Enlarged glomeruli, multiple ruptures in the mesangium, ectactic glomerular capillaries, and fragile capillary walls were found in the nonconstricted right kidney. Fibrinoid necrosis with occasional fibrin thrombi in the lumina was present in the arterioles. Pronounced intimal edema with fragmented cells and monocytes embedded in the intima were observed in the interlobular and larger arteries. The histologic findings in the blood vessels of the nonconstricted kidney resembled those found in the hemolytic-uremic syndrome (Gasser's syndrome).

Pulmonary arterial changes and hemodynamic parameters in isolated ventricular septal defect.

Lung biopsy specimens of 20 patients (aged 6 months to 29 years) with isolated ventricular septal defect (VSD) and various degrees of pulmonary hypertension were obtained at operation. The cross-section area of the pulmonary arteries was measured by a morphometrical method in each biopsy specimen and the grade of the hypertensive pulmonary vascular disease was determined according to the Health and Edwards classification. These findings were correlated to the pulmonary-to-systemic pressure ratio, to the pulmonary-to-systemic resistance ratio, and to the pulmonary-to-systemic flow ratio. The thickness of the media of pulmonary arteries (measured as area ratio) was well correlated to both the systolic blood pressure ratio and the resistance ratio resulting in a product moment correlation of r = 0.73. No direct relationship was found between the hypertrophy and the pulmonary-to-systemic flow ratio. Our quantitative morphometric data were well correlated with the qualitative Health and Edwards classification. Medial wall thickness was not significantly higher in grade III when compared to grade II. The mean values of the hemodynamic parameters were not significantly different in the Health and Edwards grade II and grade III groups. Our results indicate that the calculation of the mean pulmonary pressure and of the pulmonary-to-systemic resistance ratio are of no higher predictive value than the systolic pressure ratio. The classification of Health and Edwards appears to the sufficient for clinical evaluation.