Spontaneous intrauterine umbilical artery thrombosis leading to severe fetal growth restriction.

Intrauterine thrombosis of umbilical cord vessels is a rare event (2.5-4.5/10,000) and usually followed by poor fetal outcome. We present the rare case of spontaneous intrauterine thrombosis of an umbilical artery leading to severe intrauterine growth restriction (IUGR) and provide clinical and pathological findings. A 28-year-old nulliparous third gravida was referred to our institution because of IUGR at 32+4 weeks of gestation. Fetal growth had been appropriate until the 31st week of gestation and had stopped thereafter. There were no signs of abruption of the placenta and no structural abnormalities except an absent paravesical colour Doppler flow in the region of the right umbilical artery. Other Doppler measurements, karyotype and TORCH serology were normal. Intermittent non-reassuring fetal heart rate led to cesarean section at 34+3 weeks of gestation. A healthy girl with measurements on the 3rd centile was born (weight of 1,590 g, length of 41 cm and head circumference of 29 cm). Gross examination displayed an elongated, highly twisted umbilical cord with a length of 70 cm, central insertion and three umbilical vessels. Microscopic examination confirmed the diagnosis of umbilical artery thrombosis along the entire length of the umbilical cord. Calcification within the thrombus and microcalcification in occluded chorionic vessels were observed as well as hemorrhagic endovasculitis and endangiopathia obliterans in the stem villi arteries. This fetal thrombotic vasculopathy (FTV) comprised about 40% of the parenchyma. The coagulation parameters and blood counts of the mother and the infant were normal apart from transient neonatal thrombocytopenia. The reason for thrombosis remained unclear but could be attributed to the elongated and highly twisted umbilical cord. Intrauterine arterial thrombosis may cause severe IUGR. This condition might be detectable by ultrasound in the course of an IUGR workup, especially when no other reasons can be found.

Extraosseous schwannoma of the mental nerve clinically simulating intraosseous.

We report a case of a benign neurilemmoma arising from the right mental nerve. Schwannomas are rare neurogenic tumours that originate from Schwann cells of the peripheral nervous system. Frequent locations are the head and neck region. Most of the tumours occur in the soft tissue whereas intraosseous schwannomas are rare. This case report deals with a young patient who was referred to our hospital with an unidentified fast-growing tumour located mainly in the premolar region of the right mandible. The tumour presented as an expansive, unilocular, well defined, radiolucent lesion on orthopantomography. No expansion of the mandibular canal could be seen. Computerized tomography scans of the mandible helped to identify the solid nature of the tumour. A biopsy was necessary to make the final diagnosis and the tumour was then excised surgically. Postoperative magnetic resonance imaging scans and a histological examination of the surgical specimen showed no signs of neurofibromatosis type 2.

Ex vivo expanded unselected peripheral blood: progenitor cells reduce posttransplantation neutropenia, thrombocytopenia, and anemia in patients with breast cancer.

The safety and efficacy of administering ex vivo expanded peripheral blood progenitor cells (PBPC) to patients with breast cancer who undergo high-dose chemotherapy and PBPC transplantation was investigated. Unselected PBPC were cultured in gas-permeable bags containing 1-L serum-free media, granulocyte colony-stimulating factor, stem cell factor, and pegylated megakaryocyte growth and development factor for 9 days. Cell dose cohorts were assigned to have between 2 and 24 x 10(9) PBPC cultured at 1, 2, or 3 x 10(6) cells/mL. Twenty-four patients received high-dose chemotherapy followed by infusion of the cultured PBPC and at least 5 x 10(6) CD34(+) uncultured cryopreserved PBPC per kilogram. No toxicities resulted from infusions of the ex vivo expanded PBPC. The study patients had shorter times to neutrophil (P =.0001) and platelet (P =.01) recovery and fewer red cell transfusions (P =.02) than 48 historical controls who received the same conditioning regimen and posttransplantation care and at least 5 x 10(6) CD34(+) PBPC per kilogram. Improvements in all these endpoints were significantly correlated with the expanded cell dose. Nine of 24 (38%) patients recovered neutrophil counts above 500/microL by day 5 or 6 after transplantation, whereas none of the controls had neutrophil recovery before the eighth day. Seven (29%) patients had neutropenia for 3 or fewer days, and 9 (38%) patients did not experience neutropenic fevers or require broad-spectrum antibiotics. Therefore, ex vivo expanded PBPC are capable of ameliorating posttransplantation neutropenia, thrombocytopenia, and anemia in patients receiving high-dose chemotherapy.

[Accessory mitral valve tissue as a rare cause of subaortic stenosis and valvular aortic insufficiency]. / Akzessorisches Mitralklappengewebe als seltene Ursache einer Subaortenstenose und valvulären Aorteninsuffizienz.

An unusual case of subaortic stenosis and aortic regurgitation caused by accessory mitral valve tissue in a 10 year old boy is reported. Two-dimensional and Doppler echocardiography revealed the characteristic feature of a mobile, parachute-like mass in the left ventricular outflow tract pro-lapsing into the aortic valve during systole and, thus, producing a systolic pressure gradient of 70 mm Hg between the left ventricle and aorta and causing mild aortic regurgitation. The accessory valve tissue was completely excised via an aortotomy without injury to the normal mitral and aortic valves. Two dimensional echocardiography provides excellent morphological information about the relationship between the accessory mitral valve tissue and the mitral and aortic valves, respectively. Accurate preoperative evaluation by two-dimensional echocardiography facilitates the successful surgical management of this rare condition.

Age dependency of selenium and cadmium content in human liver, kidney, and thyroid.

Selenium and cadmium concentrations were investigated in 60 autopsy tissue samples obtained from fetal life up to adulthood (defined in this study as 25-87 y of age) in Styria, a moderately industrialized region in Austria that has a low selenium supply. During the first 2 y after birth, median liver selenium concentrations were slightly lower (i.e., 1.5 nmol/g wet weight) than concentrations found in fetal life (i.e., 2.9 nmol/g) and adulthood (2.1 nmol/g). Whereas in the fetal period median selenium content in the kidney cortex (2.1 nmol/g) and the thyroid gland (1.6 nmol/g) was lower than that found in the liver, the reverse was true for adults (i.e., kidney, 5.5 nmol/g; thyroid, 4.3 nmol/g). Tissue cadmium concentrations approached 0 during gestation. Accumulation in the kidney and liver commenced immediately after birth. In the thyroid gland of adults, significantly higher concentrations of cadmium were found. Median concentrations in adults showed no statistical significant age dependency (i.e., liver, 7.6 nmol/g; kidney, 59.8 nmol/g; thyroid, 11.2 nmol/g). In summary, the data revealed very low tissue selenium concentrations and low cadmium burdens for the Styrian population that was not exposed occupationally.

[Spontaneous splenic rupture during portal triad clamping]. / Spontane Milzruptur bei Einflussokklusion der Leber.

A 45-year-old woman underwent a resection for a hepatic metastasis of the liver caused by a carcinoma of the colon. Shortly after the Pringle maneuver severe hemorrhage from the upper left quadrant of the abdomen was observed. Exploration revealed a spontaneous parenchymal laceration of the spleen.

Symmetric hypoplasia of the temporal cerebral lobes in an infant with glutaric aciduria type II (multiple acyl-coenzyme A dehydrogenase deficiency).

Symmetric hypoplasia of the temporal cerebral lobes was demonstrated by magnetic resonance imaging of the brain in a macrocephalic male patient with glutaric aciduria type II within the first week of life. Psychomotor development was normal until the age of 11 months, when the patient died of sudden cardiac arrest. Autopsy revealed symmetric hypoplasia of the temporal cerebral lobes with loss of axons and hypomyelination in the temporal medullary layers.

Atypical presentation of Kawasaki disease in an infant.

A 7-month-old male infant with clinical symptoms of severe toxic shock syndrome died on day 9 of illness. At autopsy, demonstration of coronary vasculitis together with thrombosis of the left coronary artery revealed the true diagnosis of atypical Kawasaki disease. The marked similarity in many clinical features makes the distinction between these two diseases difficult when atypical clinical presentation of Kawasaki disease is present.

[Iodine content of thyroid tissue in the Styrian population]. / Untersuchungen zum Jodgehalt von Schilddrüsengewebe in der steirischen Bevölkerung.

The iodine concentration was determined in 89 thyroid tissue samples of post-mortem examination material of thyroid healthy persons in dependency to the age. Before the 22nd week little iodine is stored in the fetal thyroid gland (median and range: 24 micrograms/g wet weight [0 to 27 micrograms/g]). From the 23rd week on the concentration is significantly higher (p = 0.004). There is a further increase of iodine concentration in the first 6 months of life, with a median concentration of 171 micrograms/g (75 to 808 micrograms/g). With a median of 599 micrograms/g (464 to 699 micrograms/g) the iodine concentration in the second year of life is comparable with that of adults (640 micrograms/g [218 to 2772 micrograms/g]). From the 60th year of life the median concentration is 462 micrograms/g (155 to 2100 micrograms/g), but this decrease is not statistically significant. Additionally the iodine concentration was determined in 7 topographically defined regions of 28 thyroid glands. The median iodine concentration did not differ in the 7 regions, but the iodine content in one and the same thyroid gland differed from region to region. Therefore in the healthy thyroid gland no regions exist, which have generally stored less iodine than others. However, no conclusion can be drawn from the iodine concentration of one biopsy to the whole iodine concentration of the organ.

Congenital generalized cystic lymphangiomatosis diagnosed by prenatal ultrasound.

We describe a previously unknown congenital abnormality of the lymphatic system, characterized by multiple cystic lesions of the lower extremities and thorax. Diagnosis was made by ultrasound in the second trimester. The ultrasound findings, pathology results, and differential diagnosis are presented.

[Sacrococcygeal teratoma. Prenatal diagnosis and prognosis]. / Das Sacrococcygeale Teratom. Präpartale Diagnose und Prognose.

The routine use of ultrasonics during pregnancy allows an early diagnosis of a sacrococcygeal teratoma (SCT) with a high degree of certainty. The perinatal management is shown in a four case example prenatally diagnosed. The assessment of N-Acetylcholinesterase in the amniotic fluid in cases of cystic teratomas does not allow a safe differentiation from a myelomeningocele. A puncture of the tumor can be helpful for the verification of the diagnosis. Cells of all three germ layers may be found in the cytological fluid. The sonomorphological differentiation into solid and cystic tumors may play a significant role for the intrauterine growth. Even when diagnosed very early, cystic tumors do not compromise the intrauterine growth. Large solid tumors may lead to hydrops fetalis and intrauterine death. Furthermore, the prognosis depends on concomitant malformation as well as on the intracorporal spreading of the tumor. Therefore, a careful ultrasonic examination allows a good prenatal prognosis as to the severity of the malformation. Delivery is done by caesarean section in cases of large cystic-solid tumors. Small teratomas as well as most cystic tumors allow vaginal delivery after having been punctured previously.

[Severe fetopathy--toxoplasma despite serologic screening. A case report]. / Schwere Fetopathia--Toxoplasmotica trotz serologischem Screening. Ein Fallbericht.

Toxoplasmosis is a special prenatal infection because it is susceptible to medication. Serologic screening and early diagnosis of a new infection is a prerequisite for successful treatment, which protects the fetus from infection. A case of a twenty-two year old gravida-II shows, that infection is possible despite negative initial screening. A new infection was diagnosed because of seroconversion at a routine screening at the end of the second trimester. An ultrasound scan showed severe hydrocephalus, hepatomegaly, and ascites. Labor was induced in the 33rd gestational week. Conatal toxoplasmosis was confirmed by histologic and microbiologic studies of the placenta and membranes. We discuss the difficulties resulting from negative primary serology.