[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.

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.

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.