Simonart syndrome: a brief review of the literature and your own observation

Author(s):  
L.I. Pavlenko, S.A. Gribova

A case of prenatal diagnosis of Simonart syndrome (syndrome of amniotic constrictions) is presented. An amnioscopic operation was performed — dissection of the ligaments. There was an outpouring of amniotic fluid due to a high rupture of the amniotic membranes and premature birth at 28 weeks of gestation. During the external examination of the newborn, the data obtained during the ultrasound study was fully confirmed.

Author(s):  
E.N. Glavatskaya , O.V. Pribushenya , N.A. Venchikova

Two clinical cases of meconium peritonitis in the fetuses are presented. The diagnosis was made prenatally at 30+5 and 20+1 weeks of gestation. The main ultrasound signs were ascites, loop expansion and thickening of the intestinal wall, peritoneal calcifications, meconium pseudocysts. In one case, pregnancy was complicated by polyhydramnios. In both cases, the pregnancy ended in premature birth, followed by surgical treatment during the first days of life. A review of the literature on the topic are discussed the etiology, the spectrum and frequency of ultrasound signs suggesting this condition in the fetus, the effectiveness of prenatal diagnosis, the prognosis for the life and health of the newborn, as well as the impact of the quality and timeliness of the prenatal diagnosis on the management of pregnancy and timeliness of surgical care for the newborn.


Author(s):  
N.V. Mashinets, V.N. Demidov, Y.L. Podurovskaya et all

We present a review of the literature and own case report of prenatal diagnosis of epignatus at 31 week of gestation. Ultrasound of the fetus revealed a massive formation of solid structure of the blubber of the mouth. The size of epignatus was 3,0  3,0  2,8 cm, intratumoral blood flow was not determined, the amount of amniotic fluid was normal. With the progression of pregnancy the tumor size has not increased. After the birth the neonate was an independent breath. Carried out surgical treatment with a positive effect. The diagnosis of epignatus confirmed by histological examination.


Author(s):  
M.Y. Morozova, V.V. Zotov, M.S. Kovalenko et all

Despite the rapid technological advance, the expansion of prenatal ultrasound diagnosis, as well as the accumulation of experience by both domestic and foreign experts, prenatal recognition of true knots of the umbilical cord causes significant difficulties. Three cases of successful ultrasound diagnosis of true knots of the umbilical cord and brief review of the literature are presented.


Cancers ◽  
2021 ◽  
Vol 13 (5) ◽  
pp. 997
Author(s):  
Sophie E. van Peer ◽  
Corine J. H. Pleijte ◽  
Ronald R. de Krijger ◽  
Marjolijn C. J. Jongmans ◽  
Roland P. Kuiper ◽  
...  

In children presenting with a predominantly cystic renal tumor, the most likely diagnoses include cystic partially differentiated nephroblastoma (CPDN) and cystic nephroma (CN). Both entities are rare and limited information on the clinical and molecular characteristics, treatment, and outcome is available since large cohort studies are lacking. We performed an extensive literature review, in which we identified 113 CPDN and 167 CN. The median age at presentation for CPDN and CN was 12 months (range: 3 weeks–4 years) and 16 months (prenatal diagnosis–16 years), respectively. No patients presented with metastatic disease. Bilateral disease occurred in both entities. Surgery was the main treatment for both. Two/113 CPDN patients and 26/167 CN patients had previous, concomitant, or subsequent other tumors. Unlike CPDN, CN was strongly associated with somatic (n = 27/29) and germline (n = 12/12) DICER1-mutations. Four CPDN patients and one CN patient relapsed. Death was reported in six/103 patients with CPDN and six/118 CN patients, none directly due to disease. In conclusion, children with CPDN and CN are young, do not present with metastases, and have an excellent outcome. Awareness of concomitant or subsequent tumors and genetic testing is important. International registration of cystic renal tumor cohorts is required to enable a better understanding of clinical and genetic characteristics.


Author(s):  
Márcia Marinho ◽  
Sara Nunes ◽  
Cátia Lourenço ◽  
Mónica Melo ◽  
Cristina Godinho ◽  
...  

2008 ◽  
Vol 21 (5) ◽  
pp. 354-355 ◽  
Author(s):  
O. Török ◽  
K. Norregaard-Hansen ◽  
M. Szokol ◽  
K. Csécsei ◽  
A. Harsányi ◽  
...  

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