Isolated low grade prenatally detected unilateral hydronephrosis: do we need long term follow-up?

ABSTRACT Purpose: To assess the need for postnatal evaluation and the medium term outcome in patients with isolated unilateral low grade prenatally detected hydronephrosis. Materials and Methods: We prospectively selected 424 patients (690 kidney units) with a prenatal diagnosis of urinary tract dilatation between 2010 and 2013. We included only those patients with isolated unilateral low-grade hydronephrosis who underwent at least 2 postnatal ultrasound examinations. The Society for Fetal Urology (SFU) grading system was utilized for assessment of the hydronephrosis. We excluded patients with bilateral dilation or other urological abnormalities. The fate of hydronephrosis including resolution, stability or worsening was documented. Results: A total of 66 infants (44 boys and 22 girls) with antenatally diagnosed unilateral urinary tract dilation (23 right and 43 left) were identified. Ultrasounds showed SFU grade 1 hydronephrosis in 32 patients (48%) and SFU grade 2 hydronephrosis in 34 (52%). After a mean follow-up period of 32 months (range 12 to 60), 37 patients (56%) had complete resolution of hydronephrosis while the remaining 29 were stable (44%). None of our patients developed UTIs during follow-up and none required surgical intervention. Conclusions: Prenatally detected, isolated unilateral low-grade hydronephrosis usually have a favorable prognosis. All cases in our cohort showed either stability or resolution of hydronephrosis without any harmful consequences. Based on our findings on medium-term in this category of patients, long-term follow-up is not warranted.

Maternal administration of cannabidiol promotes an anti-inflammatory effect on the intestinal wall in a gastroschisis rat model

Gastroschisis (GS) is an abdominal wall defect that results in histological and morphological changes leading to intestinal motility perturbation and impaired absorption of nutrients. Due to its anti-inflammatory, antioxidant, and neuroprotective effects, cannabidiol (CBD) has been used as a therapeutic agent in many diseases. Our aim was to test the effect of maternal CBD in the intestine of an experimental model of GS. Pregnant rats were treated over 3 days with CBD (30 mg/kg) after the surgical induction of GS (day 18.5 of gestation) and compared to controls. Fetuses were divided into 4 groups: 1) control (C); 2) C+CBD (CCBD); 3) gastroschisis (G), and 4) G+CBD (GCBD). On day 21.5 of gestation, the fetuses were harvested and evaluated for: a) body weight (BW), intestinal weight (IW), and IW/BW ratio; b) histometric analysis of the intestinal wall; c) immunohistochemically analysis of inflammation (iNOS) and nitrite/nitrate level. BW: GCBD was lower than CCBD (P<0.005), IW and IW/BW ratio: GCBD was smaller than G (P<0.005), GCBD presented lower thickness in all parameters compared to G (P<0.005), iNOS and nitrite/nitrate were lower concentration in GCBD than to G (P<0.005). Maternal use of CBD had a beneficial effect on the intestinal loops of GS with decreased nitrite/nitrate and iNOS expression.

Comunicación interventricular en el feto / Intraventricular communication in fetus

Se reporta el caso de una gestante que en ultrasonido realizado en el segundo trimestre de la gestación se sospecha un defecto de septación ventricular, se confirmó la presencia de una comunicación interventricular tipo perimembranosa con extensión muscular y mal alineamiento del tabique interventricular, que trajo como consecuencia disminución del tamaño del tracto de salida del ventrículo izquierdo, sin causar obstrucción. En el estudio posnatal se evidenció la patología señalada así como la presencia de una comunicación interauricular y persistencia del conducto arterioso. Las ilustraciones empleadas se corresponden con imágenes grabadas en el Cardiocentro Pediátrico William Soler y en su servicio de anatomía patológica. Se trata del primer caso reportado en nuestro país

The case of a pregnant with suspicion of a ventricular septate defect according to ultrasound carried out during the second trimester of pregnancy is reported confirming the presence of a peri-membranous intraventricular communication with muscular lengthening and a malalignment of interventricular septum leading to a decrease in the size of outlet tract from the left ventricle without obstruction. In the postnatal study it was evidenced the marked pathology, as well as the presence of a interauricular communication and persistence of arterial conduct. Illustrations used are in correspondence with the images taped in the William Soler Children Heart Center and in its pathological anatomy service

Ressonância nuclear magnética fetal: aplicabilidade e indicações no período pré-natal / Fetal nuclear magnetic resonance: applicability and indications in the prenatal period

A ultrassonografia é o exame de imagem de primeira escolha para rastreamento de doenças fetais devido a sua utilidade, aplicabilidade e baixo custo. Contudo, ocasionalmente, seus achados são inconclusivos ou insuficientes para guiar o tratamento, e a ressonância nuclear magnética (RNM) funciona como ferramenta complementar. A aplicabilidade da RNM fetal inclui indicações neurológicas e não neurológicas. Das indicações neurológicas, a RNM fetal auxilia o diagnóstico de: ventriculomegalia, agenesia de corpo caloso, anormalidades da fossa posterior e malformações do desenvolvimento do córtex cerebral. Dentre as dismorfias fetais não neurológicas, a RNM fetal auxilia o diagnóstico de complicações de gestações gemelares monocoriônicas, hérnia diafragmática congênita, sequestros pulmonares, malformações adenomatoides císticas congênitas e obstrução de vias aérea. Objetivou-se com o presente artigo apresentar a aplicabilidade e as indicações da RNM no diagnóstico de malformações, auxiliando o ginecologista e obstetra no entendimento deste novo recurso diagnóstico na área de medicina fetal. O uso da RNM fetal fornece informações adicionais à ultrassonografia, autorizando mudanças no aconselhamento ao paciente e no seu tratamento.

Ultrasonography is the examination of image of first choice for tracking of fetal illnesses due to its utility, applicability and low cost. However, occasionally, its findings are inconclusives or insufficient to guide the treatment, so the magnetic resonance imaging (MRI) functions as a complementary tool. The applicability of the fetal MRI includes neurological and not neurological indications. Of the neurological indications, the fetal MRI assists the dignosis of ventriculomegaly, corpus callosum agenesis, abnormalities of posterior fossa and malformations of the development of the brain cortex. Amongst the not neurological, the fetal MRI assists the diagnosis of complications of monochorionic twin gestations, congenital diaphragmatic hernia, lung sequestration, congenital cystic adenomatoid malformations and airways obstruction. The objective of the present article is to present the applicability and the indications of MRI in the diagnosis of malformations, assisting the gynecologist and obstetric in the agreement of this new diagnostic resource in the area of fetal medicine. The use of fetal MRI supplies information, authorizing changes in the counseling to the patient and its treatment.

Prenatal MRI Findings of Fetuses with Congenital High Airway Obstruction Sequence

OBJECTIVE: To define the MRI findings of congenital high airway obstruction sequence (CHAOS) in a series of fetuses. MATERIALS AND METHODS: Prenatal fetal MR images were reviewed in seven fetuses with CHAOS at 21 to 27 weeks of gestation. The MRI findings were reviewed. The MRI parameters evaluated included the appearance of the lungs and diaphragm, presence or absence of hydrops, amount of amniotic fluid, airway appearance, predicted level of airway obstruction, and any additional findings or suspected genetic syndromes. RESULTS: All the fetuses viewed (7 of 7) demonstrated the following MRI findings: dilated airway below the level of obstruction, increased lung signal, markedly increased lung volumes with flattened or inverted hemidiaphragms, massive ascites, centrally positioned and compressed heart, as well as placentomegaly. Other frequent findings were anasarca (6 of 7) and polyhydramnios (3 of 7). MRI identified the level of obstruction as laryngeal in five cases and tracheal in two cases. In four of the patients, surgery or autopsy confirmed the MRI predicted level of obstruction. Associated abnormalities were found in 4 of 7 (genetic syndromes in 2). Postnatal radiography (n = 3) showed markedly hyperinflated lungs with inverted or flattened hemidiaphragms, strandy perihilar opacities, pneumothoraces and tracheotomy. Two fetuses were terminated and one fetus demised in utero. Four fetuses were delivered via ex utero intrapartum treatment procedure. CONCLUSION: MRI shows a consistent pattern of abnormalities in fetuses with CHAOS, accurately identifies the level of airway obstruction, and helps differentiate from other lung abnormalities such as bilateral congenital pulmonary airway malformation by demonstrating an abnormally dilated airway distal to the obstruction.

[Lethal osteochondrodysplasia: foetopathological study of 32 cases]

The lethal osteochondrodysplasias are rare, their prevalence is estimated at 1 per 10 000 births. Mostly have genetic determinism. To describe the malformations and dysmorphic features in lethal osteochondrodysplasias.Our study involved 32 cases of lethal fetal Osteochondrodysplasias, collected over a period of 14 years in the pathological department of Sousse.Our series consisted of 23 foetuses from a medical termination of pregnancy, 6 newborns and 3 stillbirths. The mean age of mothers was 28 years old, consanguinity was observed in 61%. 3 cases of recurrence of the disease in three families were noted. The bone abnormalities were detected in antenatal ultrasonography in 25 cases [87%] and at birth in 7 cases. Ultrasound showed micromelia in all cases, a narrow chest in 5 cases and spina bifida in 3 cases. The foetopathological exam, including a macroscopic examination, radiological and histological samples of bone, has allowed us, based on the International Classification of 2001 to classify the 32 cases of Osteochondrodysplasias in: 8cases of Achondrogenesis type I [type Parenti-Fraccaro], 3 cases of Achondrogenesis type II [Langer Saldino], 9 cases of lethal osteogenesis imperfecta, 8 cases of thanatophoric dysplasia, 4 cases of Schneckenbecken dysplasia, 2 cases of Short rib polydactyly syndrome, Majewski type and 1 case of asphyxiating thoracic dysplasia

Thanatophoric dysplasia: a case report and review of literature.

Autopsy was performed on a fetus of 6 months gestation with sonologic features of skeletal dysplasia to arrive at a pathological diagnosis. Radiograph of fetus was taken which showed short limb bones, curved femora, flattened vertebral bodies with wide intervertebral spaces, bell-shaped chest, short ribs with flared ends which are features described in Thanatophoric dysplasia. This was confirmed by histopathological findings in the epiphyseal growth plate. In this paper, we attempt to describe the radiological and pathological findings of our case with a discussion on comparative literature.

Anomalias do trato urinário fetal: aspectos de interesse clínico / Congenital uropathies: aspects of clinical interest

As anomalias fetais ocorrem em 2 a 3 porcento das gestações, sendo as aquelas do trato urinário responsáveis por 15 a 30 porcento de todas as anomalias fetais e por grande parte das alterações que levam à insuficiência renal crônica e à doença renal em estágio terminal na infância. Convergindo com o desenvolvimento da ultrasonografia obstétrica, não só o momento do diagnóstico foi antecipado, como também a história natural dessas condições pôde ser melhor compreendida. Apresentamos uma atualização abrangente, tecendo considerações gerais sobre as principais anomalias urológicas, correlacionando-as com aspectos evolutivos de interesse clínico e com o prognóstico perinatal

Adriamycin-induced fetal hydronephrosis

INTRODUCTION: At the end of pregnancy, the amniotic fluid (AF) depends basically on renal function, corresponding to fetal urine. Changes in AF, especially oligohydramnios, are reported in association with fetal hydronephrosis (FH). The experimental model using adriamycin in pregnant female rats has a teratogenic effect and has been classically employed to study esophageal atresia. Nevertheless, adriamycin promotes FH with high frequency as well. In the present study, using this animal model, we tried to identify the incidence and microscopic changes of FH, as well as its correlation with AF weight. MATERIALS AND METHODS: Eight Spreague-Dawley pregnant female rats received adriamycin 2.2 mg/kg on the 8th and 9th gestational days (considering term gestation = 22 days). Those fetuses that received adriamycin (Adriamycin Group) were compared with fetuses from 2 female rats (Control Group), which received 0.9 percent saline solution. On the 21.5 gestational day, the fetuses were collected by cesarean incision, sacrificed, and examined for macro and microscopic changes in kidneys and ureters. Fetuses with bilateral hydronephrosis formed the Hydronephrosis Group. AF weight was determined as well. RESULTS: Hydronephrosis occurred in 70 (95 percent) of the 74 fetuses in the adriamycin group against none of the 21 fetuses from the control group. The amniotic fluid weight was increased in the adriamycin group in relation to the control group (p < 0.001). The histomorphometric study revealed dilation of the renal pelvis and reduction of renal parenchyma in the hydronephrosis group in relation to the control group. Severe cortical atrophy, cortical tubular atrophy and medullar atrophy were observed in the hydronephrosis group. CONCLUSIONS: Slight renal lesions were in agreement with changes in AF weight, since they suggest that there was production of urine with the maintenance of AF.

Rabdomioma gigante intracardíaco en la etapa neonatal. Reporte de un caso / Giantintracardiaac rhabdomymma

Los tumores primarios del corazón son poco comunes. Los más frecuentes durante la niñez son los rabdomiomas. El diagnóstico puede efectuarse desde la etapa fetal. La evolución es variada, en algunos casos no existe sintomatología y el diagnóstico representa un hallazgo ecocardiográfico, en otros se manifiesta por la presencia de soplo cardíaco, otros más se detectan asociados con esclerosis tuberosa y un pequeño grupo se manifiesta por arritmias o bajo gasto. Por lo raro de su presentación sobre todo en etapa fetal se presenta este caso.

Primary heart tumors are not very common, frequent ones during childhood are the rhabdomyomas. This diagnostic can be made since fetal life. Evolution variates, in some cases there are no symptoms and the diagnostic represents an echocardiographic finding, other cases are detected associated with tuberous sclerosis and a small group express by arrhythmias or low output. Because of the strange of this presentation this case is presented. (Arch Cardiol Mex 2004; 74:49-52).

Partial Trisomy 2q(2q37.3->qter)and Monosomy 7q(7q34->qter) Due to Paternal Reciprocal Translocation 2;7: A Case Report

We report an unbalanced translocation involving chromosome 2 and 7 due to a balanced reciprocal translocation 2;7 in the father. The female fetus had a partial trisomy of the long arm of chromosome 2 with a partial monosomy of distal 7q. Ultrasound at the first trimester had indicated normal fetal anatomy, including normal intracranial structures. Parental karyotypes showed a paternal balanced translocation: 46,XY,t(2;7)(q37.3;->q34). The unbalanced translocation in the fetus resulted in trisomy for 2q37.3 qter and monosomy for 7q34->qter. Postnatal examination showed that the female abortus had a cleft lip and palate, and mild dysmorphic features. The clinical phenotype was in agreement with previous descriptions and allowed us to propose a fetal phenotype for this chromosomal abnormality.

Prenatal Sonographic and MR Imaging Findings of Extensive Fetal Lymphangioma: A Case Report

We report the imaging findings in a case of fetal lymphangioma involving the retroperitoneum and right lower extremity, and diagnosed by ultrasonography and magnetic resonance (MR) imaging at 26 weeks of gestation. Prenatal ultrasonograms and T2-weighted single-shot fast spin-echo MR images clearly revealed an extensive, multilocular cystic mass with internal hemorrhage in the retroperitoneum extending to the lower extremity.

Klippel-Trenaunay syndrome.

Klippel Trenaunay syndrome is a rare congenital disorder characterised by vascular anomalies and life threatening complications. Clinical recognition, prenatal diagnosis and counselling is important in these cases. Here we present a case of Klippel-Trenaunay syndrome and emphasize upon the clinical significance of such cases.

Achondrogenesis type II (Langer-Saldino achondrogenesis): a case report

Achondrogenesis is a lethal form of congenital chondrodystrophy characterized by extreme micromelia. We describe a case of achondrogenesis type II (Langer-Saldino achondrogenesis) detected by prenatal ultrasonography at 20-week gestation. A dwarfed fetus with large head, short neck and chest, prominent abdomen and short limbs was terminated transvaginally. Radiologic and histopathologic examination revealed features of mild form of achondrogenesis type II. Although the case had no known risk factor and the phenotypic abnormality was mild, modern development in prenatal screening made the early detection possible.

Macerated fetus in utero relating to intracranial angioma: report of 2 cases.

Two macerated fetuses in utero are presented. The mothers experienced no abnormalities during pregnancy. Both fetuses were found postmortem to have intracranial angiomas. In case 1, the angioma in the choroid plexus of the right lateral ventricle was associated with intraventricular and subarchnoid hemorrhages. There was additional angiomatosis in the leptomeninges and substance of the brain. In case 2, there was leptomeningeal angiomatosis with diffuse subarchnoid hemorrhage. It is suggested that spontaneous rupture of the angioma of the choroid plexus in case 1 and of the leptomeninges in case 2 resulted in immediate death of the fetuses with subsequent maceration in utero. Asymptomatic angioma of the fourth ventricular choroid plexus is also described in case 2 because of its extreme rarity.