Prenatal diagnosis of a retroperitoneal lymphangioma: a case and review

Prenatal sonographic findings of lymphangiomas characteristically appear as thin-walled, multiseptate hypoechogenic masses. In our case, a retroperitoneal hypoechogenic mass was detected at 26 weeks of gestation by sonography. Serial sonographic examinations demonstrated multiple septations at 29 weeks of gestation, which in retrospect was a classical finding of retroperitoneal lymphangioma. MRI at 38 weeks of gestation revealed a multilocular mass, which was homogeneous, low on T1-weighted and high on T2-weighted images, in the left retroperitoneal cavity. These findings were compatible with the diagnosis of a lymphangioma. This case shows the change in characteristic imaging features, from a unilocular to multilocular pattern, of a lymphangioma with regard to gestational age. It is important to observe the size and extension of such tumors in order to determine the prospect for neonatal prognosis, as well as to make decisions on the delivery timing and style.     

Prenatal ultrasonographic diagnosis of abdominal cystic lymphangioma: A case report

Abdominal lymphangioma is a rare tumor of the lymphatic vessels. A case of an abdominal cystic lymphangioma identified at 22 weeks of gestation is reported. Ultrasonographic monitoring showed a progressive increase of mass size during the gestation. Pregnancy was terminated at 38 weeks and the newborn was submitted to a laparotomy with resection of all cystic structures. At the present time the infant is three years old and is doing well.     

Right ventricular hypertrophic cardiomyopathy in the fetus --may it be caused by chronic oral tocolysis with fenoterol?

The use of fenoterol in the treatment of preterm labor is associated with the risk of many complications in the mother and the fetus. We present a case of a multipara treated with oral fenoterol due to threatening preterm labor 14 weeks. At 35 weeks of gestation the fetus was diagnosed with hypertrophic cardiomyopathy with severe impairment of the right ventricle. The only factor that might have caused such a state of the fetal circulatory system was fenoterol, used from 21 weeks of gestation. After the withdrawal of the fenoterol the fetal right ventricular function improved gradually. However fetal cardiac hypertrophy persisted until the birth at 39 weeks of gestation. Concentric hypertrophy of the right ventricular wall and interventricular septum were confirmed in the newborn.     

Amniotic band syndrome following septostomy in management of twin-twin transfusion syndrome: a case report.

Septostomy, a rupture of the diamniotic membrane separating monozygotic twins essentially creating a monoamniotic gestation, is a potential therapeutic modality for twin-twin transfusion syndrome (TTTS). This may be associated with complications including cord entanglement or complete rupture of the membranes. We report a case of severe amniotic band syndrome with cord amputation after septostomy. A 33-year-old woman with a Mo-Di twin pregnancy was diagnosed with TTTS at 18 weeks of gestation. Septostomy as well as amnioreduction were performed at 24 weeks of gestation. A repeat cesarean delivery was performed at 31 weeks resulting in a live recipient baby of 1340 g and a dead donor with amniotic band syndrome. The donor showed pieces of membrane tightening both legs. The right thigh became entangled in the bands connecting to the umbilical cord of the live fetus. The umbilical cord of the dead twin was completely amputated, whereas the umbilical cord of the live infant was also entrapped within the amniotic band resulting in small diameter and some degree of stricture. This is the first report of a rare but serious complication following septostomy.     

Prenatal diagnosis of hemifacial microsomia by magnetic resonance imaging

We present a case of hemifacial microsomia, first detected by prenatal sonography and confirmed by ultrafast magnetic resonance (MR) imaging. A 26-year-old patient was referred to our hospital at 20 weeks of gestation because of unilateral right-sided ventriculomegaly and of a possible ventriculoseptal defect (VSD). Our sonographic examination suggested a right orbital hypoplasia and a hemiatrophy of the nose. The ultrafast T2-weighted single-shot fast-spin echo MR imaging, demonstrated a right ear hypoplasia (microtia), a right orbital hypoplasia, and a right renal hypoplasia. Epibulbar dermoid or conjunctival lipodermoid were not recognized. The fetus was prenatally diagnosed as hemifacial microsomia. Termination of pregnancy was performed at 21 weeks gestation, and a male baby weighing 342g was stillborn. The baby showed facial asymmetry, including hemiatrophy of the right nose, and right ear hypoplasia. Autopsy revealed a 2mm area of VSD, agenesis of the right kidney and ureter, pancreatic and renal aberration into right adrenal gland, thymus hypoplasia and an unfixed ascending colon without intestinal malrotation. To the best of our knowledge, this is the first report of prenatal diagnosis for hemifacial microsomia using fetal MR imaging. In our case, fetal MR imaging has evolved into a powerful diagnostic tool, for the accurate prenatal diagnosis.     

Prenatal diagnosis of a fetal chest wall cystic lymphangioma using ultrasonography and MRI: a case report with literature review

A case of fetal chest wall cystic lymphangioma diagnosed prenatally at 28 weeks' gestation is reported. Ultrasound examination showed a multilocular, large cystic mass (10 x 22 x 29 mm) on the left side of the fetus in the area of the lower chest and upper abdomen, without color flow imaging. Magnetic resonance imaging was used to evaluate the extent and the tissue characteristics of the lesion. Two months after birth the lymphangioma was surgically removed, following an infection and a rapid increase in size. The case is discussed, and a short review of the literature is reported.     

Prenatal sonographic findings of massive lower extremity lymphangioma.

A massive lymphangioma was detected sonographically in the lower extremity of a fetus at 28 weeks' gestation. On ultrasound study, the mass had multiple cysts and extended from the left lower abdomen to the left knee. The differential diagnosis and obstetric management following prenatal diagnosis of a fetal lower extremity mass are presented.     

Arrhythmogenic right ventricular cardiomyopathy with implantable cardioverter defibrillator placement in pregnancy.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disorder that predominantly affects the right side of the heart and causes ventricular arrhythmias. In many patients the disease is familial. ARVC may account for as many as 5% of unexpected sudden deaths. We report a case of ARVC diagnosed at 21 weeks gestation treated with an implantable cardiac defibrillator. The remainder of her pregnancy was uneventful and the patient underwent induction of labor at 39 weeks gestation with a passive second stage forceps-assisted delivery resulting in delivery of a normal infant. In the gravida with cardiac arrhythmias, defibrillator placement may offer a safe, life-preserving treatment and should be considered.     

Ovarian carcinoma, endometrial carcinoma, and pregnancy

A 31-year-old G1 P0 patient with a history of infertility presented with light spotting and cramping at the end of her first trimester. An ultrasonogram at 19 weeks gestation revealed an intrauterine gestation of 21 weeks, a large leiomyoma, and a 8.9 x 6.8 cm complex left ovarian mass. At 35 weeks gestation she had an emergency cesarean section and left salpingo-oophorectomy due to a presumed ruptured ovarian mass. The ovarian mass was diagnosed as a serous cystadenocarcinoma. An exploratory laparotomy with a total abdominal hysterectomy, a right salpingo-oophorectomy, omental biopsy, and periaortic node sampling at 9 weeks postpartum revealed a diagnosis of stage IC ovarian serous cystadenocarcinoma and a stage IA secretory endometrial adenocarcinoma. Adjunctive 32P therapy was successfully administered and at this time the patient has had no recurrence.     

Prenatal diagnosis of de novo (7;19)(q11.2;q13.3) translocation associated with a thick corpus callosum and Wilms tumor of the kidneys

We present a case of prenatal diagnosis of a de novo (7;19)(q11.2;q13.3) translocation associated with ultrasound features, including enlarged cisterna magna, normal vermis, thick corpus callosum, micrognathia, small and low-set ears and right hyperechogenic kidney. Karyotyping was performed at 24 weeks of gestation. Termination of pregnancy was accepted at the parents' request. Postmortem examination confirmed the prenatal findings, but revealed bilateral Wilms tumors of the kidneys. Parental karyotype was normal.     

In utero diagnosis of an aneurysm of the vein of Galen causing hydrocephalus and heart failure

A prenatally diagnosed aneurysm of the vein of Galen was presented in the fetus of a patient referred to our hospital at 31 weeks of gestation. Ultrasonography demonstrated polyhydramnios, cardiomegaly, dilatation of the right atrium and superior vena cava, tricuspid valve regurgitation, hydrocephalus, and a large hypoechoic mass with blood flow in the suboccipital region. Skin edema was shown thereafter. A 3262-g male was delivered by cesarean at 35 weeks of gestation. Computed tomography imaging demonstrated a large mass in the suboccipital region, after which thrombocytopenia appeared and the neonate died at 18 days of age.     

May-Thurner syndrome resulting in acute iliofemoral deep vein thrombosis during the second trimester of pregnancy

Described is a 27-year-old pregnant woman with May-Thurner syndrome who experienced extensive pelvic and lower extremity thromboses during the antepartum period. The patient was referred for a symptomatic deep venous thrombosis at 23 weeks of gestation. Ultrasonography demonstrated a massive thrombus in the left iliofemoral vein. Heparin was given intravenously. Due to the possibility of pulmonary embolism during or immediately after delivery, a temporary inferior vena cava filter was inserted at 36 weeks of gestation. Labor was induced at 37 + 5 weeks of gestation; labor proceeded uneventfully and a male infant was born. Postpartum computed tomography (CT) demonstrated compression of the left common iliac vein by the right common iliac artery and lumbar vertebra. CT venogram demonstrated poor flow through the common iliac vein and well-developed collateral vessels. Critical stenosis at the origin of the left common iliac vein was consistent with a diagnosis of May-Thurner syndrome.     

Isolation of cytomegalovirus from the amniotic fluid during the third trimester

A case is presented in which cytomegalovirus was isolated from the amniotic fluid at 36 weeks' gestation in a pregnancy complicated by cytomegalovirus hepatitis at 10 weeks of gestation. Abnormalities noted in the newborn infant included an undescended testis, right equinovarus, and hypotonia. All cultures revealed cytomegalovirus. Subsequent immunoglobulin studies, chest x-ray film, and bone films were all normal.     

Right neck venous thrombosis following ovarian hyperstimulation syndrome in a patient with protein S deficiency: A case report and review of literature

ObjectiveThe risk of venous thromboembolism in pregnancies increased in ovarian hyperstimulation syndrome (OHSS) after assisted reproductive technologies (ART). We present a rare case with protein S deficiency receiving ART treatment with OHSS, following right neck venous thromboembolism.Case reportA 34-year-old women with primary infertility underwent IVF treatment and presented with OHSS. However, thromboembolism in the right jugular and subclavian veins was diagnosed at eight weeks of gestation. She was continuously treated with low molecular weight heparin (LMWH) since eight weeks of gestation and the diagnosis of protein S deficiency was made. Due to placenta previa with massive bleeding, she gave live birth to two healthy babies via cesarean section at 34 weeks of gestation.ConclusionThromboembolism is one of life-threatening complications among women with OHSS. Although inherited thrombophilia is rare diseases, thrombophilia workup may be taken into consideration for women with thrombotic events.     

Prenatal diagnosis and outcome of lymphangiomas and its relationship with fetal chromosomal abnormalities

Objectives: Our aim was to evaluate ultrasound findings and perinatal outcome after prenatal diagnosis of lymphangioma.

Methods: This was a retrospective case series study. We searched the archives of our ultrasound database at our center for cases with the prenatal diagnosis of the lymphangioma in the period between January 2008 and November 2014. We described maternal, fetal and perinatal variables for all cases.

Results: Nine fetuses with lymphangioma were identified. All cases were diagnosed during the second and third trimesters with the average gestational age of 22.6?±?3.9 weeks. The average diameter of lymphangioma was 55.4?±?20.1?mm at the time of diagnosis. Five fetuses (55.6%) had lymphangioma on the neck, and four fetuses (44.4%) had lymphangioma on other localizations. Normal fetal karyotype was detected in all cases. There were a total of six live births, one intrauterine death and two medical terminations of pregnancy following the diagnosis of lymphangioma. No abnormal Doppler finding or hydrops were detected in the antenatal follow-up of remaining six cases.

Conclusion: The risk of chromosomal abnormalities is very low in pregnancies with isolated lymphangioma. The outcome of pregnancies with lymphangioma is generally favorable and prognosis depends on their locations and size.     

Sonographic diagnosis of cystic adenomatoid malformation in-utero

Routine ultrasound examination at 30 weeks gestation revealed an intrapulmonary cystic mass in an otherwise normal fetus. Following delivery at term, the diagnosis of congenital cystic adenomatoid malformation of the right lung was confirmed, and an elective right middle lobectomy successfully performed at nine days of age.     

Prenatal management of inherited urogenital malformation: case report

Documentation of unique kidney renal function early in pregnancy can be helpful in defining prenatal management and therefore in improving prognosis. Antenatal diagnosis of a solitary kidney was performed at 20 weeks' gestation in a foetus with a 1,7 chromosome translocation. Because of the decreasing renal function and the increasing pelvic dilatation, an early in utero stenting was placed at 23 weeks' gestation. Optimal outcome occurred and the baby was delivered at 32 weeks. Complete assessment of the malformation showed a left hydronephrosis due to a megaureter, right renal agenesis with ipsilateral cryprorchidism and agenesis of the right vas deferens. The chromosomic translocation was inherited from the mother who was affected by uterus didelphys, obstructed right hemivagina and right renal agenesis. Renal function of the unique kidney with hydronephrosis can be early diagnosed and promptly treated. This condition should also increase the index of suspicion of underlying genital and chromosomal anomalies.     

In utero progressive pulmonary stenosis successfully treated with transcatheter intervention after delivery.

It is unclear whether pulmonary stenosis with intact ventricular septum is a secondary cardiac malformation. We report an infant with pulmonary stenosis (diagnosed by fetal echocardiography) with progressive obstruction in late gestation who presented with increasing transvalvular pressure gradients (15 mm Hg at 22 weeks' gestation to 47 mm Hg at 35 weeks). The tricuspid/mitral valve annulus ratio decreased from 1.25 at 24 weeks' gestation to 0.96 at 33 weeks. At 38 weeks' gestation, a male infant weighing 3,524 g, with Apgar scores of 9 and 9 at 1 and 5 minutes, respectively, was delivered by cesarean section. Critical pulmonary stenosis was confirmed by postnatal catheterization. These findings support the postulation that pulmonary stenosis is a progressive disorder. After percutaneous balloon dilatation, the transvalvular pressure gradient decreased and the right ventricular cavity increased gradually. The transvalvular pressure gradient had decreased to 15 mm Hg and the tricuspid/mitral valve annulus ratio was 0.93 at the age of 2 years.     

Reduction of pleural effusion by OK-432 in a fetus complicated with congenital hydrothorax

A 29-year-old, primiparous woman was referred to our hospital at 21 weeks of gestation because of right pleural effusion in the fetus shown by routine ultrasonographic examination. Cytology revealed abundant lymphocytes, suggesting chylothorax. We removed the pleural effusion and injected OK-432 into the chest cavity at 24 and 25 weeks of gestation. Pleural effusion declined and an adhesion between the lung surface and the pleural membrane seemed to form. At 33 weeks of gestation, a female infant was born by cesarean section (1,090 g and Apgar score 6/8). Although she demonstrated slight retraction and tachypnea, management could be achieved by administration of oxygen alone without mechanical ventilation. Later, the baby was diagnosed as suffering from the Cornelia de Lange syndrome with characteristic features. OK-432 injections could thus prevent complications of chylothorax and hypoplastic lungs, without injury to either the baby or the mother.     

Pregnancy in a woman with unilateral lung agenesis.

Unilateral lung agenesis is a rare congenital condition of unknown etiology. A 33-year-old nullipara with right lung agenesis and scoliosis was admitted to the hospital at 30 weeks of gestation because of oligohydramnios. At 32 weeks she was treated for an upper respiratory tract infection with azithromycin. She went into premature labor at 34 weeks and was delivered by Cesarean for breech presentation. Both mother and infant did well.