Tadatsugu Kinjo

Diffuse Venous Malformation of the Uterus in a Pregnant Woman with Klippel-Trénaunay Syndrome Diagnosed by DCE-MRI.

Background. We experienced a rare case of a pregnant woman with Klippel-Trénaunay syndrome complicated with diffuse venous malformation of the uterus. This is the first report on the usefulness of dynamic contrast-enhanced-MRI for the diagnosis of diffuse venous malformation of the uterus. Case Presentation. A 23-year-old woman presented with convulsions and talipes equinus position of both lower limbs at 11 weeks of gestation. At 27 weeks, ultrasonography demonstrated tubular echolucent spaces throughout the myometrium. Dynamic MRI at 37 weeks revealed that the myometrial lesion was enhanced slowly and showed homogeneous enhancement even on a 10 min delayed image. Taken together with unilateral foot hypertrophy, varices, and port-wine stain, the patient was diagnosed as having Klippel-Trénaunay syndrome complicated with diffuse venous malformation of the pregnant uterus. The patient underwent elective cesarean section because of severe dystonia. The lower uterine segment was thickened and heavy venous blood flow was observed at the incision. Histological diagnosis of the myometrial biopsy specimen was venous malformation. Conclusions. Both diffuse venous malformation and Klippel-Trénaunay syndrome during pregnancy can involve considerable complications, in particular, massive bleeding during labor. Women who suffer from this syndrome should be advised about the risk of complications of pregnancy.

Acute congestive heart failure due to ruptured mitral chordae tendineae in late pregnancy

A 31‐year‐old woman complained of dyspnea and orthopnea at 38 weeks of gestation. A grade 3/6 pansystolic murmur was heard, and echocardiography revealed severe mitral regurgitation with a hyperechoic obstacle on the posterior mitral valve leaflet, consistent with a diagnosis of acute heart failure due to a ruptured chordae tendineae or an infectious endocarditis. An emergency cesarean section was performed under general anesthesia. A male infant was born weighing 2928 g with Apgar scores of 7 and 8 at 1 and 5 min, respectively. The patient was managed in the intensive care unit and underwent open‐heart surgery for mitral valve repair on postpartum day 3. The two chordal tendineae appeared torn and frail, and a mitral annuloplasty was performed. No finding of infectious endocarditis was observed. Because it is a dramatic and life‐threatening clinical situation, proper diagnosis and treatment in the intensive care unit assure a good outcome for both mother and fetus.

Multimodality imaging in secondary postpartum or postabortion hemorrhage: retained products of conception and related conditions

Secondary postpartum hemorrhage (PPH) and postabortion hemorrhage are rare complications. Retained products of conception (RPOC) is among the most common causes of both secondary PPH and postabortion hemorrhage. Other less common causes of secondary PPH are uterine vascular abnormalities such as arteriovenous malformations and pseudoaneurysms. These are usually related to a history of a procedure such as dilation and curettage or cesarean delivery. Subinvolution of the placental site is an idiopathic cause of secondary PPH; this condition may be underrecognized and therefore could have a higher incidence than currently reported. Gestational trophoblastic disease is rare but commonly presents as secondary PPH and resembles RPOC in radiologic appearance. The first-line imaging modality for secondary PPH is ultrasound, but computed tomography and magnetic resonance imaging may be used if the ultrasound findings are indeterminate. Angiography is an important tool for the definitive diagnosis of uterine vascular abnormalities. Appropriate management requires radiologists to be familiar with the multimodality imaging features of secondary PPH or postabortion hemorrhage.

Fetal Sirenomelia Associated with an Abdominal Cyst Originating from a Saccular Cloaca

A 40-year-old pregnant woman presented with a fetal abdominal cyst and oligohydramnios. Color Doppler scan revealed a single blood vessel from the fetal aorta into a single umbilical artery. Severe oligohydramnios limited ultrasonographic evaluation of the fetal lower limbs, kidneys, or bladder. The pregnancy was terminated; the fetus showed fused lower limbs, bulging abdomen, and absent external genitalia and was diagnosed with type III sirenomelia. On autopsy, no normal bladder was observed, but duodenal atresia, anorectal atresia, and right renal agenesis were found. An intra-abdominal cyst, diagnosed histologically as a saccular cloaca, occupied the abdominal cavity. Ultrasonographic diagnosis of fetal sirenomelia is difficult due to poor depiction of the lower limbs. A vitelline artery leading to a single umbilical artery and a fetal abdominal cyst occupying most of the abdominal cavity are considered fetal sirenomelia associated with large defects of the gastrointestinal and genitourinary tracts.

A Case of Intrathoracic Gastric Duplication Cyst Detected on Prenatal Ultrasound Examination

A 37-year-old (G4P3) woman was referred to our hospital at 32 weeks of gestation for the evaluation of a fetus with an intrathoracic cystic lesion. Ultrasonography and magnetic resonance imaging revealed that a fetal cystic lesion without a mucosal layer was located in the posterior mediastinum. These findings were consistent with a bronchogenic cyst. At 38 3/7 weeks of gestation, an elective cesarean section was performed because of her previous cesarean section. A female neonate without any external anomalies, weighing 2,442 g, with Apgar scores of 8 and 9, and requiring no resuscitation was born. Four weeks after delivery, the neonate was admitted because of respiratory distress due to mass effect. At right lateral thoracotomy, a 105 × 65 mm of solitary smooth-walled cyst containing serosanguineous fluid was found in the posterior mediastinum, which was excised completely. Histologic examination revealed the diagnosis of the mediastinal gastric duplication cyst. The neonate made an uneventful recovery. Accurate diagnosis is not necessary, but detection and continuous observation are logical. Although gastric duplication, particularly intrathoracic, is a rare pathology, it should be considered in the differential diagnosis of any intrathoracic cyst.

Fetal Congenital Peripheral Bronchial Atresia Diagnosed by Magnetic Resonance Imaging: Two Case Reports

Two types of congenital bronchial atresia (proximal and peripheral) have been classified. We report two cases of peripheral bronchial atresia diagnosed by prenatal ultrasonography (US) and magnetic resonance imaging (MRI). Evaluating an enlarged lung mass that is homogeneously hyperechoic on US and hyperintense on T2-weighted MRI can help in determining whether bronchial atresia is present. Proximal type is suggested when a dilated main bronchus is observed as a tubule structure of an involved lung hilum. In our cases, T2-weighted MRI revealed homogeneously hyperintense lung lesion with decreased signal intensity of adjacent lobe, flattening diaphragm, and mediastinal shift. Dilatation of the main bronchus was not observed and the opposite lung was normal in appearance. These findings were explained by secondary compression due to enlargement of the involved lung. The preservation of vascular structure and the retained normal shape, though enlarged, in the affected lobe were observed, which demonstrated undisrupted pulmonary architecture of the lobe. Thus, congenital cystic adenomatoid malformation was excluded because pulmonary architecture was relatively preserved. Finally, presumed diagnoses of the peripheral bronchial atresia were made and confirmed by postnatal chest computed tomography.

Fetal Thoracoamniotic Shunting in a Case of Congenital Pulmonary Airway Malformations with Hydrops Fetalis.

Aim  We report a case of congenital pulmonary airway malformation (CPAM) with hydrops in which the fetus underwent thoracoamniotic shunting. Case Report  A 40-year-old (G1P1) woman was diagnosed with a macrocystic CPAM. Thoracoamniotic shunting was performed at 19 weeks of gestation but not well drained and was successfully performed again at 23 weeks. However, the CPAM volume ratio, abdominal circumference, and amniotic fluid index started increasing from 28 weeks and hydrops worsened. The insufficient shunting and the fetal cardiac failure had to be considered. At 32 weeks, a male infant with general edema and massive ascites was born weighing 3,362 g (+4.79 SD) with Apgar scores of 2 and 4. The infant was intubated and high-frequency oscillation and nitric oxide therapies were instituted. The resection of CPAM was performed on day 2. Nasal continuous positive airway pressure was instituted on day 16. The infant was discharged and prescribed with home oxygen therapy (HOT) on day 65. The infant was able to leave the HOT at 30 months and is currently 34 months of age in good condition. Conclusion  Fetal thoracoamniotic shunting may be life-saving in CPAM complicated by hydrops and that this treatment might be sufficient to cure the child.

A Case of Vaginal Stillbirth in the Presence of Placenta Previa at 33 Weeks of Gestation

It was demonstrated that second- and third-trimester therapeutic termination of pregnancy (TOP) is feasible in cases with placenta previa. We report a 34-year-old woman with complex fetal malformations associated with placenta previa. An ultrasound examination at 21 weeks of gestation revealed fetal growth restriction (FGR) and complex fetal malformations associated with a placenta previa. After extensive information, the parents opted for careful observation. Thereafter, FGR gradually progressed and we observed arrest of end-diastolic velocity of the umbilical artery. Finally, intrauterine fetal death (IUFD) was confirmed at 33 weeks of gestation. Two days after IUFD, the patient experienced labor pain. The placenta and dead fetus weighing 961 g were vaginally delivered, and total bleeding was 270 mL. Although further studies to confirm the dynamic change of the uteroplacental blood flow are necessary to avoid the risk of maternal hemorrhage, vaginal TOP with placenta previa after feticide or IUFD would be feasible.