Search

Sigmoid volvulus requires urgent treatment, and it is particularly rare among pregnant women without a history of laparotomy. A delay in diagnosis may lead to serious consequences for the mother and fetus, and a rapid diagnosis and treatment in this setting is essential. The patient was a 19-year-old primiparous woman. She complained of repeated exacerbations and remissions of abrupt lower abdominal pain for the past 2 days and was transported to our hospital at 33 weeks of gestation. Ultrasonography revealed no placental thickening, and maternal bowel dilation was difficult to identify. Plain abdominal X-ray showed a dilated colon on the left side of the abdomen. Contrast-enhanced CT scan of the abdomen revealed a volvulus on the dorsal side of the uterus. The proximal end of the transverse to sigmoid colon was markedly dilated, and the distal end was collapsed. The elevated lactate level on blood gas analysis suggested intestinal ischemia. She was suspected of having a sigmoid volvulus at 33 weeks and 3 days of gestation. We decided to perform a cesarean section to secure the operative field for an intestinal resection following delivery. A male weighing 1840 g with Apgar scores 8/8 was delivered. The sigmoid colon was approximately 80 cm in length. A 360-degree clockwise rotation of was observed with a very distended but viable sigmoid loop. Following reduction of the volvulus, the sigmoid colon was fixed to the left side of the peritoneum. The mother had an uneventful postoperative course, and the infant was discharged without any sequelae. This case demonstrates two important lessons. First, sigmoid volvulus can occur in pregnant women even if they never had a laparotomy. Second, abdominal contrast-enhanced CT is useful for rapid diagnostic and treatment decisions relative to this pathology.

Pseudoaneurysms generally develop when an arterial puncture site is inadequately sealed. We encountered a case of vaginal pseudoaneurysm that developed 3 years after cesarean section in a 35-year-old gravida 7 para 4 woman who was prescribed with anticoagulant and antiplatelet drugs after surgeries for ventricular septal defect and aortic valve replacement. Pelvic computed tomography scan revealed a large mass, which showed a dappled contrast filling on the arterial phase, located in the posterior vaginal wall. The vaginal pseudoaneurysm was completely occluded by embolization of the left vaginal artery. Anticoagulation and antiplatelet therapies can be potential causes of spontaneous pseudoaneurysm rupture. Extrauterine pseudoaneurysm has a long period of time between cesarean section and pseudoaneurysm discovery. Considering that pseudoaneurysm shows different clinical features for each patient, we should always consider pseudoaneurysm when we assess a patient with postpartum hemorrhage.

Massive subchorionic thrombohematoma (MST), termed Breus’ mole, is a rare condition in which a large maternal blood clot separates the chorionic plate from the villous chorion. Common complications of MST include fetal growth restriction, preeclampsia, and intrauterine fetal death. Here, we present a case of a 17-year-old Japanese woman referred to our institution at 21 weeks of gestation. Ultrasound examination revealed a large placental mass with mixed high and low echogenicity measuring approximately 7.6 cm in thickness. Doppler examination showed absence of end-diastolic velocity of the umbilical artery. At 22 weeks of gestation, the patient had a stillbirth weighing 138g. The placenta weighed 502 g and was 8 cm thick, and the total blood loss was 270 g. Macroscopic examination revealed that a subchorionic blood clot measuring 12 cm × 5 cm covered a large portion of the placenta with well-defined margins on the fetal surface. Microscopic examination revealed an intervillous hematoma and fibrinous deposits directly beneath the chorionic plate with adjacent compressive effects. Based on these findings, MST was diagnosed. Because MST is rare, it must be considered in the differential diagnosis of parental conditions. Magnetic resonance imaging can be optimal for diagnosing MST when ultrasound diagnosis is difficult.

We report a case of Cornelia de Lange syndrome (CdLS) where prenatal diagnosis was not made even with major anomaly. A 33-year-old Japanese woman was referred to our institution at 23 weeks of gestation because of fetal forearm defect. Ultrasound examination revealed short forearms and short humeri and femurs (–2.1 SD). The fetal estimated body weight was 450 g (–1.3 SD). Fetal MRI at 26 weeks of gestation revealed short forearms and hypoplasty of hand fingers. Fetal growth restriction became evident thereafter, leading to intrauterine fetal death occurring at 29 weeks of gestation. A stillbirth baby was of 798 g in body weight and 33.0 cm in length. External examination showed a low hairline, synophrys, low-set ear, hypertrichosis, and smooth long philtrum with thin lips. The neck appeared short and broad. Finally, CdLS was diagnosed. The prenatal diagnosis might be possible as the arm findings were totally characteristic in a small fetus, regardless of whether an overhanging upper lip was identified. Because CdLS is a rare condition, it is important to consider its possibility as a part of differential diagnosis.

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.

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.

学位論文

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.