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Foetal membranes bulging into the abdominal cavity is a unique initial manifestation of silent or complete uterine rupture during pregnancy. Since silent uterine rupture has potential risk for complete uterine rupture, which leads to acute life-threatening complications for both the mother and baby, it is difficult to determine whether to manage expectantly or surgically, including repair of the uterine wall or termination of the pregnancy, especially in the early second trimester. We present here a case of a herniated amniotic sac with overstretched uterine wall of the fundus presenting as silent uterine rupture, which was incidentally detected on routine ultrasonography at 18 weeks' gestation in a 38-year-old primigravida with a history of myomectomy for diffuse uterine leiomyomatosis. Magnetic resonance imaging examination revealed that the myometrium thickness was fully maintained at the site of the foetal membranes ballooning. The pregnancy was therefore managed expectantly and continued to successful delivery at 30 weeks' gestation. The precise assessment of the uterine wall may be essential to manage a herniated amniotic sac presenting as silent uterine rupture and to optimise the outcome of the pregnancy. We review all cases of a herniated amniotic sac out of focally overstretched uterine wall before 34 weeks' gestation. [ABSTRACT FROM AUTHOR]

D, Sutures targeting a single bleeding point using blunt straight needles [Color figure can be viewed at wileyonlinelibrary.com] gl Finally, we would like to address our opinion on the role of UCS when dealing with atonic PPH after vaginal and cesarean delivery, respectively. The hemostatic suture targeting a single arterial bleeding point may be a more effective method for intractable atonic PPH requiring a hysterectomy. [Extracted from the article]

We thank Dr Matsubara and his colleagues for their comments.[1] The suggested experiments are interesting and would provide additional information regarding the usefulness of a silicone three-dimensional-printed postpartum uterine cavity model. Novel subtype of atonic postpartum hemorrhage: dynamic computed tomography evaluation of bleeding characteristics and the uterine cavity. [Extracted from the article]

Background: Conservative management for placenta previa accreta (PPA) may be preferred, but uncontrollable hemorrhage sometimes occurs during a cesarean section. Case: We present a case with a successful maneuver for the management of intraoperative hemorrhage in PPA. Profuse hemorrhage occurred due to partial placental separation during a cesarean section. The bleeding was stopped by the placement of intrauterine tamponade balloons with their shafts passed through the anterior abdominal wall. The catheters were removed after 24 h. The patient had no complications, and the placenta spontaneously resorbed three months after delivery. Conclusion: This is a useful addition to the options for management of PPA at cesarean section. [ABSTRACT FROM AUTHOR]

Adult-onset metachromatic leukodystrophy (MLD) often shows schizophrenia- or encephalopathy-like symptoms at an early stage, such as behavioural abnormalities, cognitive impairment, mood disorders and hallucinations. The authors report the case of an adult woman with MLD who had been given antipsychotic medication for schizophrenia. In the differential diagnosis, screening of auto-antibodies was important for ruling out other encephalopathies as she had a euthyroid Hashimoto thyroiditis. Diagnosis was based the results of MRI, nerve conduction velocity, sensory evoked potential, motor evoked potential, lysosomal enzyme activity and gene analysis studies. Brain MRI showed diffuse demyelination spreading from the deep white matter to subcortical area as high signals at the edges of these lesions in diffusion and apparent diffusion coefficient-map images with the U-fibres conserved. The authors diagnosed adult-onset MLD coexisting with euthyroid autoimmune Hashimoto thyroiditis. [ABSTRACT FROM AUTHOR]

The patient described herein was diagnosed with left breast, endometrial, and early gastric cancers at 49, 53, and 57 years of age, respectively. Magnetic resonance cholangiopancreatography performed when she was undergoing treatment for cholecystitis at 50 years of age showed local pancreatic duct dilatation in the pancreatic head. She was followed in the Department of Gastroenterology at our hospital for an intraductal papillary mucinous neoplasm(IPMN). An abdominal computed tomography scan obtained at 59 years of age revealed dilation of the main pancreatic duct in the pancreas body and tail, therefore an endoscopic ultrasound-guided fine needle aspiration was performed. She was diagnosed with pancreatic cancer and underwent a laparoscopic distal pancreatectomy. The postoperative course was uneventful; however, the pancreatic cancer recurred and she died approximately 14 months postoperatively. Reports of multiple cancers associated with IPMNs are rare, yet we managed a patient with a pancreatic head IPMN complicated by metachronous quadruple carcinomas( breast, endometrial, gastric, and pancreatic cancers).

Background: Premature rupture of membranes occurring in previable midtrimester patients is associated with perinatal mortality, and optimal therapeutic methods are yet to be established., Objective: This study's objective was to investigate whether an elastomeric sealant, which has been used as a hemostatic agent for arterial anastomosis, could plug the uterine endocervical canal to prevent leakage of intrauterine fluid in an ex vivo model., Methods: The elastomeric sealant or fibrin glue was applied to the cervix of uteri removed for benign gynecological disease ( n  = 4). Normal saline was administered into the ex vivo uterine cavity through a catheter using a pressure infusion bag. Intrauterine pressure was measured using a digital pressure gauge, and the pressure at which normal saline started leaking out of the uterine cervix was compared between both the sealants., Results: No fluid leakage was observed with the elastomeric sealant until the pressure exceeded 20 kPa (150 mmHg), while the leakage onset pressure with fibrin sealant was 6.6 ± 1.8 kPa (50 ± 14 mmHg). The threshold leak pressure where the onset of liquid flow was initiated was significantly different between both the sealants ( p  < .0001)., Conclusions: Intracervical elastomeric sealant exhibited powerful fluid leakage prevention in an ex vivo model. The sealant would have potential to prevent the leakage of amniotic fluid in pregnancies with previable premature rupture of membranes.

Squeezing bubbles and impurities out of interlayer spaces by applying force through a few-layer graphene capping layer leads to van der Waals heterostructures with the ultraflat structure free from random electrostatic potential arising from charged impurities. Without the graphene capping layer, a squeezing process with an AFM tip induces applied-force-dependent charges of Δ n ∼ 2 × 10 12 cm -2 μN -1 , resulting in the significant intensity of trions in photoluminescence spectra of MoSe 2 at low temperature. We found that a hBN/MoSe 2 /hBN prepared with the "graphene-capping-assisted AFM nano-squeezing method" shows a strong excitonic emission with negligible trion peak, and the residual line width of the exciton peak is only 2.2 meV, which is comparable to the homogeneous limit. Furthermore, in this high-quality sample, we found that the formation of biexciton occurs even at extremely low excitation power (Φ ph ∼ 2.3 × 10 19 cm -2 s -1 ) due to the enhanced collisions between excitons.

A 57-year-old man developed migraine at the age of 25 years. Thereafter, he developed depression at the age of 50 years, and was admitted to a psychiatric hospital at the age of 54 years because of deteriorating depression. He returned to his work after receiving treatment for depression; however, he made mistakes several times in his work. He was referred to our hospital for further neurological evaluation. The results of the neurological examination performed on admission were unremarkable. His Mini Mental State Examination (MMSE) score was 24/30, and neuropsycological evaluations revealed executive dysfunction. There was no family history of dementia or cerebral infarction. Magnetic resonance fluid attenuated inversion recovery (MR FLAIR) image of the brain showed hyperintense lesions around the lateral ventricle without involvement in the temporal pole and external capsule. Despite a lack of family history of dementia and cerebral infarction and non-specific brain MRI findings, his history of headache and depression were suggestive of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Therefore, skin biopsy was performed; electron microscopy of the biopsied sample revealed granular osmiophilic material deposits. Genetic analysis of the NOTCH3 gene showed a missense mutation with substitution of R427C in exon 8, i.e., out of the hot-spot, exon 3, and 4. Thus, skin biopsy is a useful tool for diagnosing atypical CADASIL.

This study elucidates the genotypic and phenotypic spectrum and histopathological findings related to cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) in Japan. For this single-center retrospective observational study, we enrolled 215 patients who were clinically suspected of having CADASIL and were examined at Kumamoto University from 1997 to 2014, and we diagnosed CADASIL in 70 patients. We found 19 different NOTCH3 mutations in the patients, with the NOTCH3 Arg133Cys mutation being found most frequently. We also found the Arg75Pro mutation, a cysteine-sparing NOTCH3 mutation. CADASIL patients with this Arg75Pro mutation were frequently found throughout Japan, and fewer patients with the Arg75Pro mutation showed MRI hyperintensity in the anterior temporal pole compared with patients with other NOTCH3 mutations. Significantly more CADASIL patients with the NOTCH3 Arg133Cys mutation had hyperintensity in the external capsule compared with CADASIL patients with the other mutations not including the NOTCH3 Arg75Pro mutation. We also showed postmortem pathological findings of the first Japanese CADASIL case with the NOTCH3 Arg133Cys mutation, and histopathological findings of fresh frozen skin biopsy specimens of CADASIL patients. In conclusions, the spectrum of NOTCH3 mutations in Japanese CADASIL patients may be partially explained by founder effects. Genotype-phenotype correlations may exist in CADASIL, which should be considered so as to make an accurate diagnosis of CADASIL in each population. Fresh frozen skin biopsy specimens may aid detection of Notch3 deposits on vascular walls for an improved diagnosis of CADASIL.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) is one of the most common heritable causes of stroke and dementia in adults. The gene involved in the pathogenesis of CADASIL is Notch3; in which mutations affect the number of cysteine residues in its extracellular domain, causing its accumulation in small arteries and arterioles of the affected individuals. Besides the usual neurological and vascular findings that have been well-documented in CADASIL patients, this paper additionally reports multiple neoplastic lesions that were observed in an autopsy case of CADASIL patient; that could be related to Notch3 mutation. The patient was a 62 years old male, presented with a past history of neurological manifestations, including gait disturbance and frequent convulsive attacks. He was diagnosed as CADASIL syndrome with Notch3 Arg133Cys mutation. He eventually developed hemiplegia and died of systemic convulsions. Autopsy examination revealed-besides the vascular and neurological lesions characteristic of CADASIL- multiple neoplastic lesions in the body; carcinoid tumorlet and diffuse idiopathic pulmonary neuro-endocrine cell hyperplasia (DIPNECH) in the lungs, renal cell carcinoma (RCC), prostatic adenocarcinoma (ADC) and adenomatoid tumor of the epididymis. This report describes a spectrum of neoplastic lesions that were found in a case of CADASIL patient that could be related to Notch3 gene mutations.

Extensively distended and tortuous vessels on both sides of the uterus are rare incidental findings on transvaginal sonography in early pregnancy. A 31-year-old woman with a history of surgical repair for congenital intestinal stenosis was noted to have utero-ovarian varices on a transvaginal ultrasound examination during her first prenatal visit at 6 weeks' gestation. Magnetic resonance imaging revealed dilated ovarian veins along with infrarenal absence of inferior vena cava as well as the absence of external and common iliac veins. Despite concerns regarding spontaneous utero-ovarian vessels rupture, her antenatal course was uneventful. A vaginal delivery was successfully achieved without any postpartum complication. We also demonstrated an intraoperative view of the utero-ovarian vessels during cesarean section in her subsequent pregnancy. Absence of inferior vena cava and pelvic varices can occur in women with a history of neonatal surgery. Although the risk of utero-ovarian varices rupture remains unclear, vaginal delivery may be safely achieved., (© 2014 The Authors. Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology.)

Aim: Post-partum hemorrhage (PPH) is the leading cause of maternal mortality. Identification of the precise bleeding site is generally important to control hemorrhage, but such an approach has not been fully established in the context of PPH. We postulated that visualization of bleeding sites could aid treatment decisions in the management of PPH., Methods: We conducted a prospective review of 26 patients who underwent dynamic computed tomography (CT) for PPH., Results: A total of 17 cases presented with uterine bleeding, eight with vaginal hematomas, and one with hemoperitoneum. Overall, dynamic CT identified contrast media extravasation in the arterial phase in 12 of 26 (46.2%) cases: the upper (n = 4) and the lower uterine segment including the cervix (n = 2), subfascial space (n = 1) and vagina (n = 5). Identification of precise arterial bleeding sites using CT provided informative guidance about where to place balloons for intractable uterine bleeding, and how to manage hemoperitoneum and vaginal hematomas. In addition, dynamic CT revealed the existence of a subtype of uterine atony, which is characterized by focal active arterial bleeding in the upper uterine segment. Furthermore, negative contrast extravasation extracted cases of PPH that were well controlled without the need for surgical or radiological intervention. No patient required emergency hysterectomy to control PPH., Conclusion: Dynamic CT has potential clinical utility in treatment decision-making for PPH., (© 2013 The Authors. Journal of Obstetrics and Gynaecology Research © 2013 Japan Society of Obstetrics and Gynecology.)

An increasing number of women with severe endometriosis have conceived through assisted reproductive technology, but endometriosis can cause life-threatening complications for both the mother and baby during pregnancy. We describe a case of endometriosis-induced spontaneous ileal perforation in pregnancy with massive intestinal haemorrhage that required caesarean section, right hemicolectomy and terminal ileum resection at 33 weeks of gestation. Spontaneous perforation associated with intestinal endometriosis in pregnancy is a rare complication, and only seven cases have been reported in the English literature. To the best of our knowledge, this is the first report of spontaneous ileal perforation due to endometriosis. Moreover, this is probably the first case of massive intestinal haemorrhage in pregnancy that resulted from intestinal endometriosis. These risks must be explained to couples suffering from endometriosis-related infertility prior to conception by assisted reproductive technology, and multidisciplinary management may be mandatory for women with severe endometriosis in pregnancy., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Vulvar varicosities are generally not an indication for a caesarean section but in a rare case of severe vulvar varicosities, it would be a controversial issue whether to perform a caesarean section for fear they might rupture during a vaginal delivery. We present a case of huge vulvar varicosities during pregnancy. MRI revealed obstruction of the internal iliac system by the gravid uterus and subsequent incompetence of a collateral pathway via the round ligament vein that emptied into the ovarian vein. The patient went into labour at 38 weeks, and successfully vaginally delivered a baby. The vulvar varicose veins became compressed by the foetal head from the inside, and markedly diminished in size during crowning and after delivery. Women with vulvar varicosities can be allowed to attempt a vaginal birth regardless of their severity. The use of MRI aids in the overall anatomical understanding of vulvar varicosities in pregnancy., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

An intrauterine balloon tamponade is a simple but highly effective method for the management of postpartum hemorrhage. However, treatment failure can occur due to prolapse of an intrauterine balloon into the vagina. We present two cases with a successful maneuver in maintaining the intrauterine placement of the balloon by clamping the cervix with two ring forceps in the management of postpartum hemorrhage. Although the balloon was initially expelled through a dilated cervix, a cervical clamp using ring forceps prevented displacement of the balloon, and the hemorrhage ceased. Clamping the cervix with two ring forceps to retain the balloon can be a simple and readily available approach to consider when an intrauterine balloon tamponade does not work due to its expulsion., (© 2012 The Authors. Journal of Obstetrics and Gynaecology Research © 2012 Japan Society of Obstetrics and Gynecology.)

We present two cases of patients with juvenile amyotrophic lateral sclerosis (ALS), who had no history of familial ALS. The symptoms of both patients started as weakness of the unilateral upper limb and neck, and extended to bulbar and respiratory weakness in a relatively short period. Of note, the first patient was mentally retarded before the onset of weakness. Fused in sarcoma/translocated in liposarcoma (FUS/TLS) gene analyses revealed mutations of p. G492EfsX527 (c. 1475delG), which is a novel deletion/frameshift mutation, in the first patient and p. R514S mutation (c. 1542G > T) in the second patient. Molecular analysis revealed that the mutant FUS/TLS, especially the deletion/frameshift mutation, showed significant cytoplasmic localization in transfected motor neuron-like cells. Our findings suggest the association of mental retardation with the FUS/TLS mutation. Further investigation, including the effect of FUS/TLS on cognitive function, would aid better understanding of FUS/TLS proteinopathies.

A 68-year-old Italian woman who had a clinical history of thyroidectomy in 2002 presented with slowly progressing renal insufficiency and non-nephrotic proteinurea in 2004. A renal biopsy showed the occurrence of amyloid; the thyroid biopsy previously taken also revealed amyloid infiltration. Other amyloid-containing tissues included bone marrow and heart. The plasma cell level in the bone marrow was found to be less than 5% and both serum and urine samples were positive for a monoclonal kappa light chain band. DNA analysis unexpectedly revealed the presence of a novel transthyretin (TTR) mutation, ATTR Asn124Ser. Histologically, amyloid deposits in the thyroid had a homogeneous appearance with moderate Congophilia. In immunohistochemistry, a kappa light chain antiserum showed positive immunoreactivity with amyloid deposits in the thyroid. Furthermore, a TTR antiserum, anti-TTR50-127, also recognized a number of amyloid deposits stained positive with the kappa light chain antiserum. Overall, the kappa light chain antiserum reacted with most of the amyloid deposits in the thyroid, whereas TTR immunoreactivity was scarcer, with a scattered appearance. In contrast, only the anti-TTR50-127 antiserum labeled amyloid in the kidney, albeit not all deposits. In this study, we report a patient having a novel TTR variant, ATTR Asn124Ser, with co-localization of kappa light chains in the amyloid deposits in the thyroid tissue.

A 68-year-old man was admitted to our hospital with left hemiparesis, unilateral spatial neglect, and anosognosia at nine hours after blunt neck trauma. Carotid ultrasonography and angiography revealed occlusion of the right common carotid artery. A second ultrasonographic examination detected a double lumen, intimal flap, and a movable thrombus at the occlusion site, with reversed flow in the external cerebral artery and antegrade flow in the internal cerebral artery. Magnetic resonance angiography showed occlusion of the M1 distal section of the right middle cerebral artery. We diagnosed dissection of the right common carotid artery and artery-to-artery embolism from the thrombus in the dissecting common carotid artery. We started anticoagulation on the second hospital day. The patient was able to walk independently at discharge. The finding of carotid dissection on ultrasonography varied during the acute phase. We observed a mural thrombus in the true lumen and vessel recanalization. Treated only by anticoagulants without surgical therapy, the patient had a good outcome without reattack or hyperperfusion syndrome.

We present two patients who developed bilateral border zone brain infarctions after massive bleeding. Patient 1 was a 46-year old woman who developed bilateral visual disturbance and left hand monoparesis after excessive menstruation with severe anemia. Diffusion-weighted MRI of brain showed multiple border zone infarcts bilaterally in cerebral and cerebellar hemispheres while brain MRA was normal. Patient 2 was a 67-year old man who developed disturbance of consciousness and right hemiplegia after upper gastrointestinal tract bleeding. Diffusion-weighted MRI of brain showed bilateral multiple border zone infarcts in cerebral hemispheres, although he had asymptomatic occlusion of the left internal carotid artery. The bilateral multiple border zone infarcts are one of the key findings suggesting the presence of anemic hypoxia as a result of global brain hypoperfusion caused by massive blood loss.

A 38-year-old man was admitted to our hospital with headache, dysarthria and paraparesis. Brain CT and diffusion MRI disclosed cerebral infarction at bilateral anterior cerebral artery (ACA) territories. His symptoms and signs deteriorated in several days despite intensive antithrombotic therapy, resulting in right hemiparesis, akinetic mutism, memory disturbance, change of personality, urinary incontinence, forced grasping, and starting delay of speech and motion. Cerebral angiography demonstrated occlusion with contrast pooling at the right ACA A2 portion. Stenosis and dilatation were found at left ACA A2 portion. An intimal flap was also demonstrated on serial angiography. This case was diagnosed as cerebral infarction caused by dissection of bilateral ACA. Although no definite primary arteriopathy was demonstrated, bilateral dissection could be occurred simultaneously.