A very rare case report of bilateral maldescended ovaries and müllerian duct anomaly associated with inflammatory myositis, myasthenia gravis and thymic pathology

Background Maldescended ovaries are a rare condition. Despite its different embryologic development with the uterus, maldescended ovary is usually accompanied by uterine malformations and is found during the course of infertility. In other cases, it may be incidentally diagnosed in examinations due to abdominal pain or in a survey of finding paraneoplastic origin. Probable immune-related developmental conditions are associated with this abnormality; sometimes cross-reaction with other immune-related diseases is possible. Case presentation Here, the probable paraneoplastic origin is surveyed for a patient with coexisting inflammatory myositis and myasthenia gravis. According to this survey non recognized Mullerian duct and ovarian anomalies were found. Conclusions Knowledge about this anatomical abnormality is helpful for clinicians to prevent misdiagnosis and improper management. Moreover, understanding the probability of accompanying other conditions such as immune-related and neuromuscular junction disorders with Mullerian duct anomalies can offer a comprehensive insight.

Apixaban Causing Hepatic Cystic Bleeding: A Rare but a Life-Threatening Complication

Cystic lesions of the liver are a heterogeneous group of disorders with varied etiology, prevalence, and clinical manifestations. Fibropolycystic liver disease encompasses a spectrum of related liver and biliary tract lesions caused by abnormal embryologic development of the ductal plates. These disorders include congenital hepatic fibrosis, biliary hamartomas, polycystic liver disease (PCLD), choledochal cysts, and Carolis disease. PCLD is arbitrarily defined as a liver that contains >20 cysts. Most liver cysts are incidentally found on imaging studies, and the majority of the patients with liver cysts are asymptomatic. Rarely, complications such as compression, infection, and bleeding within the cyst can occur. Under the effect of the increased pressure, the epithelial lining of the cyst undergoes necrosis and sloughing, causing injury of the fragile blood vessels, leading to intracystic bleeding. The bleeding within or from the cyst can be precipitated by anticoagulation. We present a patient with PCLD who developed intracystic bleeding after he was started on apixaban for the prevention of thromboembolism.

The cavernous carotid inferolateral trunk and persistent primitive maxillary arteries: analysis of dissected arterially injected fetal specimens and high-resolution micro-CT of the dog’s anastomotic arteries

Purpose The presence of a persistent primitive maxillary artery is described in the literature dealing with the development of the cavernous carotid inferolateral trunk, and the relevant similarities of the cranial circulation of the human and dog. The literature includes no dissection photographs of the above-mentioned two human fetal arteries, only diagrammatic representations. This study’s objectives were to analyze photographs of fetal dissections for the presence of these two arteries, and also investigate the possibility of obtaining, in preserved dog specimens, high-resolution micro-CT imaging of arteries homologous with the above-mentioned two human arteries. Methods The literature describing the embryologic development of the cavernous carotid inferolateral trunk, the persistent primitive maxillary arteries, and their homologies in the dog was reviewed. Relevant dissections of fetal specimens were analyzed. High-resolution micro-CT images of un-dissected dog arteries were produced and analyzed. Results Photographs of fetal specimen dissections demonstrate the cavernous carotid inferolateral trunk. A separate persistent primitive maxillary artery was not present in the dissected specimens. High-resolution micro-CT images of the dog demonstrate homologous arteries with segments of the human inferolateral trunk, and other skull base and brain arteries. Conclusion This investigation provides the only photographs in the literature of dissected human fetal cavernous carotid inferolateral trunks. A persistent primitive maxillary artery was not present in the dissected specimens and is a non-existent structure, likely a previously misidentified carotid inferolateral trunk. High-resolution micro-CT images of the dog visualized arteries that are homologous to segments of the human cavernous carotid inferolateral trunk artery.

Thoracoabdominal Compartment Syndrome

As we advance our knowledge in understanding abdominal compartment syndrome, it is worth going back to revisit our basic embryologic development of the main determinant of the abdominal and thoracic cavities, i.e., the diaphragm. The abdominal and thoracic cavities used to be one cavity at some stage of the embryonic life — “intraembryonic coelom” — before the “septum transversum” — diaphragmatic origin — divided it into two cavities. Therefore, if a condition develops that will impair the diaphragm from separating the cavities, leading to the possibility of pressures to transmit from one cavity to another, this becomes relevant as abdominal compartment syndrome. Diaphragmatic eventration is a congenital developmental defect in the muscular portion of the diaphragm with preserved attachments to the sternum, ribs, and dorsolumbar spine, leading to a semi-membranous diaphragm that anatomically separates the two cavities, but not physiologically. In the case of high abdominal pressure, the pressure will transmit to the thoracic cavity, causing derangement in both the anatomy and physiology. This was reported and named “Thoracoabdominal Compartment Syndrome”.

Rethinking the biology of metastatic melanoma: a holistic approach

Over the past decades, melanoma-related mortality has remained nearly stable. The main reason is treatment failure of metastatic disease and the inherently linked knowledge gap regarding metastasis formation. In order to elicit invasion, melanoma cells manipulate the tumor microenvironment, gain motility, and adhere to the extracellular matrix and cancer-associated fibroblasts. Melanoma cells thereby express different cell adhesion molecules like laminins, integrins, N-cadherin, and others. Epithelial-mesenchymal transition (EMT) is physiological during embryologic development, but reactivated during malignancy. Despite not being truly epithelial, neural crest-derived malignancies like melanoma share similar biological programs that enable tumorigenesis, invasion, and metastasis. This complex phenomenon is termed phenotype switching and is intertwined with oncometabolism as well as dormancy escape. Additionally, it has been shown that primary melanoma shed exosomes that create a favorable premetastatic niche in the microenvironment of secondary organs and lymph nodes. Although the growing body of literature describes the aforementioned concepts separately, an integrative holistic approach is missing. Using melanoma as a tumor model, this review will shed light on these complex biological principles in an attempt to clarify the mechanistic metastatic pathways that dictate tumor and patient fate.

Intracranial arachnoid cysts in an infant: A technical note on the innovative use of navigation and flexible endoscopy for cyst fenestration

Background: Intracranial arachnoid cysts (ACs) are a cerebral spinal fluid (CSF) collection within the meninges. They typically arise during embryologic development. Some are stable overtime with little consequence, but large or growing cysts may require surgical intervention. The optimal surgical technique is debated and may be more technically challenging in the infant age group. Case Description: Our unique case report details a 10-month-old (6 months corrected age) infant who presented with a drastic increase in head circumference and was found to have midline shift and three cysts – one large and two smaller ones. He was treated with an innovative surgical approach combining stereotactic introduction of a catheter to facilitate subsequent flexible endoscopy allowing three separate cysts to be treated through one small surgical incision with no complications and a stable examination on 2-year follow-up. Conclusion: Symptomatic ACs in the infant population that require treatment can be addressed with open surgery to fenestrate the cyst, endoscopic cyst fenestration, or cystoperitoneal shunting. Typically, surgeons must choose between a rigid endoscope which allows stereotactic navigation or a flexible endoscope which allows multiple trajectories but precludes navigation. Our case demonstrates that combining stereotactic ventricular placement before flexible endoscopy provides the benefit of both approaches and allows for successful endoscopic treatment in a young patient with durable results.

Congenital diaphragmatic hernia: a retrospective study regarding the clinical experience of Bihor County Genetics Center

Congenital diaphragmatic hernia is an abnormality of embryologic development characterized by the persistence of communication between the thoracic and abdominal cavities, followed by an extremely high death rate despite medical progress. In Bihor county, according to a retrospective analysis of patient records from the Regional Center of Genetics, 30 children were diagnosed with congenital diaphragmatic hernia over a span of 28 years, from 1990 to 2018, most being male (73.3%). Predominant were the complex congenital diaphragmatic cases, meaning the cases of diaphragmatic hernia associated with other congenital abnormalities, 67% (20 patients) presenting at least one other congenital malformation. 95% (19 cases) of patients with complex congenital diaphragmatic hernia presented pulmonary abnormalities, the most frequent being pulmonary hypoplasia; 35% (7 cases) presented cardiac abnormalities. The Apgar score at 5 minutes after birth was significantly lower for patients with the clinical form of complex congenital diaphragmatic hernia. The mortality for the patients presenting the complex form was 100%, and for the patients presenting the isolated form was 20%. Karyotype analysis was performed for 17 patients, and 23.53% of them (4 patients) presented chromosomal abnormalities.

Technical Considerations In Performing One Anastomosis Gastric Bypass In Case of Intestinal Malrotation

Intestinal malrotation is a rare congenital anomaly that originates from the failure of the normal rotation and fixation of the midgut during embryologic development. There are different types of Midgut Malrotation which includes non-rotation, incomplete rotation, reverse rotation, or anomalous fixation of the mesentery. Methods: We present a case of a 58-year-old woman with a BMI of 45.3 kg/m2. The patient underwent laparoscopic appendectomy 15 years ago and was diagnosed with a partial situs inversus. She has no other past medical conditions. Results: At the operation, four meters of small bowel were counted backward from the ileocaecal valve until the duodenum. We performed a one anastomosis gastric bypass with three meters of common limb and one meter of biliopancreatic limb, gastro-intestinal anastomosis made with a linear stapler, and the defect sutured with absorbable suture. Conclusion: One anastomosis gastric bypass is a feasible and safe alternative for morbidly obese patients with intestinal malrotation or multiple intestinal adhesions.

Large Canal of Nuck Hernia: The Female Equivalent of the Inguinoscrotal Hernia

Our patient was a 78-year-old woman with a large indirect inguinal hernia who underwent Lichtenstein repair. Literature review of the pathophysiology of indirect hernias revealed important discussion of the canal of Nuck in women and understanding embryologic development of ovaries and descent of testes.