Management of congenital cervical atresia with vaginal aplasia: A case report and review of the literature.

INTRODUCTION: Vaginal agenesis is a rare congenital condition, with an incidence of 1 in 4500 female births. CASE REPORT: We present a clinical case of vaginal aplasia with cervical atresia in a 31-year-old woman with primary amenorrhea. We aim to report the diagnostic process and provide a comprehensive outline of different possible treatments. DISCUSSION: The most common etiology of these agenesis cases is Mayer-Rokitansky-Küster-Hauser syndrome associated with uterine aplasia. However, vaginal aplasia can occur in 9 % of cases where the uterus is present. During embryogenesis, the Müllerian ducts give rise to the fallopian tubes, uterus, and upper two-thirds of the vagina, while the lower portion of the vagina develops from the urogenital sinus. Vaginal aplasia arises from a failure in the development of the terminal portion of the paramesonephric ducts. Abdominal pain, especially periodic pain, is the most common symptom, followed by primary amenorrhea. MRI is considered the gold standard for the diagnosis and precise description of female genital tract anomalies. CONCLUSION: Total hysterectomy remains a preferred option for cases of complete vaginal atresia to mitigate the risk of cervical or vaginal stenosis, adhesions, and pelvic inflammation resulting from poor menstrual blood drainage.

Antenatal imaging diagnosis of thoraco-omphalopagus conjoined twins.

Conjoined twins occur in an estimated one in every 200,000 live births. The etiology remains largely speculative, with genetic and environmental factors being considered. The thoraco-omphalopagus type accounts for approximately 40% of cases, making it a focal point for clinical and radiological research. Radiological imaging plays a pivotal role in delineating anatomical details, offering insights into the feasibility of surgical interventions and informing parental counselling regarding prognosis and management options. We present a case of thoracoomphalogus conjoined twins diagnosed during the third trimester of pregnancy in a 19-year-old woman. The detailed radiological assessment using ultra-sound and MRI provided crucial information on organ sharing and vascular anatomy, which is critical for management strategies. This case underscores the critical role of prenatal imaging in detecting complex congenital anomalies, facilitating informed decision-making by healthcare providers and families.

Angiomyofibroblastoma of the vulva: A case report and review of the literature.

Angiomyofibroblastoma (AMFB) represents a rare, benign mesenchymal tumor with a predilection for the vulvovaginal region. It is usually diagnosed in middle-aged women. Histopathology and immunohistochemical study remain the key to diagnosis. Like other benign mesenchymal vulval tumors, AMFB shows indolent behavior and rarely recurs after complete surgical excision. Herein, we present a case of vulvar AMFB in a 51-year-old woman to highlight the diagnostic difficulties when considering this rare entity.

Management of severe factor XI deficiency in pregnancy: A case report.

Factor XI (FXI) deficiency is one of the rarest coagulation disorders with a frequency of 1:1,000,000. A 30-year-old woman, diagnosed with FXI deficiency at the age of 4, was admitted to our maternity service at 25 weeks of gestation. The patient had no history of hemorrhage or abnormal bleeding, and the clinical examination was unremarkable. Antenatal care was also normal. The parturient was admitted in early labor at 38 weeks of gestation. Despite the absence of clinical hemorrhagic syndrome, a transfusion of fresh frozen plasma combined with tranexamic acid was initiated once the active stage of labor was started. Management of FXI deficiency in pregnant women is a challenge due to its unpredictable bleeding tendency, and careful planning and knowledge of appropriate hemostatic management is pivotal for their care.

[Chronic inflammatory bowel diseases: what happens when SARS-CoV-2 occurs? Preliminary results from a study conducted at the Hassan II University Teaching Hospital in Fes, Morocco (a case report)]. / Les maladies inflammatoires chroniques de l'intestin: que se passe-t-il quand le SARS-CoV-2 arrive? Résultats préliminaires du Centre Hospitalier Universitaire Hassan II de Fès, Maroc (à propos d'un cas).

SARS-CoV-2 infection is a major concern and a new threat to immunocompromised patients. Patients with chronic inflammatory bowel diseases (IBDs) are at increased risk of infections, in particular when they have active disease and are on immunosuppressive treatment. The purpose of this study was to assess the clinical, biological and radiological features of three patients with COVID-19 associated with chronic IBD as well as their management and outcomes. The study was conducted at the Hassan II University Teaching Hospital in Fes, Morocco over a 3-month period. We assessed all patients with disease onset. All patients had mild symptoms or were asymptomatic. No changes or delays in treatment regimens occurred and none of patients developed severe COVID-19. Reverse transcription polymerase chain reaction (RT-PCR) test results were positive in all patients. Radiological examinations were conducted. Chest scanner showed ground-glass opacities in one case. Treatment was based on hydroxychloroquine with azithromycin. Outcome was good in all cases. This preliminary report suggests that patients with chronic IBD aren't at higher risk of developing COVID-19 compared to the general population.

The Prognostic Value Of The ART Score Before The Second Transarterial Chemoembolization.

The transarterial chemoembolization (TACE) is a firstline therapeutic option for advanced hepatocellular carcinoma (HCC). Their indications are clearly defined by learned societies but the challenge is to determine the optimal number of TACE sessions that will benefit patients before switching to other therapies. For this reason, the Assessment for Retreatment with Transarterial chemoembolization (ART) score has been developed. The objective of our work is to show the prognostic value of the ART score before the second TACE. METHODS: This is a retrospective and prospective study of patients with hepatocellular carcinoma on cirrhosis liver who received a TACE between January 2012 to July 2019. The diagnosis of HCC was made according to the non-invasive criteria of EASL with the use of histology for doubtful cases. The ART score was calculated after the first chemoembolization. Patients were divided into 2groups: group A with an ART score between 0 and 1.5 and group B with a score =2.5. RESULTS: During the study period, 58 patients with HCC on cirrhosis liver received a TACE: 55.17% had an ART score between 0 - 1.5 before the second session and 44.8% had an ART score =2,5. Both groups were comparable regarding age, circumstances of discovery and Child's score. The size of the HCC as well as the value of the AFP was further increased in the group B. We observed a significant difference in the radiological response, the Child score and aspartate transaminase rate between the two groups after the first TACE. The overall survival rate at 3 years was 81% in group A versus 19% in group B. CONCLUSION: The ART score has an independent prognostic value and should be taken into account in the therapeutic strategy before the second TACE.

[IgG4-related disease: about 3 cases]. / La maladie à IgG4: à propos de 3 cas.

IgG4-Related disease (IgG4-RD), formerly known as IgG4-related autoimmune polyexocrinopathy, is a new condition including Plasminogen Activator Inhibitor-1 (PAI-1). It can affect different organs (central nervous system, salivary glands, thyroid, lungs, pancreas, bile ducts, liver, digestive tract, kidneys, prostate, etc.) with symptoms depending on the organ that is affected. It is more common in men older than 50 years of age. Its incidence and prevalence are poorly known because it is an uncommon disease. It is most common in Asia, accounting for only 20-30% of PAI in the Western world. Diagnosis is based on histological examination which shows dense lymphoplasmocytic infiltration in the organ affected associated with IgG4-positive plasma cells (immunohistochemistry), organ fibrosis and obliterating venulitis, all this in the context of increased serum IgG4 levels in more than 80% of cases. Patients are sensitive to corticosteroid therapy, with a high risk of relapse after discontinuation of corticosteroid therapy. This leads to the use of immunomodulators, mainly: thiopurines (azathioprine or 6-mercaptopurine), methotrexate and more recently rituximab, which can also be used as induction therapy. Given recent advances, accurate histological and clinical criteria are currently known to limit inappropriate management such as surgery. However, knowledge gaps remain concerning: pathophysiology, identification of specific biomarkers other than IgG4, natural history of the disease and long-term cancer risk assessment, performances of diagnostic tools such as endoscopic ultrasound-guided pancreatic biopsy. As well, consensual international management should be defined in the early stages of the disease and when patients develop recurrences. The purpose of this study was to report 3 cases of IgG4-Related disease on the basis of clinical and radiological criteria as well as therapeutic response.

Small bowel adenocarcinoma complicating coeliac disease: a report of three cases and the literature review.

Coeliac disease is associated with an increased risk of malignancy, not only of intestinal lymphoma but also of small intestinal adenocarcinoma which is 82 times more common in patients with celiac disease than in the normal population. We report three additional cases of a small bowel adenocarcinoma in the setting of coeliac disease in order to underline the epidemiological features, clinicopathological findings, and therapeutic approaches of this entity based on a review of the literature. The three patients underwent a surgical treatment followed by adjuvant chemotherapy based on capecitabine/oxaliplatin regimen, and they have well recovered.

Dysphagia caused by a lateral medullary infarction syndrome (Wallenberg's syndrome).

A 68-year-old man was referred to our hospital for a dysphagia evolving for 10 days. Clinical examination had found neurological signs as contralateral Horner's syndrome, ipsilateral palatal paresis, gait ataxia and hoarseness. Video-fluoroscopy showed a lack of passage of contrast medium to the distal esophagus. Esogastroduodenoscopy was normal. The cranial MRI had shown an acute ischemic stroke in the left lateral medullar region and the diagnosis of Wallenberg syndrome (WS) was established. WS remains an unknown cause of dysphagia in the clinical practice of the gastroenterologist.

Torsion of a wandering spleen.

Wandering spleen is a rare condition defined as a mobile spleen only attached with its pedicle. It can be complicated by a volvulus, which is a surgical abdominal emergency. Preventing infarction is the aim of a prompt surgery that can preserve the spleen and then proceed to splenopexy. We report a rare case of torsion of a wandering spleen associated with a dolichosigmoοd.

[Gestationis pemphigoid. From three observations and in the light of a review of the literature]. / Pemphigoide gestationnelle. A propos de trois cas et revue de la littérature.

The gestationis pemphigoid is a rare auto-immune vesiculo-bulleuse dermatosis occurring during the pregnancy or of the postpartum. The diagnosis is evoked by the localization and the type of eruption like by which has occurred of successive pushes. The cutaneous biopsy in immunofluorescence confirms the diagnosis by showing the linear deposit of C3 along the basal membrane. The risk of complications fetales is high in the event of pemphigoid gestationis of or the interest of an early diagnosis and a treatment, the ci rests on the corticotherapy in topic or according to the gravity of the symptoms in general administration. From three observations and in the light of a review of the literature, the authors report the characteristics of this pathology.