Spontaneous uterine rupture revealing vascular Ehlers-Danlos syndrome: An uncommon case report.

INTRODUCTION AND IMPORTANCE: Vascular Ehlers-Danlos syndrome also referred to as Ehlers-Danlos Type IV is an uncommon autosomal dominant genetic disorder linked to connective tissue abnormality. Its evolution is marked by the occurrence of severe vascular, digestive and obstetrical complications. The current case highlights the importance of early diagnosis and physician awareness about this disorder as it can improve the patient's prognosis. CASE PRESENTATION: We present the case of a 34-year-old woman, who presented at 36 weeks of amenorrhea with labor pain. The labor evolution was marked by an increased fluctuating abdominal pain, a sudden loss of the fetal station detected during cervical examination and decelerations to 60 beats per min, leading to an emergency caesarean section. During the laparotomy, the patient presented a spontaneous bilateral extension of the cutaneous incision requiring the realization of stopping stitches. The fetus and placenta had been expelled via a 9 cm long uterine wall rupture also known as an open book uterine rupture. A live male infant weighting 2890 g was promptly delivered and transported to NICU for respiratory distress. Physical features typical of EDS-IV allowed us to suspect this disorder and genetic analysis identified the presence of COL3A1 gene mutation, confirming the diagnosis. CLINICAL DISCUSSION AND CONCLUSIONS: Early recognition of Vascular Ehlers-Danlos syndrome is of paramount importance to improve the prognosis of affected patients, who often present themselves with life-threatening situations. Clinicians should maintain a high index of suspicion for the clinical signs of this inherited connective tissue disorder that is characterized by distinctive features.

Malignant Degeneration of a Mature Ovarian Teratoma.

Mature cystic teratoma is the most common type of ovarian germ cell neoplasm, but occasionally, it can undergo malignant transformations, especially in postmenopausal women. These secondary malignant neoplasms are most commonly squamous cell carcinomas. The absence of clinical and radiological specificity of this transformation means that the diagnosis remains purely histological. Data is insufficient regarding the appropriate management given their rarity. However, the treatment is multidisciplinary and is based on surgery and a platinum-based chemotherapy regimen. We report the case of a 53-year-old postmenopausal female patient with malignant transformation of the ovarian teratoma who was treated surgically and whose outcome was favorable. The diagnosis of the teratoma was evoked on imaging, while the diagnosis of squamous cell carcinoma was revealed on histology. Malignant transformation is an uncommon complication of mature ovarian teratomas. No clinical, radiological, or biological sign is specific; therefore, resection of any ovarian mass, even asymptomatic, is required.