Defective blastogenesis of postnatally diagnosed type VI sirenomelia in a young primigravida: A case report.

Sirenomelia is a rare lethal multi-systemic birth malformation in which the two lower limbs are replaced with a rotated single midline tail-like limb. Several hypotheses try to explain this syndrome, with the most prominent theories being the "vascular steal hypothesis" and the "defective blastogenesis hypothesis." We report a case of a baby with sirenomelia who had a single femur and a single tibia, which classify the case as type VI on Stocker and Heifetz classification. The only risk factor in our case is young maternal age. The baby had a single umbilical artery, a prominent feature of the vascular steal hypothesis. Nonetheless, it also had upper limb deformity, which can be better explained by the defective blastogenesis hypothesis. Our case supports the defective blastogenesis theory of sirenomelia more than the vascular steal hypothesis as it has both a single umbilical artery and upper limb deformity. Also, our case serves as a teaching lesson that indicates the importance of an obstetric ultrasound before a cesarean section has to be done to avoid unnecessary surgery for life incompatible congenital anomaly.

A large post-caesarean Niche (Isthmocele) with amenorrhea, a symptom that was not reported in the medical literature: A rare case report.

INTRODUCTION AND IMPORTANCE: Isthmocele is identified as an iatrogenic defect in the myometrium of the anterior uterine wall at the site of a previous cesarean scar due to defective tissue healing. Patients may have varied symptoms including abnormal uterine bleeding (AUB) and pelvic pain. Herein, we report a rare case of a large isthmocele that manifested with secondary amenorrhea; which was not reported in the medical literature previously. CASE PRESENTATION: A 30-year-old Syrian woman, G5P5, came to our clinic with a complaint of secondary amenorrhea that began two years ago. She was treated symptomatically with progesterone with no response. She has had five cesarean sections. Ultrasonography findings suggested a large uterine niche. Trans-Abdominal niche repair was the obtained technique, depending on the drainage of the isthmocele, excising the fibrotic tissue from the edges and re-approximating them. On follow-up, menstruation returned to normal. CLINICAL DISCUSSION: Isthmocele can be, radiologically, defined as a hypoechoic or anechoic, triangular area at the scar site. Its pathophysiology is still unknown. Although, an isthmocele can be diagnosed using a variety of imaging techniques like ultrasonography (US), magnetic resonance imaging (MRI), sonohysterography, and hysteroscopy; transvaginal ultrasound (TVUS) is the first method described for assessing it. The goal of isthmocele treatment is to alleviate symptoms. CONCLUSION: We recommend that health awareness campaigns alert people to the need to see a specialist doctor in the context of a serious complaint. For the uterine niche, many risk factors can be avoided to reduce its probability.

Successful management of Herlyn-Werner-Wunderlich syndrome in a 17-year-old virgin girl in the challenging socio-cultural-logistic setting of Syria: a case report.

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare developmental anomaly of the female reproductive system caused by a failure of fusion during Mullerian duct development. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes HWWS. The most common presenting symptoms are dysmenorrhoea, pelvic pain, primary infertility in later years, and an abdominal mass due to hematometrocolpos. Case Presentation: A 17-year-old girl presented to the authors' department with recurrent low back pain, which was neither responsive to analgesics nor associated with urinary complaints, vomiting, or fever. Imaging techniques confirmed she had the triad of uterus didelphys, obstructed hemivagina, and right renal agenesis. Clinical Discussion: The genital system is the same for males and females before 6 weeks of pregnancy. HWWS is a rare congenital disorder as a result of the failure of fusion during Mullerian duct development. It consists of a didelphic uterus, hemivaginal septum, and unilateral renal agenesis. Conclusion: Shame and social stigma associated with virginity continue to endanger the lives of many girls in Syria. To complicate matters further, the low resources in Syria produced by war pose a difficult challenge in managing many gynecological conditions, including HWWS, like this case, in which endoscopic technologies were not available, necessitating open surgery while keeping in mind preserving hymen intactness. So, the authors indicate that preserving virginity could be conducted even though the approach is open surgery by very careful intervention and experienced surgeons.

Rare manifestations of alobar holoprosencephaly and the potential causes: a report of two cases.

Holoprosencephaly is a rare and possibly fatal neural tube defect represented by complete or partial forebrain noncleavage. It can be classified into four types: alobar, semilobar, lobar, and middle interhemispheric fusion variant. It is usually diagnosed through prenatal ultrasound or after birth by visually observing the morphological abnormalities and/or through neurological screening. Potential causes include maternal diabetes, alcoholism, infections during pregnancy, drugs, and genetic causes. Case presentation: Herein, we report two cases of holoprosencephaly's rarest manifestations, albeit cebocephaly in the first case, and cyclopia with a probocis in the second. Cebocephaly, (hypotelorism with a single nostril and a blind-ended nose) was present in the first case; a Syrian newborn girl for a 41-year-old mother who works in collecting Capparis spinosa, and cyclopia with skull vault absence and posterior encephalocele in the second case; a Syrian newborn girl for a 26-year-old mother, the parents here where second-degree relatives. Conclusions: Early diagnosis through ultrasound is preferred in such cases and management options should be assessed and discussed with the parents due to poor prognosis. Adherence to pregnancy follow-up programs is essential to detect malformations and disorders as early as possible, especially when risk factors exist. Also, this paper may suggest a potential correlation between C. spinosa and holoprosencephaly. Therefore, we suggest that more research should be done.

Methotrexate for management of twin pregnancy with complete hydatidiform mole and co-existing live fetus: A case report.

INTRODUCTION AND IMPORTANCE: Here, we discuss novel management with methotrexate for the rare case of a complete hydatidiform mole with a co-existing fetus (CHMCF). The management of CHMCF is controversial, and methotrexate might represent a solution. CHMCF management with methotrexate needs more study, especially its side effects, safe dosage, and the permissible period of pregnancy. CASE PRESENTATION: A 23-year-old Syrian primigravida came to our hospital with vaginal bleeding. The patient was diagnosed with a complete hydatidiform mole with a co-existing fetus. The mother had no complications but elevated B-HCG. After counseling, the decision was made to continue pregnancy with methotrexate to control B-HCG levels. The outcome was favorable though the infant had tetralogy of Fallot. CLINICAL DISCUSSION: In our case, the patient was stable except for the elevation of B-hCG levels, so we considered methotrexate to control it. On the other hand, methotrexate is considered a human teratogen. Case reports and case series of exposure to it during pregnancy began appearing in the 1960s. The sensitive period is suggested to be 6 to 8 weeks after conception. After discussing the choices with the patient, she elected to continue pregnancy and accepted methotrexate exposure to control B-hCG levels despite its risks. CONCLUSION: Methotrexate usage within a safe dosage should be studied more to determine the benefits and risks it carries in cases such as ours.

A Neglected Case of Multifocal Liposarcoma Presented in Five Different Sites in a Syrian Woman: A case report.

INTRODUCTION: Multifocal soft tissue sarcoma is a rare clinical entity occurring in 1 % of patients with extremity soft tissue sarcoma and in 4.5 % of patients with liposarcoma. Multifocal disease may arise either synchronously or metachronously and has been associated with poor prognosis. CASE PRESENTATION: Herein, we report a rare case of a Syrian woman patient with six different foci of liposarcoma in five sites at the time of diagnosis. DISCUSSION: Liposarcomas are currently classified into four different subtypes based on histologic or genetic analysis according to the World Health Organization (WHO), including cell-differentiated, dedifferentiated, myxoid, and pleomorphic. In the present work, we report a neglected patient with a multicentric round cell liposarcoma in five different sites. CONCLUSION: Medicine cannot provide much care for these advanced cases of multicentric liposarcoma so far. Therefore, more research should be conducted to improve the ability to manage these entities and to identify potential novel therapies.