This case was an extremely rare form of cervical dysgenesis that presented with cyclic pain. Diagnostic laparoscopy and vaginoscopy showed the presence of a blind uterus at the level of the internal cervical os with a normal vagina and exocervix. Mullerian ducts are the embryologic origin for the uterus, cervix and upper part of the vagina. Mullerian duct migration initiates from the upper part of the Mullerian system. Therefore an obstructed uterus is usually associated with cervical and upper vaginal anomalies. This case was unusual because of the presence of an isolated segmental atresia at the level of the internal cervical os. However the vaginal portion of the cervix, vagina and urinary system were normal. We theorized that the absence of an appropriate fusion between the Mullerian duct and its underlying mesoderm, loss of cell-to-cell communication and special gene expression during a critical time period or a vascular accident between 12–22 weeks of gestation might have caused this anomaly. The patient underwent a laparotomy to create a utero-cervical canal using a peritoneal graft.

Introduction: Herlyn-Werner-Wunderlich Syndrome (HWWs) is a rare variant of Mullerian duct anomalies. It is associated with a wide range of gynecological and obstetric complications, such as urinary incontinence, urinary retention, endometriosis, pelvic infection, pelvic pain and infertility. Case presentation: We conducted the present study to investigate and manage HWWs with pelvic pain. The surgery was performed on the operative room of Imam Reza Hospital in April 2018. Conclusion: The reported case is related to Herlyn-Werner-Wunderlich Syndrome, a rare congenital Mullerian duct anomaly, which it is not included in the current classification system of the Mullerian abnormalities of the American Fertility Association and is based on the typical pattern of caudal evolution towards cranial.

Introduction: . Various methods have been proposed to treat Cervicovaginal agenesis with the functional uterus, but unfortunately, some of these methods eventually lead to a hysterectomy. In this study, we investigated a new surgical method in treating these patients and its short and long-term consequences, which is performed for the first time in the world by the laparoscopic method. Methods: Seven women aged 18 (12-27) years who had a functional uterus with cervicovaginal agenesis (between 2016 and 1398) in the Ghadir and Dena hospital underwent cervicovaginoplasty with sigmoid colon replacement by laparoscopic method to establish normal menstrual cycles and have a functional uterus and vagina. The average follow-up duration was 25. 9 months (2-48 months). Results: The mean BMI of the patients was 19. 7 (17. 6-22. 4). The average time interval between the initiation of disease symptoms and the operation was 52. 28 months (2-156 months). All the patients had regular menstrual cycles. The average length of the vagina was 8. 9 cm (7. 6-10. 5 cm). One patient observed proximal stenosis of neovagina 12 months after surgery. We had no significant postoperative complications. We did not perform hysterectomies on our patients. Two subjects had sexual activities without dyspareunia, post-coital bleeding, or malodor vaginal discharge. Conclusion: Sigmoid cervicovaginoplasty is a safe and effective procedure with satisfactory long-term outcomes. This surgery eliminates the psychological burden of hysterectomy in these patients.

Background: Gonadal dysgenesis, the most common cause of primary amenorrhea, is characterized by absent or underdeveloped ovaries. Although the coexistence of gonadal dysgenesis and Mayer-Rokitansky-Kü ster-Hauser (MRKH) has been reported, it is still quite infrequent. To the extent that authors searched, just one study reported the association between Rokitansky sequence and Dandy-Walker malformation. Clinical Presentation and Intervention: We aimed to report a case with gonadal dysgenesis, MRKH, and the Dandy-Walker variant. In this care report, the authors reported a 15-year-old girl with primary amenorrhea and underdeveloped secondary sexual properties. Her karyotype was 46, XX. The abdominopelvic MRI without contrast demonstrated bilateral ovarian agenesis and no uterus or cervix. Vagina was normal in length. Brain MRI was consistent with the Dandy-Walker variant. Conclusion: Although some affected chromosomal regions have been identified, further genetic analyses should be performed to elucidate the probable association between these anomalies.

Bilateral maxillary sinus aplasia is an extremely rare congenital anomaly. Only a few cases have been reported in the literature. We present a 24- year-old woman with bilateral maxillary sinus aplasia associated with the bilateral absence of the uncinate process and infundibular passage on CT images.

Background & Aims: aplasia Cutis Congenital (ACC) is the most common benign isolated defect but it can be associated with other physical anomalies or malformation and syndromes. No unifying theory can account for all lesions of ACC. Because this condition is the phenotypic result of more than one disease process; it is likely that more than one mechanism is involved. Mechanisms include genetic factors, teratogens, compromised vasculature to the skin, and trauma. Frieden created a classification system for ACC consisting of 9 groups based on number and location of acc and the presence or absence of associated malformations.Materials & Methods: In this observational cross-sectional study, a census method for gathering information was used. The obtained data were analyzed descriptively via EP16 software. we have studied 20 newborns, 9 males, and 11 females with skin defects, from September 1997 to September 2005 in Akbarabadi hospital.Results: In this study, the skin lesions of 85% were single, 65% on the scalp, 50% on the vertex, 5% on parietal, 15% on parietoocciputal, 15% on lumbosacral, 20% on extremities, and 15% with trisomy13. Mothers of two newborns had the history of drug use and abuse during pregnancy. Extensive skin lesions during neonatal period have high mortality rate, in our study also the rate of mortality with extensive lesions were 55%.Conclusion: According to this study isolated skin defect was observed in most of the cases (85%), and the most signifant cause of newborns’ mortality with skin defect was sepsis.

Backgrounds & Aims Rokitansky-Kü ster-Hauser (MRKH) syndrome is a rare genetic disease that can be one of the causes of primary amenorrhea in young girls. These patients usually refer with primary amenorrhea despite the normal appearance of the genital tract and normal secondary sexual traits. Although these patients have no other concomitant symptoms, a small percentage of functional endometrial islets are observed. This report investigated a 19-yearold young woman with Rokitansky-Kü ster-Hauser syndrome with severe endometriosis. Patient Information The patient was a 19-year-old woman who referred to the hospital with primary amenorrhea and severe cyclic pain and was diagnosed with uterus larger than normal with vaginal agenesis and cervix in early studies, with primary uterine enlargement and severe endometriosis due to Rokitansky syndrome. So the patient underwent hysterectomy and was a candidate for vaginoplasty Conclusion Functional endometriosis in patients with Rokitansky syndrome is approximately 7-10%, but in the event of primary amenorrhea and symptoms of cyclic pain in the patient, Rokitansky syndrome should be considered and for preventing secondary endometriosis caused by it, cutting off the monthly menstrual cycles or bringing out the uterus containing the endometrium or creating an anastomosis between the functional uterus and vaginal, if it is possible, seem logical techniques.

Cutis aplasia is a rare, benign, congenital, and localized skin defect disorder with multiple underlying causes. Vascular events are one of the underlying causes. However, they are not a common cause. Small cutis aplasia without bony defect gradually heals without any complication. Rare complications are seen in scalp aplasia with bony defect like hemorrhage, meningitis and thrombosis. We reported a neonate with limb skin aplasia of 4×3 cm size and normal other physical signs and symptoms. Gradually he developed overriding of skull sutures near his discharge time. We found a similar picture of the brain ischemia in imaging on the 24th day of his birth. This case and the similar cases may suggest an association between the vascular events and the cause of the disease. Thus, it is important to consider thrombosis and other vascular events in every patient with cutis aplasia even in a neonate with normal examination in the first day. We recommend that physicians should not judge that the disease always has a good prognosis and follow up the patients’ condition anyway.

aplasia cutis congenita (ACC) is a rare congenital disorder that commonly affects the scalp. In this disease, some parts of the skin with or without underlying structures were not formed at birth. The aplastic lesion always presents as a solitary lesion; however, multiple lesions also were reported. In more severe cases, deeper structures, such as the subcutaneous tissue, bone, and dura, can be affected. In this study, we report a case of ACC and its management.

Background: aplasia cutis congenital (ACC) is a congenital absence of skin most commonly affecting the scalp. No definite etiology is available but multiple causes such as intrauterine infection, fetal exposure to cocaine, heroin, alcohol or antithyroid drugs, vascular disruption, genetic causes, syndromes and teratogens have been suggested.Case Presentation: We present an infant with symmetrical type of aplasia cutis on the trunk and proximal limbs. She was product of triple pregnancy with two fetuses papyraceous at 12th week of gestational age and at birth. She is treated by non surgical management despite remarkable extent of the lesion.Conclusion: ACC of the trunk is less common than of scalp. Lesion often is symmetric and seen after fetus papyraceous in multiple pregnancies.