Ovarian inguinal hernia - a possibility in MURCS syndrome. Saini Rahul,Bains Lovenish,Kaur Tarangpreet,Lal Pawan,Pal Veer,Beg Mohd Yasir,Kaur Daljit Journal of ovarian research BACKGROUND:Inguinal hernia containing ovary and fallopian tube can be found in paediatric population and is a rare finding in women of reproductive age group. Most of the cases are associated with congenital abnormalities of the female genital tract. CASE PRESENTATION:A 20 year old female presented with right reducible inguinal hernia, primary amenorrhea and normal secondary sexual characteristics. Clinical examination revealed scoliosis with convexity towards left side, prominence of left rib cage with Sprengel deformity and right sided heart sounds. Ultrasound of the inguinal swelling revealed right ovary within the hernial sac, Chest X-ray revealed right lung collapse and dextrocardia. Further Magnetic resonance imaging (MRI) of pelvis revealed inguinal hernia with right ovary as its content, normal left ovary and absent uterus. Computed tomography (CT) revealed complete collapse of right lung with compensatory left lung hyperinflation and absent right kidney. Karyotyping of the patient was normal, 46XX. A diagnosis of MURCS syndrome with right ovarian hernia was made. The hernia was surgically managed with repositioning of ovary and fallopian tube into the pelvis. DISCUSSION:Ovary in inguinal hernia is rare in women of reproductive age group. MRKH syndrome, a mullerian duct anomaly, is the congenital aplasia of uterus and upper two-thirds of vagina in a female with normal ovaries, fallopian tube, secondary sexual characteristics and 46XX karyotype. MURCS is a subtype of MRKH type 2 having mullerian duct agenesis with renal, cardiac, muscular & vertebral defects. General physical examination and primary investigations if yields abnormal findings; the patient must undergo an array of investigations to rule out MRKH/MURCS, or other congenital abnormality. Early diagnosis is essential to prevent its incarceration or torsion. The primary treatment of ovary in inguinal hernia is repositioning the ovary and fallopian tube back to pelvis to preserve fertility and repair of inguinal hernia. A multidisciplinary team is required to deal with various abnormalities present in a patient with MURCS. 10.1186/s13048-021-00869-y

Complex Müllerian malformation without any present classification: unilateral ovarian and tubal absence with an arcuate uterus. Tanaka Yusuke,Koyama Shinsuke,Kobayashi Masaki,Kubota Satoshi,Nakamura Ryo,Isobe Masanori,Shiki Yasuhiko Asian journal of endoscopic surgery Müllerian duct anomalies are known to cause infertility and reproductive problems. The true incidence of such abnormalities is not well defined. The most widely accepted method of classification for a Müllerian duct anomaly is the American Society of Reproductive Medicine classification (1988). However, there are some rare anomalies inconsistent with the current classification. Herein, we report a rare case of Müllerian duct anomaly, unilateral ovarian and tubal absence with an arcuate uterus. The failure of the Müllerian ducts to canalize can also lead to the development of a unicornuate uterus and adnexal agenesis. An arcuate uterus indicates incomplete septal absorption after normal fusion of the Müllerian ducts. Therefore, its coexistence with adnexal absence and an arcuate uterus is considered to be extremely unlikely. 10.1111/j.1758-5910.2012.00159.x

Unilateral congenital ovarian and partial tubal absence: report of four cases with review of the literature. Uckuyu Ayla,Ozcimen Emel E,Sevinc Ciftci F Ceylan Fertility and sterility OBJECTIVE:To present four rare cases of congenital unilateral tubal and ovarian abnormalities with their laparoscopic images. DESIGN:Case report. SETTING:Baskent University Faculty of Medicine, Department of Obstetrics and Gynecology, Konya, Turkey. PATIENT(S):Three patients with infertility showing abnormality on hysterosalpingography and a fourth patient with acute pelvic pain who were admitted to our clinic. INTERVENTION(S):All four patients underwent laparoscopic surgery. MAIN OUTCOME MEASURE(S):Case 1 showed an absence of a distal tubal segment with ipsilateral ovarian agenesis. Case 2 showed a unilateral partial distal tubal absence with normal adjacent ovary. Case 3 showed a twisted and adherent tube with ipsilateral ovarian absence. Case 4 showed a unilateral ovarian agenesis and contralateral acute adnexal torsion. RESULT(S):We detected four cases of unilateral ovarian and partial tubal absence in these operations. CONCLUSION(S):We present four unusual cases of unilateral ovarian and partial tubal absence. The absence of one ovary and tube may be explained by two possible etiopathogenic causes: asymptomatic adnexial torsion or congenital malformation. 10.1016/j.fertnstert.2008.09.022

Ovarian absence: a systematic literature review and case series report. Journal of ovarian research Ovarian absence is an uncommon condition that most frequently presents unilaterally. Several etiologies for the condition have been proposed, including torsion, vascular accident, and embryological defect. A systematic review was conducted to describe the clinical presentation of ovarian absence, as well as its associations with other congenital anomalies, through a systematic search of Cochrane Library, ClinicalTrials.gov, Google Scholar, Ovid Embase, Ovid Medline, PubMed, Scopus, and Web of Science. Exclusion criteria included cases with suspicion for Differences of Sex Development, lack of surgically-confirmed ovarian absence, and karyotypes other than 46XX. Our search yielded 12,120 citations, of which 79 studies were included. 10 additional studies were found by citation chasing resulting in a total 113 cases including two unpublished cases presented in this review. Abdominal/pelvic pain (30%) and infertility/subfertility (19%) were the most frequent presentations. Ovarian abnormalities were not noted in 28% of cases with pre-operative ovarian imaging results. Approximately 17% of cases had concomitant uterine abnormalities, while 22% had renal abnormalities. Renal abnormalities were more likely in patients with uterine abnormalities (p < 0.005). Torsion or vascular etiology was the most frequently suspected etiology of ovarian absence (52%), followed by indeterminate (27%) and embryologic etiology (21%). Most cases of ovarian absence are likely attributable to torsion or vascular accidents, despite many references to the condition as "agenesis" in the literature. Imaging may fail to correctly diagnose ovarian absence, and diagnostic laparoscopy may be preferable in many cases as genitourinary anatomy and fertility considerations can be assessed during the procedure. Fertility is likely minimally or not affected in women with unilateral ovarian absence. 10.1186/s13048-022-01090-1