OHVIRA syndrome with Orofacial clefts: a rare association

Obstucted hemivagina with ipsilateral renal anomaly (OHVIRA) syndrome or Herlyn-Werner-Wunderlich syndrome (HWW) is a rare congenital anomaly consist of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We are reporting an adolescent girl with orofacial defect who presented with lower abdominal pain. She attained menarche 3 months earlier and had a regular menstrual cycle with cyclical abdominal pain. On abdominal examination a firm, mobile tender mass extending from left iliac fossa up to umbilicus (24 weeks size) was found. Lower border of mass could not be approached. Further evaluation with ultrasound showed enlarged uterus with collection with internal echoes and non-visualization of the left kidney. CECT showed absent left kidney and didelphys uterus with large left hematocolpometra with left complex adenexal cyst. Patient was posted for hematocolpos drainage and vaginoplasty. An unusual presentation of regular menstruation and nonspecific abdominal pain delays the diagnosis, which can lead to severe complications such as endometriosis and infertility.

Download Full-text

Uterine Didelphys Associated With Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome: a Case Report

Pulse ◽

10.3329/pulse.v6i1-2.20358 ◽

2014 ◽

Vol 6 (1-2) ◽

pp. 66-69

Author(s):

A Parvin ◽

BS Khan ◽

J Alam ◽

FA Ruby ◽

TJ Iqbal

Keyword(s):

Left Kidney ◽

Lower Abdominal Pain ◽

Radiology Department ◽

Cervical Canal ◽

Posterior Aspect ◽

Pelvic Brim ◽

Renal Anomaly ◽

Unicornuate Uterus ◽

Ohvira Syndrome ◽

Obstructed Hemivagina

A 30 year old nulligravida female reported to the fertility centre of AHD with the complaints of primary infertility for three and half years and spasmodic dysmenorrhoea. There is also history of progressively increasing right lower abdominal pain as well as discomfort which was cyclically associated with the onset of menses. Transabdominal sonography showed-Endometrial splitting into two at the fundussuggesting bicornuate uterus. Echogenic soft tissue in the cervical canal due to blood clots. Non visualized right kidney. Mildly enlarged left kidney. HSG done outside AHD suggestive of unicornuate uterus with single fallopian tube. IVU showed non visualized right kidney. Normally excreting left kidney. TVS showed normal sized septated nulliparus uterus with homogeneous myometrium and thick endometrium with proliferative phase echo. Mildly enlarged right ovary with mildly distended right tube. Mild collection adjacent to the vagina. Then the patient came to the gynaecology dept of AHD from where she was sent to our Radiology department to undergo MRI of pelvis. The MRI showed uterine didelphys. Obstructed hemivagina (right) with hematocolpos extended upto pelvic brim along right and posterior aspect of uterus through anomalous dilated remnant of right lower ureter with ipsilateral renal agenesis. Patient was diagnosed as OHVIRA syndrome radiologically. DOI: http://dx.doi.org/10.3329/pulse.v6i1-2.20358 Pulse Vol.6 January-December 2013 p.66-69

Download Full-text

Multiple Variants of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome – One Clinical Center Case Series and the Systematic Review of 734 Cases

Journal of Pediatric Urology ◽

10.1016/j.jpurol.2021.06.023 ◽

2021 ◽

Author(s):

Grzegorz Kudela ◽

Agnieszka Wiernik ◽

Agnieszka Drosdzol-Cop ◽

Magdalena Machnikowska-Sokołowska ◽

Aneta Gawlik ◽

...

Keyword(s):

Systematic Review ◽

Case Series ◽

Renal Anomaly ◽

Clinical Center ◽

Ohvira Syndrome ◽

Obstructed Hemivagina ◽

Multiple Variants

Download Full-text

Imperforate hymen mimicking acute appendicitis in an adolescent woman: a rare presentation

BMJ Case Reports ◽

10.1136/bcr-2020-238547 ◽

2021 ◽

Vol 14 (3) ◽

pp. e238547

Author(s):

Victoria Rose Russell ◽

Mohamed Ibrahim ◽

Georgina Phillips ◽

Tom Setchell ◽

Sanjay Purkayastha

Keyword(s):

Abdominal Pain ◽

Acute Appendicitis ◽

Iliac Fossa ◽

Female Genital Tract ◽

Delayed Diagnosis ◽

Rare Presentation ◽

Left Iliac Fossa ◽

Imperforate Hymen ◽

Retrograde Menstruation ◽

Low Incidence

Imperforate hymen is a rare congenital malformation of the female genital tract. The condition poses several diagnostic challenges owing to its low incidence and often atypical presentation. Classical symptoms include amenorrhoea and cyclical abdominal pain. Delayed diagnosis leads to potentially irreversible and lifechanging sequelae including infertility, endometriosis and renal failure. A premenarchal 13-year-old girl with a background of chronic constipation presented with symptoms mimicking acute appendicitis. The underlying cause was imperforate hymen and retrograde menstruation. The diagnosis was made during diagnostic laparoscopy. As with this patient, pre-existing symptoms are often troublesome long before the true diagnosis is made. This case report highlights the importance of recognising imperforate hymen as a potential cause of acute abdominal pain in premenarchal adolescent girls. The clinical picture may present as right or left iliac fossa pain. Early identification reduces the risk of adverse complications and avoids unnecessary and potentially harmful interventions.

Download Full-text

Radiological Manifestations of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Rare Complex Müllerian and Wolffian Duct Anomaly

International Journal of Recent Surgical and Medical Sciences ◽

10.5005/jp-journals-10053-0009 ◽

2016 ◽

Vol 02 (01) ◽

pp. 033-039

Author(s):

Tushar Patil ◽

Devarati Khurjekar ◽

Vikash Ojha

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Pelvic Pain ◽

Wolffian Duct ◽

Resonance Imaging ◽

Renal Anomalies ◽

Renal Anomaly ◽

Ohvira Syndrome ◽

Obstructed Hemivagina ◽

Magnetic Resonance Imaging Mri

AbstractObstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare complex Müllerian and Wolffian duct anomaly. It is also known as Herlyn–Werner–Wunderlich syndrome (HWWS). It includes unilateral renal anomalies and uterine didelphys. It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain shortly following menarche, causing a delay in the diagnosis. We report here a 16-year-old female presenting with progressive cyclical pelvic pain, where magnetic resonance imaging (MRI) suggested the diagnosis of the OHVIRA syndrome. She was managed by surgical resection of the septum and draining of the obstructed vagina.

Download Full-text

Uterine Didelphys with OHVIRA Syndrome (Obstructed Hemivagina and Ipsilateral Renal Anomaly)

Journal of Minimally Invasive Gynecology ◽

10.1016/j.jmig.2018.09.220 ◽

2018 ◽

Vol 25 (7) ◽

pp. S123

Author(s):

R.B. Smith ◽

K. Steck-Bayat ◽

L. Stoycheff ◽

J. Mourad

Keyword(s):

Renal Anomaly ◽

Ohvira Syndrome ◽

Obstructed Hemivagina

Download Full-text

Torsion of a parasitic leiomyoma: a rare but important differential in women presenting with lower abdominal pain

BMJ Case Reports ◽

10.1136/bcr-2019-232797 ◽

2021 ◽

Vol 14 (1) ◽

pp. e232797

Author(s):

Clemmie Stebbings ◽

Ahmed Latif ◽

Janakan Gnananandan

Keyword(s):

Abdominal Pain ◽

Iliac Fossa ◽

Lower Abdominal Pain ◽

Sudden Onset ◽

Greater Omentum ◽

Caribbean Woman ◽

History Of ◽

Right Iliac Fossa Pain ◽

The Right ◽

Fat Stranding

A 39-year-old multiparous Afro-Caribbean woman attended the emergency department with sudden-onset severe right iliac fossa pain. Her inflammatory markers were mildly elevated. Computerised tomography of the abdomen demonstrated features of fat stranding in the right iliac fossa suspicious of acute appendicitis. The scan also noted uterine leiomyomas. The patient was taken to theatre for an emergency diagnostic laparoscopy where her appendix was found to be macroscopically normal. A necrotic heavily calcified parasitic leiomyoma was seen in the right adnexa, free of the uterus and adherent to the greater omentum on a long torted pedicle. The parasitic leiomyoma was successfully removed piecemeal laparoscopically. Complications of leiomyomas, namely, torsion and necrosis, are important differentials in women presenting with sudden-onset lower abdominal pain. A history of sudden-onset severe lower abdominal pain with a background of known leiomyoma should prompt the clerking surgeon to consider a complication of leiomyoma as part of the differential diagnoses.

Download Full-text

SP3.2.1 Consultant-led Urgent Ambulatory Management of Righted-sided Lower Abdominal Pain: Prospective data collection

British Journal of Surgery ◽

10.1093/bjs/znab361.068 ◽

2021 ◽

Vol 108 (Supplement_7) ◽

Author(s):

Mubashar Hussain

Keyword(s):

Abdominal Pain ◽

Iliac Fossa ◽

Lower Abdominal Pain ◽

Clinical History ◽

P Value ◽

Prospective Data ◽

Female Ratio ◽

Blood Tests ◽

Complications Rate ◽

Male To Female

Abstract Aims To study if right lower abdominal pain could be managed safely on ambulatory basis when referred from emergency department or primary healthcare as suspected acute appendicitis to surgical ambulatory unit (SAU). Methods Data collated prospectively involving 586 patients, who presented with right iliac fossa/right-sided pelvic pain from january 2019 to January 2021. Results 0ut of 586 patients, 234 (40%) were admitted and 352 (60%) managed in ambulatory settings after being reviewed by Consulant-lead surgical oncall team. Age ranged from 3 to 92 years. Male to female ratio was 3:2. Their management included clinical history/and examination, urinalysis and blood tests. 157 (44%) patients had scans, 43 (12%) had CT scans and 114 (32%) had pelvic USS the same day or the following morning/day and were reviewed by oncall-surgeons and 107 (30%) patients had repeat blood tests. 39 (11%) out of 352 paitents were admitted the following day review, of which 18 (5%) patients underwent laproscopic appendicectomy (LA) and 8 (2.2%) patients treated conservatively. 9 (1.98%) patients represented with persistent/or worsening symptoms and 5 (1.4%) patients of these had LA and 2 (0.56%) patients had pelvic collection, drained radiologically and 2 (0.56%) had wound infections treated with antibiotics. Complications rate in ambulatory patients compared to in-patients was insignificant, P-value set at 0.05% using Mann-Whitney U test. Conclusions

Download Full-text