scholarly journals Laparoscopic Davydov Procedure for Creation of Neovagina in MRKH Syndrome

2021 ◽  
Vol 5 (9) ◽  
pp. 837-841
Author(s):  
Ratih Krisna ◽  
Rizky Agustria
Keyword(s):  
Intraoperative Complications ◽  
Abdominal Cavity ◽  
Operating Time ◽  
Vaginal Wall ◽  
Primary Amenorrhea ◽  
Mrkh Syndrome ◽  
Gynecological Disorders ◽  
Interrupted Suture ◽  
Sexual Characteristics ◽  
Vaginal Canal

Introduction: The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a condition in which there is an absence of the uterus and the upper part (2/3) of the vagina. Women with MRKH syndrome show normal development of secondary sexual characteristics and a normal 46, XX karyotype. MRKH syndrome is a common cause of primary amenorrhea. Ultrasonography examination confirms kidneys, presence of the ovaries, and absence of uterus. Treatment should include a multidisciplinary approach to create a functional vagina. One of the procedures is laparoscopic Davydov vaginoplasty. Aim: To report MRKH syndrome case treated with laparoscopic Davydov vaginoplasty. Method: Ms. A, 21 years old, P0A0, came to FER clinic Moh. Hoesin Hospital Palembang with complaint, never had menstruation, the patient admitted her breast, and her pubic had growth. She wants to get married in the next six months. From the US result, there was non visualized uterus, genital band (+), and both ovaries within normal limit ~ MRKH Syndrome. Karyotype Examination: 46, XX. Patient was diagnosed with primary amenorrhea caused by MRKH syndrome and was planned for neovagina (Davydov Procedure). Result: A woman 21-year-old, P0A0, was diagnosed with primary amenorrhea caused by MRKH syndrome. The Davydov procedure was done to create a neovagina. Intraoperative, after protrusion of the wood mold as a marker shown, an incision is made to the left and right side so that the mold penetrates the abdominal cavity, then the mold is pulled into the vaginal canal slowly. The anterior and posterior peritoneal layers are drawn into the vaginal canal. The anterior and posterior peritoneal layers were withdrawn with an ovum clamp and then interrupted suture. The anterior and posterior peritoneal layers were sutured in a circular method to form a vaginal cuff. The vaginal wall is sutured (interrupted) to hold mold fixed. Conclusion: MRKH syndrome is a disorder that occurs in females and mainly affects the reproductive system. This condition causes the vagina and the uterus to be underdeveloped or even absent at birth. Patients with diagnosed MRKH syndrome require interdisciplinary care. Davydov procedure is a laparoscopically-assisted technique, which has an advantage over traditional approaches. It has shorter operating time, lower intraoperative complications, shorter hospital stay, and no external scars. Postoperatively, sexual function is similar to women without gynecological disorders.

scholarly journals Laparoscopic Davydov Procedure for Creation of Neovagina in MRKH Syndrome

2021 ◽  
Vol 5 (3) ◽  
pp. 809-813
Author(s):  
Ratih Krisna ◽  
Rizky Agustria
Keyword(s):  
Intraoperative Complications ◽  
Abdominal Cavity ◽  
Operating Time ◽  
Vaginal Wall ◽  
Primary Amenorrhea ◽  
Mrkh Syndrome ◽  
Gynecological Disorders ◽  
Interrupted Suture ◽  
Sexual Characteristics ◽  
Vaginal Canal

Introduction: The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a condition in which there is an absence of the uterus and the upper part (2/3) of the vagina. Women with MRKH syndrome show normal development of secondary sexual characteristics and a normal 46, XX karyotype. MRKH syndrome is a common cause of primary amenorrhea. Ultrasonography examination confirms kidneys, presence of the ovaries, and absence of uterus. Treatment should include a multidisciplinary approach to create a functional vagina. One of the procedures is laparoscopic Davydov vaginoplasty. Aim: To report MRKH syndrome case treated with laparoscopic Davydov vaginoplasty. Method: Ms. A, 21 years old, P0A0, came to FER clinic Moh. Hoesin Hospital Palembang with complaint, never had menstruation, the patient admitted her breast, and her pubic had growth. She wants to get married in the next six months. From the US result, there was non visualized uterus, genital band (+), and both ovaries within normal limit ~ MRKH Syndrome. Karyotype Examination: 46, XX. Patient was diagnosed with primary amenorrhea caused by MRKH syndrome and was planned for neovagina (Davydov Procedure). Result: A woman 21-year-old, P0A0, was diagnosed with primary amenorrhea caused by MRKH syndrome. The Davydov procedure was done to create a neovagina. Intraoperative, after protrusion of the wood mold as a marker shown, an incision is made to the left and right side so that the mold penetrates the abdominal cavity, then the mold is pulled into the vaginal canal slowly. The anterior and posterior peritoneal layers are drawn into the vaginal canal. The anterior and posterior peritoneal layers were withdrawn with an ovum clamp and then interrupted suture. The anterior and posterior peritoneal layers were sutured in a circular method to form a vaginal cuff. The vaginal wall is sutured (interrupted) to hold mold fixed. Conclusion: MRKH syndrome is a disorder that occurs in females and mainly affects the reproductive system. This condition causes the vagina and the uterus to be underdeveloped or even absent at birth. Patients with diagnosed MRKH syndrome require interdisciplinary care. Davydov procedure is a laparoscopically-assisted technique, which has an advantage over traditional approaches. It has shorter operating time, lower intraoperative complications, shorter hospital stay, and no external scars. Postoperatively, sexual function is similar to women without gynecological disorders.

scholarly journals MRKH syndrome: a review of literature

2018 ◽  
Vol 7 (12) ◽  
pp. 5219
Author(s):  
Nidhi Jain ◽  
Jyotsna Harlalka Kamra
Keyword(s):  
Ovarian Function ◽  
Surgical Techniques ◽  
Primary Amenorrhea ◽  
Mrkh Syndrome ◽  
Secondary Sexual Characteristics ◽  
Secondary Sexual Characters ◽  
Vaginal Aplasia ◽  
Rare Congenital Disorder ◽  
Sexual Characteristics ◽  
Secondary Sexual

Primary amenorrhea is defined as failure to achieve menarche till age of 14 years in absence of normal secondary sexual characters or till 16 years irrespective of secondary sexual characters. The most common cause of primary amenorrhea is gonadal pathology followed by Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome). MRKH syndrome is a rare congenital disorder characterised by uterine and vaginal aplasia. It occurs due to failure of development of Müllerian duct. Its incidence is 1 per 4500 female births. Mostly girls present with primary amenorrhea. It is characterised by presence of normal secondary sexual characteristics, normal 46 XX genotype, normal ovarian function in most of the cases and absent or underdeveloped uterus and upper part (2/3) of vagina. It is of two types: type A is isolated type while type B is associated with other renal/skeletal/cardiac anomalies. Treatment includes psychological counselling and vaginoplasty. Vaginoplasty can be done by various non-surgical and surgical techniques. The authors hereby review the literature of MRKH syndrome regarding its embryology, etiopathogenesis, approach to work up and management.

scholarly journals MAYER-ROKITANSKY-KUSTER-HAUSER (MRKH) SYNDROME: A CASE REPORT

2020 ◽  
pp. 44-45
Author(s):  
Vamanagunta. Pranavi ◽  
Kurapati. Sai Anusha
Keyword(s):  
Abnormal Development ◽  
Primary Amenorrhea ◽  
Mullerian Duct ◽  
Fallopian Tubes ◽  
Mrkh Syndrome ◽  
Müllerian Duct ◽  
Sexual Characteristics ◽  
Endocrine Status ◽  
Congenital Aplasia ◽  
Uncommon Condition

Mayer-Rokitansky-Kuster-Hauser syndrome is an uncommon condition, with an incidence of 1 in 4000- 5000 female births and is the second most frequent cause of primary amenorrhea after gonadal dysgenesis .The reproductive abnormalities of MRKH syndrome are due to incomplete development of the Müllerian duct which develops into the uterus, fallopian tubes, cervix, and the upper part of the vagina. The cause of the abnormal development of the Müllerian duct in affected individuals is unknown. This abnormality is characterized by congenital aplasia/hypoplasia of the uterus and the upper part (2/3) of vagina, in young women presenting otherwise with normal endocrine status. It may include absence or hypoplasia of the uterus and fallopian tubes. The patients present with normal secondary sexual characteristics, as the functional ovaries are present, but menstruation is absent .

scholarly journals Primary Amenorrhea with Mayer-Rokitansky-Kuster-Hauser Syndrome

2021 ◽  
Vol 9 (T3) ◽  
pp. 175-177
Author(s):  
Muhammad Rusda ◽  
Delfi Lutan ◽  
Mervina Andarini ◽  
Andrina Yunita M. Rambe
Keyword(s):  
Pubic Hair ◽  
Vaginal Wall ◽  
Breast Development ◽  
Transvaginal Sonography ◽  
Enzyme Linked Immunosorbent Assay ◽  
Primary Amenorrhea ◽  
Mrkh Syndrome ◽  
Examination Result ◽  
Mullerian Ducts ◽  
Axillary Hair

BACKGROUND: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare disorder described as aplasia or hypoplasia of uterus and vagina due to an early arrest in the development of Mullerian ducts. The etiology of MRKH syndrome remains uncertain: Although at the beginning, it was mentioned that this syndrome was the result of sporadic abnormalities. It has recently been assumed the genetic background is the cause of the increasing number of familial cases. CASE REPORT: A 29-year-old female patient presented with amenorrhea. She also has normal pubic and axillary hair and breast development. Routine investigations were normal. Genitalia inspection shows that pubic hair was well developed, labia majora and vagina were seen. Inspeculo show that vaginal wall was smooth, but portio was not seen. Vaginal examination result: The uterus was unpalpable, both adnexa were normal, parametrium was laxed, and Douglas pouch was not protruded. Rectal examination shows that uterus and both adnexa were difficult to identify. Transvaginal sonography revealed absence of uterus, both ovaries within normal. Transvaginal sonography diagnosed a uterine aplasia. With this, the provisional diagnosis made as primary amenorrhea due to suspected MRKH syndrome, the patient was referred for follicle-stimulating hormone, estradiol, testosterone, and prolactin levels by enzyme-linked immunosorbent assay examination and the result was within normal limit followed by genetic karyotyping and the result was normal 46, XX. CONCLUSIONS: We report a 29-year-old woman with primary amenorrhea. From our examination, the patient was diagnosed with MRKH syndrome. Uterus transplant is an alternative for that woman to treat amenorrhea and possibility to get pregnant.

Primary Amenorrhoea with Pituitary Dwarfism: A rare case report

2016 ◽  
Vol 2 (2) ◽  
pp. 145-147
Author(s):  
Siva S ◽  
Divya Gopineni ◽  
Shafi P ◽  
Chandra Sekhar
Keyword(s):  
Pituitary Gland ◽  
General Medicine ◽  
Pituitary Hormones ◽  
Thyroid Stimulating Hormone ◽  
Primary Amenorrhea ◽  
Pituitary Dwarfism ◽  
Primary Amenorrhoea ◽  
Sexual Characteristics ◽  
Loss Of Appetite ◽  
Secondary Sexual

Females with pituitary dwarfism and a multiple deficiency of pituitary hormones show ovarian dysfunction due to hypogonadotropism. Primary amenorrhea can be diagnosed if a patient has normal secondary sexual characteristics but no menarche by 16 years of age. A 16 year-old female patient admitted in general medicine department with chief complaints of shortness of breath on exertion since 15 days, swelling of both legs since 10 days, loss of weight since 5 months, loss of appetite since 3 months, history of pain during swallowing. Pelvis scan examination reveals that uterus measures 3.2×0.5×0.5cm; uterus is hypo plastic, ovaries not visualized. Patient parents reveled that from patient birth to 11years of age her growth and other developments were normal, after that her growth is stopped and no changes were observed in development since 5 years. Patient has hypothyroidism so pituitary gland make an important role to maintain hormone levels, pituitary gland produces thyroid stimulating hormone (TSH) which stimulates thyroid gland to produce thyroid hormones. Primary Amenorrhea, short stature and poorly developed secondary sexual characters which could have been contributed and should be subjected for karyotyping. This type of Pituitary Dwarfism is very difficult to manage.

scholarly journals Multidisciplinary team management of 46,XY 17α-hydroxylase deficiency: a case report and literature review

2021 ◽  
Vol 49 (3) ◽  
pp. 030006052199396
Author(s):  
Yang Zhou ◽  
Xue Xue ◽  
Panpan Shi ◽  
Qinrui Lu ◽  
Shulan Lv
Keyword(s):  
Multidisciplinary Team ◽  
Relevant Literature ◽  
Management Plan ◽  
Laboratory Evaluation ◽  
Primary Amenorrhea ◽  
Physical And Mental Health ◽  
Hydroxylase Deficiency ◽  
Holistic Management ◽  
Sexual Characteristics ◽  
Set Up

Background We report here a case study of 17α-hydroxylase deficiency in a phenotypic girl with male karyotype (46,XY). We also review the relevant literature to deepen our understanding of the disease, reduce the rate of missed diagnosis, and emphasize that holistic management of this disease requires collaborative multidisciplinary teamwork. Case presentation A 14-year-old patient with a female phenotype visited the endocrinology department because of hypertension. The patient had primary amenorrhea and lacked secondary sexual characteristics. Initial laboratory evaluation revealed normal levels of electrolytes, a hypergonadotropic hypogonadal state with high progesterone and low testosterone levels, and a 46,XY karyotype. She was referred to the urology department for gonadectomy and transferred to the gynecological endocrine clinic. On the basis of the patient’s medical history and genetic testing results, a diagnosis of 46,XY 17α-hydroxylase deficiency was made. The patient was provided with glucocorticoids, estrogens, metformin, and psychological support. Conclusions Patients with 17α-hydroxylase deficiency, a rare cause of congenital adrenal hyperplasia, should be treated by a multidisciplinary team. Relevant experts from different disciplines should set up a systematic and comprehensive individualized management plan to optimize the physical and mental health and quality of life of affected patients.

scholarly journals Minilaparotomy a Good Option in Specific Cases: A Case Report of Bilateral Ovarian Germ Cell Tumor

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
D. Bolla ◽  
N. Deseö ◽  
A. Sturm ◽  
A. Schöning ◽  
C. Leimgruber
Keyword(s):  
Acute Abdominal Pain ◽  
Adnexal Mass ◽  
Past History ◽  
Ovarian Tissue ◽  
Abdominal Cavity ◽  
Operating Time ◽  
Expectant Management ◽  
Small Incision ◽  
Risk Of Rupture ◽  
The Right

Mature cystic teratomas (MCTs) of the ovary represent 44% of ovarian neoplasmas. The surgical approach is important in young women especially for the cosmetic results. Nowadays most of the ovarian surgeries can be performed laparoscopically. An alternative between laparoscopy and laparotomy is the minilaparotomy (ML) which can be an interesting option, thanks to the small incision. We report a 39-year-old woman who was referred to our hospital with acute abdominal pain. In her past history the patient had an uncomplicated delivery. During pregnancy a 6 cm bilateral MCT was diagnosed and expectant management was followed. A left-sided ovarial torsion was postulated, and laparoscopic detorsion was performed. To avoid a rupture of the left MCT, the operation was interrupted. To remove the cyst, a ML was done two weeks later. A left-sided salpingo-oophorectomy was performed due to a large cyst including the entire ovary. On the other side, the right dermoid cyst was entirely removed. The advantage of a ML is not only shorter operating time with less learning curve compared to laparoscopy but also the possibility to extract the adnexal mass from the abdominal cavity with lower risk of rupture and in addition the possibility to preserve more ovarian tissue.

scholarly journals Microendoscope-Assisted Versus Open Posterior Lumbar Interbody Fusion for Lumbar Degenerative Disease: A Multicenter Retrospective Cohort Study

Medicina ◽  
2021 ◽  
Vol 57 (2) ◽  
pp. 150
Author(s):  
Masayoshi Fukushima ◽  
Nozomu Ohtomo ◽  
Michita Noma ◽  
Yudai Kumanomido ◽  
Hiroyuki Nakarai ◽  
...  
Keyword(s):  
Interbody Fusion ◽  
Intraoperative Complications ◽  
Operating Time ◽  
Posterior Lumbar Interbody Fusion ◽  
Fusion Rate ◽  
Open Group ◽  
Lumbar Interbody Fusion ◽  
Lumbar Degenerative Disease ◽  
Radiographic Findings ◽  
One Year

Background and objectives: Minimally invasive surgery has become popular for posterior lumbar interbody fusion (PLIF). Microendoscope-assisted PLIF (ME-PLIF) utilizes a microendoscope within a tubular retractor for PLIF procedures; however, there are no published reports that compare Microendoscope-assisted to open PLIF. Here we compare the surgical and clinical outcomes of ME-PLIF with those of open PLIF. Materials and Methods: A total of 155 consecutive patients who underwent single-level PLIF were registered prospectively. Of the 149 patients with a complete set of preoperative data, 72 patients underwent ME-PLIF (ME-group), and 77 underwent open PLIF (open-group). Clinical and radiographic findings collected one year after surgery were compared. Results: Of the 149 patients, 57 patients in ME-group and 58 patients in the open-group were available. The ME-PLIF procedure required a significantly shorter operating time and involved less intraoperative blood loss. Three patients in both groups reported dural tears as intraoperative complications. Three patients in ME-group experienced postoperative complications, compared to two patients in the open-group. The fusion rate in ME-group at one year was lower than that in the open group (p = 0.06). The proportion of patients who were satisfied was significantly higher in the ME-group (p = 0.02). Conclusions: ME-PLIF was associated with equivalent post-surgical outcomes and significantly higher rates of patient satisfaction than the traditional open PLIF procedure. However, the fusion rate after ME-PLIF tended to be lower than that after the traditional open method.

PEDIATRIC GYNECOLOGY AND ENDOCRINOLOGY

PEDIATRICS ◽  
1956 ◽  
Vol 18 (2) ◽  
pp. 318-322
Author(s):  
Robert B. Greenblatt ◽  
James W. Bennett
Keyword(s):  
Foreign Bodies ◽  
Bowel Movement ◽  
Vaginal Wall ◽  
Foreign Object ◽  
Small Children ◽  
Pathologic Process ◽  
Underlying Factor ◽  
Rapid Pace ◽  
Vaginal Canal ◽  
Local Use

MANY mothers bring their young daughters to the pediatrician because they have noticed a discharge on the child's underclothing. Most of such discharges are nothing more than desquamation of the epithelial cells at perhaps a more rapid pace than usual. Such a discharge, commonly referred to as "whites," occurs normally in the adult female. No pathologic process is involved in either the child or the woman and no treatment is indicated. On the other hand, a discharge which is irritating, malodorous, and provokes an inflammatory response, has a pathologic background. By far the greater number of such cases fall into one of the following categories: (a) Nonspecific vaginitis which may be due to the presence of organisms which find their way into the vaginal canal either through insertion of the child's finger or some foreign object, or may appear for no apparent predisposing reasons. Among the organisms found are pneumococcus, streptococcus, staphylococcus, diphtheroids, colon bacillus, etc. The treatment consists primarily of removing or correcting the underlying factor and cleansing measures. Frequently, attention to the technique of cleansing the rectal area following a bowel movement, daily bathing and a pitcher douche with saline twice a day will clear up the discharge. Sulfonamides by mouth, locally, or both may be effective in some cases. In other cases, the local use of a specific antibiotic such as Terramycin® or Aureomycin® has been effective (a 50 mg. capsule may be inserted into the vaginal canal daily for 5 to 7 days). (b) Another group of cases is due to the presence of foreign bodies in the vaginal canal. Any number and type of foreign bodies have been found in the vaginal canals of small children, such as safety pins, pencils, sticks, etc. The presence of a foreign body sets up an irritant reaction and causes a discharge, in most instances with a bloody component. The diagnosis may be made by inspection or by gentle rectal examination, pressing the vaginal wall through the rectum.

Detection of oncological diseases in patients with pelvic organ prolapse. Case report

GYNECOLOGY ◽  
2021 ◽  
Vol 23 (2) ◽  
pp. 193-197
Author(s):  
Dmitrii V. Baibuz ◽  
Oleg L. Molchanov ◽  
Zhanna V. Glushchenko ◽  
Yanina A. Lebedeva ◽  
Sergei V. Utkin ◽  
...  
Keyword(s):  
Pelvic Organ Prolapse ◽  
Preoperative Diagnosis ◽  
Abdominal Cavity ◽  
Pelvic Organ ◽  
Vaginal Wall ◽  
Benign Disease ◽  
Pelvic Tumor ◽  
Planning Treatment ◽  
Oncological Diseases ◽  
Anterior Posterior

Pelvic organ prolapse is a common benign disease among women that is characterized by an isolated or combined descent of the anterior, posterior vaginal wall, uterus, and vaginal dome after hysterectomy. Given the fact that most of the operations are performed among middle-aged and elderly patients, the probability of detecting previously undiagnosed tumor of the abdominal cavity and pelvic region increases, which may be due to the limited volume of basic preoperative diagnosis. The purpose of our report is to draw the attention of clinicians to the problem of insufficient examination of women when planning treatment of pelvic organ prolapse on the example of our experience in managing a patient with this pathology in combination with pelvic tumor.

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