Essay 287 - Developmental gynaecology

a) I will ask about the nature of pain, intensity of the abdominal pain and if the pain is preceeded by breast tenderness, I will also ask about associated urinary and bowel symptoms such as dysuria, frequency and diarrhea which will point to a non-gynaecological problem such as inflammatory bowel disase or urinary tract infection. In such cases, referral to respective department wil be done. I will ask about medical history if she has medical illness causing recurrent abdominal pain such as ulcerative colitis. Severity of pain will be asked and how the pain has affected the quality of her life such as absenteeism from school. I will also enquire about relieving and aggravating factors of abdominal pain. The mother and female's siblings age of menarche will be a good guide to her age of menarche. Clinically, I will check her height, weight and body mass index. I will look for secondary sexual characteristics such as breast development, pubic and axillary hair growth. I will palpate for pelvic mass which could have caused the pain. Perineum will be inspected to look at pubic hair as well as patency of hymen. A bluish and bulging membrane is suggestive of imperforate hymen, whereas a pink and bulging membrane is indicative of tranverse vaginal septum. Ultrasound of pelvis and abdomen will be carried out to look for abdominal and pelvic mass which could have caused the pain. I will also look for hydrometra on scan which signify obstruction of menstrual blood flow.

b) Low vaginal atresia is due to the failure of hymenal membrane to perforate during fetal life leading to menstrual blood flow collecting behind the membrane. 

c) Surgery is the treatment of imperforate hymen. The girl will be given anaesthesia, placed in lithotomy position and a cruciate incision made on the bulging hymenal membrane. Old menstural blood will be drained out. An opening will be left open for future menstruation. There is a small risk of repeated procedure due to closure of the hymen. She will need to be given antibiotics during the procedure to prevent infection. Subsequent follow up will be required to assess her condition and regularity of her menses. Prognosis is usually good.

As the girl and mother are distressed about the problem,  sensitive approach is necessary and good rapport should be established with the young girl.

History is taken about the severity of the pain and its effect on her studies. She should be asked if she is suffering with the pain cyclically. History is taken about bowel symptoms such as diarrhoea/ constipation, pain relieved after daefacation and change in the frequency and form of stools as some gastrointestinal problems present cyclically.

History is taken if she has dysuria, frequency or urgency suggesting urinary tract infection.

Regarding delayed menstrualtion, enquiry is made about family history of delayed menarche.

Opportunity is taken to ask in privacy about eating disorders (anorexia / bulimia)  psychological stress at home or at school. History is taken regarding extreme weight loss or strenuous exercise  which may be associated with amenorrhoea.

She should be asked about any mass per abdomen and associated problems daefecation  and urination such as retention of urine and which indicates collection of menstrual blood behind unperforated hymen. ( haematocolpos) 

Previous history of medical illness like renal disease , cystic fibrosis may delay ovarian function and cause amenorrhoea. History of prior chemotherapy of radiotherapy  can result in amenorrhoea. 

Examination includes measurement of height and arm span. BMI is assessed. She should be examined whether secondary sexually characteristics have developed or not. If she has developed secondary sexual characters,  breast development , axillary hair and pubic hair should be assessed according to Tanner staging.  Abdominal examination is performed to exclude any palpable mass. Inspection of vulva should be  performed to detect any bulging membrane. Blue membrane with darkened blood transilluminating through the thin membrane suggesting imperforate hymen , while pink coloured thick membranes indicates transverse vaginal septum. 

B) Hymen is a thin membrane that occurs at the junction of the sinovaginal bulb and urogenital sinus and is usually perforated during fetal life. Failure of this perforation leads to the membrane remaining intact and as puberty begins. Menstrual blood collects behind the membrane and the vagina begins to distend. 

C) Ultrasound scanning or Magnetic resonance imaging is required to confirm haematocolpos. Imaging is required to exclude any renal tract abnormalities. This problem of imperforate hymen requires surgical management. The nature of the surgery should be explained the girl and her mother in simple terminology .  The girl should be assessed whether she is Fraser competent , otherwise should be treated according to her best interests. Surgical treatment involves a cruiciate incision to relieve the obstruction. The remaining quadrants of the hymen may be left in situ or may be excised. She should be explained that haematocolpos would completely drain within 3-5 days and usually with no sequelae .

This girl has primary amenorrhoea.The investigations done by GP points towards normal hypothalamo pituitory ovarian function.history of recurrent abdominal pain points to anatomical defect.this should be explained to the girl and mother and the girl sensitevely.A detailed history should be asked about the nature of pain, whether cyclical or not and  frequency . Other associated  bladder and bowel symptoms symptoms should be asked.History of time of onset and development of secondary sexual characters like growthspurt, breast development and pubic and axillary hair should be enquired.Family history of delayed puberty in siblings or mother enqiured.

Examination of height and BMI done. secondary sexual charecters notified.features of pcos like acne , hirsutism noted as pcos sometimes present with primary amenorrhoea with eugonodotrophic  eugonodism.however pain abdomen will not be there.abdominal examination for any mass, urinary distension noted.external genitalia should be inspected.vaginal opening should be checked whether formed or ending in blind vagina or any bulge.

trans abdominal ultrasound to see any masses like haematocolpos or haematometra which indicates obstruction, presence or absence of uterus.3 d ultrasound or MRI will acurately assess the anatomical defect.If uterus and cx absent karyotyping should be done to exclude rare CAIS.

B, This is an embryological defect where the memb where urogenital sinus meets with sinovaginal bulb fails to canalise.

C.Treatment of this needs surgery.A cruciate incision should be made to let out the blood. it is not necessary to remove redundant tissue. all blood will be drained within 2-3 days.Mother and daughter should be reassured that she will have normal fertility.Sometimes the defect will be little higher and membranes appears pink and thick. Then  should be done by an expert. There is a possibility of endometriosis . This should be counselled sensitevely

(a) Discuss your clinical assessment

primary amenorrhea with normal hormonal profile is suggestive of a genital tract outflow obstruction,however other causes as anorexia nervosa or psychological stress and constitutional amenorrhea should not be ignored.asking about character, site and frequency of abdominal pain recurrence sincea colicky,low pelvic pain that recurs monthly suggests cryptomenorrhea due to imperforate hymen,transverse vaginal septum or type 2 mullerian agenesis.asking about family history regarding age of menarche of the mother or other female siblings,a positive history of late menarche suggests constitutional amenorrhea. asking about prescence or abscence of other pubertal changes in terms of pubic and axillary hair and breast development,abscence of pubic and axillary hair might suggest androgen insensetivity syndrome that will require confirmation by karyotyping later on.asking about past history of auditory,cardiac or renal problems that are common association with mullerian agenesis.asking about eating disorders and psychological stress and if on any treatment for such disorders.examination should be done in a sensetive manner realising it is stressful for the girl and her parents,in the prescence of chaperon and in good light.general examination for weight,height and BMI and plotting these against growth charts as well as examination for prescence of secondary sexual characters and tanner staging of pubertal changes if present.abdominal examination for tender mass suggestive of haematometra.external pelvic examination for development of vulva and vagina.a bluish bulge is diagnostic of imperforate hymen,a pink pale bulge suggests transverse vaginal septum while a short blind vagina suggests cervical atresia or mullerian agenesis,a blind pouch will raise the possibility of androgen insensetivity syndrome.rectal examination can identify a haematocolpos.

(b) She is thought to have low vaginal atresia (imperforate hymen). Describe the embryology of this abnormality

imperforate hymen is the result of failure of fusion of the distal end of the vulvo-vaginal pouch responsible for the formation of the lower 1/4 of the vagina  with the proximal end of the fused mullerian ducts responsible for formation of the upper genital tract and the upper 3/4 of the vagina.

(c) Discuss the management of this abnormality 

i will explain the diagnosis to the girl and her parents that it is a thin septum of skin obstructing the menstrual flow out of the vagina and that it canbe managed surgically.surgery willbe performed as a day case procedure under regional or general anaesthesia. a cruciate incision will be made to the hymen ensuring it doesn't reheal and obstruct the flow once more.after the procedure a haematocolpos or haematometra will drain spontaneously within a few days with no other sequale.explain that neither the diagnosis nor the treatment will affect future fertility or sexual activity.

(A) Discuss your clinical management.

From history taking, I would like to know when the abdominal pain started and whether it is cyclical. This will point towards a diagnosis of imperforate hymen. I would like to ask for any accompanying bowel or urinary symptoms to exclude non gynaecological of abdominal pain. I would like to know the character and severity of pain, whether she requires any analgesia and any impact on her quality of life including absence from school or limitation on physical activity like sports and any resulting depression symptoms. I want to know if she has any breast development and when it started, as well as the age of axillary and pubic hair development. I would also ask if she is sexually active and any possibility of pregnancy. I would like to ask if her sense of smell is normal, as well as any visual symptoms or headache, or excessive hair growth. I would like to know if she has any family history of delayed puberty or abnormalities of female reproductive system as well as the mother or sisters' age of menarche as constituitional delay might be a possiblity.

I will take the height and weight and calculate the BMI in corresponce to her age. I will perform a general inspection to look for any phenotypical features of Turner's syndrome including webbed neck, short stature, wide carrying angle and widely spaced nipples. I will then inspect her breasts for presence of breast buds and also presence of axillary and pubic hair. I will examine the abdomen for any palpable massess indicating a haematometra. I will then perform a inspection of the genitalia to check for any imperforate hymen with resulting haematocolpus or transverse septum. I will not perform a vaginal examination if she is yet to be sexually active.

A transabodminal ultrasound can be done to confirm the presence or absence of the uterus, cervix and vagina. If the uterus is absent, I would order a karyotyping to exclude androgen insensitivity syndrome. I would order an MRI to confirm the diagnosis of mullerian agenesis in the absence of uterus. 

(B)

Imperforate hymen is the result of failure of canalisation of tissue after proliferation of sinovaginal bulbs. 

(C) Discuss the management of this abnormality.

Following the confirmation of diagnosis, I will explain the structural defect to the patient and reassure them that the patient is genetically a female with otherwise normal reproductive organs. Fertility is not affected. I will refer the patient to a adolescent gynaecologist for surgical correction which involves making a incision on the hymen under anaesthesia to drain the haematometra/colpus. I will advise the patient post-operatively that she will have bleeding for the next 3-5 days resulting from draining of the blood. She should have cyclical menstruation following the procedure. She will require estrogen cream to the incision area and follow upto  ensure that the hymen remains patent.

A 15 year old girl has been referred to the gynaecology clinic because of a 2-3 year history of recurrent abdominal pain. Her mother is also concerned that she has not started menstruating. Endocrine profile performed by her GP (FSH, LH, prolactin, androgens and thyroid function tests) is normal. (a) Discuss your clinical assessment [13 marks]. (b) She is thought to have low vaginal atresia (imperforate hymen). Describe the embryology of this abnormality [2 marks]. (c) Discuss the management of this abnormality [5 marks]

a) 

As her hormone profile is normal, possible differentials include outflow tract obstruction, constitutionally delayed puberty, hypothalamic or pituitary disorder and androgen insensitivity syndrome. I would ask about the nature of the pain, whether it is cyclical and if there is any association with breast tenderness. I would ask about urinary and bowel symptoms, especially difficulty voiding and defecating which may occur due to bladder or bowel obstruction from a uterine mass. I would also ask about loss of weight or appetite. I would ask about history of late menarche in the family, and also ask the patient in private about sexual activity and contraceptive and drug usage. 

I would do a physical examination looking at her heigh, weight and body mass index. I would examine for secondary sexual characteristics by looking at the Tanner stage of breast and pubic hair development. I would palpate the abdomen to determine the size and location of any pelvic mass as well as for tenderness, rebound and guarding. I would obtain consent for a pelvic examination, which may need to be done under anaesthesia. I would inspect for pubic hair development, ambiguous genitalia and imperforate hymen. An imperforate hymen would appear like a bluish bulge, as opposed to a tranverse vaginal septum which is would be a pink bulge. I would do a vaginal and speculum examination to look for vaginal agenesis, longitudinal vaginal septum or cervical abnormalities, as well as to palpate for presence and size of uterus.

I would obtain an ultrasound scan for the patient to look for the presence of a uterus and ovaries. If there is uterine abnormality, an MRI would help to better delineate the abnormality, and an IV urogram, audiogram, skeletal and limb X rays and echocardiogram would help to detect associated abnormalities. I would perform a karyotype if there is no uterus to rule out androgen insensitivity syndrome. 

b) The hymen is the membrane between the sinovaginal bulbs and urogenital sinus. If this is not perforated during fetal life, an imperforate hymen results. Menstrual blood accumulates behind this after puberty. 

c) I would explain the diagnosis to the patient and explain that it usually has no long term sequelae. Patients may be at higher risk of endometriosis due to retrograde menstruation from prolonged obstruction. Treatment is via a cruciate incision into the hymen to allow drainage of menstrual blood. The remnant hymenal tissue may be left in situ or excised. This procedure may be done as a day case procedure under regional or general anaesthesia. 

this  young lady  and her mother will  be anxious  because  of delayed menstruation and recurrent pain  a sensitive approach and good repport with girl  is  very important .hx  will  be taken  from the girl  and her wishes regarding presence of   mother during examination should be respected. she will  be  reassured about maintinence of confidenciality. i will ask about  nature of  pain  , as pain  is recurrent iwill ask about  frequency of pain (monthly pain  may  suggest lower genital  obstruction while  more frequent  may have other underlying cause)nature of pain ,aggrevating or relieving  factors,association with  urinary  or bowel symptoms (may be non gynacological  cause for pain   and incidendal  primary ammenorrhea) . to assess  quality  of life  i will ask regarding  frquency  of absence from school..whether pain is associated  with abdominal mass? if yes  is mass  permanent .rapidly  growing  or gradually growing.i will also enquire about hx of menarche in mother and sisters (some  may have constitutional  delay), developmental milestones , secondary sexual charectors  and age at which they developed ,Hx of  weight gain or loss , eating and exercise  habbits ,hair growth ,acne ,.Hx off hot  flushes. i will  sensitively  ask whether she is sexually  active or not. .past  hx of   chronic medical illness will be taken eg  cystic  fibrosis ,Hx of  chemotharapy,radiotherapy or pelvic surgery.Family Hx  of  diseases like  cystic fibrisis ,galactocemia .

On  examination i will  assess BMI  , development of secondary  sexual charectors according to tanners classification ,hirsuiism (if present  scorring), on abdominal examination  i will  assess for  mass , (if present  size, nature  ,possible origin,mobility )iwill ask for  perineal examination in  sensitive way  only  external examination will be carried out . bluish bulging membrane at ineroitus  may  suggest  imperforate hymen. 

iwill arrange for  ultrasound pelvis  to assess anatomy  presence  or  absence of uterus , hematocolpus .ovarries ,. if abnormal anatomy  is found it is important to assess also  urinary tract for  annotomical bnormalities.

B in this defect membrane between  urogenital sinus  and sinovaginal  bulb  fails to canalise during  intrauterine life . at puberty  menstrual blood is trapped above membrane to cause recurrent pain and hematocolpus 

C  I will  discuss treatment  with  mother and girl in a sensitive way  and reassure her that  it is an anotomical defect  which  require surgery and after surgery  she will have normal sexual and  reproductive life .she will be  reassured about confidenciality. i will arrange  for  her surgery and give her  written infiomation regarding defect and nature of sugery .

surgery for defect is  cruciate inscision  in hymen and drainage of  collected blood. iwill councel her regarding bleeding a few days after surgery.it may be carried out under regional or gen. anesthesia.

(a) The nature of the recurrent abdominal pain should be explored as cyclical pain points towards the diagnosis of imperforated hymen. The sequence of puberty should be asked, as the correct sequence (thelarche then pubarche then growth spurt) demonstrates that the hypothalamo-pituitary axis is normal. Family history, particularly the age of menarche of both of her mother and sisters should be asked. If they also attained their menarche at a later age, then it suggest possible familial cause. History of excessive stress, significant loss of weight, eating disorder and strenous exercise should also be elicited as these can also delay menarche. In addition to that, problem with acne and hirsutism should be asked as these suggest possible PCOS. Her drug history should be explored as certain contraception for instance, depot provera can lead to amenorrhoea. It is also crucial to include in the history whether this girl is sexually active. If she is sexually active, there is possibility of pregnancy and important to ask whether she has any difficulty during penetration. In conditions like imperforated hymen and Mayer Rokitansky Kauser Hauser syndrome (MRKH), penetration is not possible. Physical examination should include the measurement of weight and height. Girls with Turner syndrome are typically shorter with the height of around 150cm. Body mass index (BMI) should be calculated as underweight and overweight are shown to cause delayed menses. Tanner staging should be used to assess the pubertal stage of breasts and pubic hair. The girl should be examined for hirsutism and acne that can be present in woman with polycystic ovarian syndrome (PCOS). Features of Turner syndrome, for instance, webbed neck, cubitus vulgus, underdeveloped and widely spaced nipple should also be included in the examination. Abdominal examination should be carried out to look for enlarged uterus suggesting pregnancy. Perineal examination should be carried out to look for the presence of bluish, bulging mass at the introitus that points towards imperforated hymen. If the mass is pinkish in colour, it is suggestive of tarnsverse vaginal septum. In the presence of short and blind-ended vagina, it makes the diagnosis of MRKH likely. A tall girl with well developed breast but in the absence of pubic hair makes the diagnosis of androgen insensitivity syndrome (AIS) very likely. Clinical assessment should include transbadominal scan and preferably transvaginal scan to look for the presence of uterus (in MRKH, uterus is absent) and haemotocolpos, haematometra and haematosalphinges that is present in imperforated hymen. Ovaries should also be scanned to look for polycystic ovaries that is present in PCOS which is diagnosed by fulfilling 2 out of the 3 Rotterdam's criteria. If mullerian agenesis is suspected, kidneys should be scanned and if abnormality is suspected magnetic resonance imaging (MRI) should be performed. Chromosomal studies can be performed in condition whereby Turner syndrome (45 XO) or AIS (46 XY) is supected. 

(b) Imperforated hymen is caused by failure of canalization of the caudal end of vaginal plate

(c) The girl and her mother should be explained about the diagnosis with written information provided. She should be scheduled for operation as early as possible. While awating for operation, adequate analgesia should be prescribed, for instance with non-steroidal anti-inflammatory drugs. It is also imprtant to ensure that she can pass urine without difficulty as large haematocolpos can lead to urinary retention. If this is present, bladder catheterization needs to be performed. She should be brought to theater with procedure done under regional anaesthesia. Cruciate incision should be made over the hymenal membrane over 2, 4, 8 and 10 position and each quadrants is excised along the lateral wall of vagina. The margin of the vagina mucosa is then approximate with fine absorbable sutures. Haemotocolpos is then drained. Following the procedure she should be followed up in specialist clinic. Kidneys should be scanned to ensure they are normal and if abnormality suspected, magnetic resonance imaging should be performed

a)I would ask a detailed history about the pain such as onset, duration ,nature ,radiation ,any exacerbating, relieving factors, associated urinary and bowel symptoms.Symptoms such as dysuria and increased  frequency of micturition suggests  urinary tract infection,while diarrhoea.constipation,change in bowel habits suggests gastrointestinal causes.If nongynaecological causes are identified,referral to respective departments should be done.  History of  cyclical abdominal pain , urinary retention and primary amenorrhaea  suggests imperforate hymen .History of  development of secondary sexual characters such as breast development, pubic hair and axillary hair should be asked.Family history of  constituitional delay in puberty,any history of anosmia,eating disorders such as anorexia nervosa and excessive exercise should be asked which suggest hypothalamic causes .Presence of anosmia is suggestive of Kallman’s syndrome.History of  visual disturbances, head ache and galactorrhoea suggest pituitary adenomas. History of excessive facial hair,acne ,acanthosis nigricans and deepening of voice suggests hyperandrogenism due to androgen secreting tumours..History of any cardiac abnormalities such as coarctation of  aorta suggests Turner’s syndrome.Any history of childhood cancer which needed chemotherapy/radiotherapy should be asked to rule out premature ovarian failure. History of chronic systemic illness such as cystic fibrosis,diabetes and renal failure needs to be elicited as it can also cause primary amenorrhoea.

Imperforate hymen

A   The absence of menstruation upto age of 14 years without secondary sexual characteristics or upto age of 16 years in the presence of secondary sexual characteristic is called primary amenorrhea, which needs investigation. This 15 years old girl will be asked about nature of pain, its severity, how long does it persist, and is it cyclical comes after regular intervals. Is the pain associated with vaginal discharge and associated symptoms like headache, irritability, tension,  abdominal bloating. The mothers concerns should be discussed sensitively and is reassured that most of cases amenorrhea is constitutional but other causes should be excluded. The girl should be addressed directly about her problem. The age of menarche of siblings and of mother is asked which will give some idea about her menarche. Her lifestyle is discussed if she is an athlete can result in delayed menarchae. Her eating habbits discussed if she has anorexia nervosa. She is asked about chronic illness like cystic fibrosis, hypothyroidism. Any history of head injury or surgery in childhood is noted. She is asked about radiotherapy or chemotherapy in the past. She is asked about abnormal hair growth on chin, chest, or back for hirsuitism. The girl is examined in the presence of chaperone for development of secondary sexual characterstics like breast development, pubic hair, external genitalia, and axillary hair growth. Hisuitism  is noted. Signs of virilisation like hoarseness of voice and clitoral enlargement is noted. Signs of hypothyroidism like cold intolerance is noted.

B  Mullarian ducts which form female internal genital organs and upper part of the vagina fuses at lower end with urogenital sinus which is lower part of cloaca, this fusion results in formation of vaginal plate in which a cavity appears at 10-15 weeks of fetal development. This results in formation of lower vagina, at lower end of vagina a membrane separates it from genital vestibule. This membrane is hymen, later on canalization occurs in membrane. If this canalization does not occur it results in imperforate hymen which results in blockage of menstrual flow.

C  Imperforate hymen typically presents with cyclical monthly abdominal pain around age of menarchae and absence of menstruation. The menstrual blood is collected in vagina causing haematocolpos, sometimes extending above upto the uterus causing haematometra and endometriosis. Pelvic ultrasound will show distended vagina or uterus. Other mullerian duct abnormalities can be noted on ultrasound like vaginal atresia, transverse vaginal septum, bicornuate uterus or septate uterus. Renal ultrasound will exclude renal abnormalities. Imperforate hymen appears as bluish membrane on vaginal examination. Simple  cruciate incision will result in drainage of blood and will resolve the problem.         

 A}History

I will ask about pain if it is cyclic in nature.i will ask her about pain or difficulty while passing urine.;any feeling of mass lower abdomen to look for symptoms of retention of urine.I will ask about secondary sexual charecteristics (breast development ,pubic and axillary hair,).I will ask patient about age of menarche in sisters ,mother to look for family history of delayed puberty.I will ask her sensitively about  sexual activity and history of aparunea/dysparunea(can be difficult  ?  as accompanied by her mother).Examination .I will measure height and weight according to age .i will examine secondary sexual characeters and pubertal tanner staging for breast pubic and axillary hairs.abdominal examination mass palpable ,tenderness.examination of exertarnal genitalia to  look for heamatocolpos(bluish color bulge), examination of inguinal areas palpable mass?gonads.No indication for Pelvic examination in this girl if she is not sexuallu active.investigationsI will request for pelvic ultrasound to see presence of ovaries ,uterus and heamatocolpos,heamatometra.Further investigation depends on history clinical examination and ultrasound .If breast is well developed with normal pubic and axillary hair and  ultrasound   done showed heamatocolpos/heamatometra just surgical excision of hymen is all that needed and no further investigation is required.If  normal breast pubic/axillary hair,cyclical ovulatory symptoms  with absent uterus or vagina will indicate mullerian agenesis( Mayer Rokitanky Kuster Hauser Syndrome) then I will  prefer to do MRI as this is associated 40% with renal tract anomalies.X.Ray bone , audiogram and echocardiography may be considered as there is association with MRKH syndrome and skeletal deformities ,cardiac abnormalities and auditery malformation.  .If clinical xamination reveal normal breast absent pubic hairs or any palbable  mass?gonads  in inguinal area I will ask  Karyotyping in this girl for androgen insensitivity syndrome .

B)

Fused mullerian ducts  elongates and reach urogeital sinus where it forms sinovaginal bulb.sinovaginal bulb forms vaginal plate and upper part of vagina while lower part is formed by urogenital sinus.Hymen is a solid membrane between the proximal uterovaginal tract and  vestibule of vagina (introitus).during normal development central part of hymen disappears creating an opening at level of vestibule.but if it donot it result in imperforate hymen resulting collection of menstrual fluid in vagina uterus  heamatocolpos /heamatometra.

C)

I will explain the diagnosis to patient and her mother.i will explain that this need to be corrected surgically as there is no medical treatment for imperforate hymen.i will explain to patient that it  has no long term implications on her sexual life and childbearing . Retrograde menstruation and development of endometriosis is a possibility  and need to be explained sensitively as risk is small.. I  will arrange an appropriate procedure schedule under GA. An elliptical  excison of membrane near hymenal ring by scalepel or electrocautery followed by the evacuation  of obstructed material.Avoid to press uterus to drain the material to avoid menstrual blood regurgitation. .Avoid needle aspiration as itwill not drain the material and cause infection..  to prevent recurrence absorbale sutures  to do marsupilisation by anchoring membrane edges to vaginal wall  retained secretion are usually sterile and does not need antibiotic.  .postop simple NSAID analgesic ,Folly,s catheter to allow free urine drainage .

Follow up review  in 6 to 8 weeks after menstrual cycle  

..

. .

A ) This scenario of primary amennorhoea with recurrent abdominal pain & normal gonadotrophins could occur due to outflow tract abnormalities causing crptomenorrhoea but a detailed history & examination needed to rule out other cause of primary amennorhoea &  abdominal pain as well.

I would obtain a detailed history about  nature of pain ,location , duration, whether cyclical  , progressively increasing in severity  , radiation, aggravation & relieving factors,  quality of life ,like absenteeism from school, any medications taken . Colicky pain in pelvis , initially cyclical ,  gradually increasing in severity and later persistent pain could suggest an outflow tract obstruction)

History of any lower abdominal pressure , fullness, any difficulty in passing urine or defecation  which could suggest pelvic collection with pressure symptoms).

I would obtain a history of the time of onset of  breast development ,  growth spurt  & pubic hair growth and their sequence of events  would be noted.  Family history of any affected sibling  with primary amennorhoea or maternal history of age on onset of menarche noted  as MRKH , CAIS has familial inheritance & delayed puberty could also be constitutional ).

History of  any hearing diasability enquired as MRKH could be associated with hearing difficulty.

History of altered bowel frequency, consistency, bloating enquired   to rule out any non gynaec pathology like IBS.

I would ask the mother or a chaperone to  be present while examination is done . Nature of examination is explained to the girl & mother to relieve their anxiety.

Clinical examination done to note the height , weight & BMI. Secondary sexual characters of breast , pubic hair & tanner staging done. Normal breast development  & blind ending with sparse pubic hair could suggest  Complete androgen insensitivity ,while  normal breast development with blind vagina &  normal pubic hair suggests MRKH syndrome.

 Abdominal examination  done to note any  mass , tenderness  . Renal angle palpated to note any abnormal mass as renal anomalies are associated with outflow tract anomalies. Any hernia or any palpable inguinal mass suggestive of gonad noted.

Examination of vulva & perineum  done gently to assess  pubertal changes , pubic hair growth  & staging.  A bluish bulging membrane in vulval  region   is characteristic of imperforate hymen  with cryptomennorhoea , whereas a pink membrane could suggest a low  tranverse vaginal septum , any blind ending vagina also noted. Any palpaple gonad in vulval region noted .

b) Hymen is a thin membrane that occurs at the junction of sinovaginal bulb & urogenital sinus which fuse to form the lower vagina. Hymen is perforated in fetal life itself. Failure of this perforation leads to a an intact membrane which accumulates blood at puberty which collects & distends the vagina appearing as a bluish bulge .

c) Ultrasound needs to be done to confirm the diagnosis of hematocolpos & any extension to uterus , tubes or pelvis noted.

Explain the nature of the problem of imperforate hymen & that it requires surgical treatment as a day case procedure either under  general or regional  anaesthesia.

Explain that drainage of the blood would relief her pain & prevent further blood collection extending into uterus or pelvis ,which may cause endometriosis .

Consent from the parent need to be obtained   but the girl needs to be  explained of the nature of procedure and any concerns addressed and to involve her in the decision about her care . Written information provided.

Surgical treat ment is under general anaesthesia  , a cruciate incision is made in the hymen & collected blood drained & the remaining quadrants are excised to avoid reunion.   No intrauterine instrumentation to be used  to avoid introducing infection.

Antibiotics single dose are necessary to prevent infection but  hematocolpos is usually sterile. Adequate post postoperative analgesics given. I would explain that blood may continue to drain for a 5-7 days. 

I would reassure them that there is usually no long term sequelae  in early cases. I would give  advice on resumption of routine activities and to report if there is any fever, foul smelling discharge pervaginum.

a)The girl has reccurent abdominal pain,so a history should be taken regarding the site, nature and intesity of pain,whether it is cyclical or not and what impact it is having on her quality of life .Associated features like difficulty in micturition should be enquired as it  can indicate a pelvic mass which can be a hematometra.Any association with defecation like relief in pain should be enquired as this can point towards inflammatory bowel disease.Symptoms suggestive of ulcerative colitis like diarrhoea or blood in stool should also be asked. Her sexual history should be taken and if sexually active risk factors of sti should be enquired.As she also has primary ammenorrhea age of menarche of mother and siblings should be enquired .She should be asked about any chronic illness ,strenuous exercise,major weight loss as all these can lead to ammenorrhea.Ahistory of eating disorder should be taken ,any history of chemotherapy or radiotherapy should be enquired as these can cause ammenorrhea.She should also be asked about any psychological stress.An examination should include weight,height,calculation of BMI.Breast development ,and development of pubic and axillary hair should be assessed.An abdominal examination should be done to note any mass or tenderness.A perineal examination may help to identify a bulging membrane at the introitus.Abluish bulge is synonymous with an imperforate hymen while a pink bulge denotes a transverse vaginal septum.Aperrectal examination may detect a mass in the vagina which may be a hematocolpos. The patient will require a transabdominal ultrasound to diagnose the presence of uterus cervix and vagina.It will also help to diagnose any hematometra ,hematosalphinx or hematocolpos.If uterine anatomy cannot be delineated MRI may be needed.B)Hymen is a membrane present between the urogenital sinus and the sinovaginal bulb.It is perforated in fetal life.Absence of this perforation will lead to imperforate hymen.C)The management of imperforate hymen is surgical.The procedure should be explained to the girl and her mother and informed consent should be taken.Under regional anesthesia and antibiotic coverage a cruciate incision is given over the bulging membrane.The four quadrants may be excised or the flaps may be left as such and the collected blood should be allowed to drain.The patient should be informed that it will take 3-5 days for the complete drainage and that there will be no sequlae of the condition.

a. Clinical assessment..

A detailed history will be taken from the girl maintaining a supportive approach. She will be asked about the nature, site and severity of pain. Its effect on her quality of life will be assessed by asking about absenteeism from school or sports activity. Cyclical severe lower pelvic pain with retention of urine suggests haematocolpus.

History of urinary symptoms like frequency,urgency,dysuria is associated with urinary tract infection. Bowel symptoms like diarrhoea,constipation,change in frequency and form is asked for inflammatory  bowel disease.History of excessive exercise, loss of weight and eating disorders  [anorexia,bulimia] is also associated with amenorrhoea. Note any anosmia and galactorrhoea as in Kallman,s syndrome. History taken for visual disturbances, headache to rule out pituitary adenoma. Enquire about onset and sequence of puberty changes for breast development, axillary and pubic hair. Family history of delayed menarche in mother and female siblings is present in constitutional delay. She will be asked about any stress at school or home .Also enquire sensitively if she is sexually active ,about  use of drugs or contraception. Past medical history of renal disease, cystic fibrosis may affect the ovarian function. Past history of chemotherapy or radiotherapy enquired as maybe the cause for amenorrhoea.

Examination will be done to note height, weight,BMI. Assessment of secondary sexual characteristics by Tanners staging for breast ,pubic and axillary hair is done. I will do an abdominal examination for any palpable masses, rigidity or rebound tenderness. Features of Turners syndrome like webbed neck, short stature,cubitus valgus noted.

 Examination of external genitalia for clitoromegaly, ambiguous genitalia .Vaginal opening inspected and presence of a bulging bluish membrane with dark blood transilluminating suggests imperforate hymen while a pink thick membrane suggests transverse vaginal septum. .A per rectal examination may show mass in vagina as in haematocolpus.An Ultrasound examination  will confirm haematocolpos ,also absence of uterus ,vagina and renal tract anomalies’.MRI may further confirm the findings.

b.Imperforate hymen is an embryological defect due to  non canalisation of hymenal membrane in foetal life. The membrane lies  between the sinovaginal bulbs and urogenital sinus and its  imperforation leads to the collection of blood after menarche.

c.I will explain  the diagnosis to the mother and daughter, reassuring about the good prognosis of  the surgical treatment. The surgery involves a cruciate incision in the hymen  to drain the collected blood which usually takes  3-5 days. Remnant hymenal tissue canbe excised or left behind .Written information maybe provided. Surgery is done as a day-care procedure usually under regional or general anaesthesia. Urinary catheterisation will be done, consent taken  from girl if she is Fraser competent. Prophylactic antibiotic and postoperative analgesia offered. A follow-up appointment is given.

Can u please correct my answer - Dr Padmaja

A.Young age with primary amenorrhoea needs very sensitive handling and preferably girl should be along with the mother. Due to recurrent abdominal pain my focus will be to rule out other causes of abdominal pain along with obstructive lesions of the genital tract. I will ask details about pain duration and interval, regularity, how much this pain effects her daily life any treatment if she is taking any relation of pain with bowel or urination , presence of diarrhea or constipation to rule out gastrointestinal causes, pain and burning micturition for urinary tract infection. Any episode of urinary retention which can be the result of lower vaginal atresia and formation of hematocolpos putting pressure on urethra.  other premenstrual symptoms like breast pain, mood changes and their relation with pain will help in diagnosing obstructive lesion of genital tract. I will ask about age at which she noticed her breast and pubic or axillary hair development. I will ask about her sexual activity, nausea vomiting to rule out pregnancy. I will ask about any excessive exercise, stress, dieting, wt loss or gain, previous systemic illness which can lead to amenorrhea, history of cancers ,chemo or radiotherapy. Wt gain acne hirsuitism to rule out polycyctic ovarian disease.her family history of age of menarche and other congenital abnormalities is also important.

Examination will include her height and BMI. After taking verbal consent I will examine her preferably in the presence of her mother to see for secondary sexual charachteristics breast and pubic hair development. Abdominal examination for any mass or tenderness. Perineal inspection to see for external genitalia, presence or absence of bluish or a pinkish membarane at hyminal area presence of which shows thin imperforate hyminal membrane or thick vaginal septum respectively. Rectal examination can reveal a large buldging mass in the vagina showing hematocolpos.

B.Vaginal development in the fetus occurs by the fusion of mesodermal mullerian ducts and endodermal urogenital sinus by around 8- 10 weeks of gestatione in the form of vaginal plate which is a column of squamous tissue, developing into a cavity by 10- 16 wks. It is controvercial that how much of vaginal tissue is made by each of tissues,mesoderm and endoderm, but usually it is believed that upper vagina( 4/5^th ??) by muleerian mesoderm and lower part by urogenital endoderm.cavity of vagina and urogenital sinus are separated by a thin endodermal membrane initially which later on by around 5^th month breaks to form hymen.failure of breakage of  this endodermal membrane will present as imperforate hymen

C.Management will include  abdominal and pelvic ultrasonography to confirm hematocolpos and renal tract abnormalities, which can be supplemented with IVU or MRI if needed. If it is found to be a thin imperforate hymen with hematocolpos , explanation of the condition to girl and the parents, and their psychological reassurance that this condition can be successfully treated by simple surgical means and probably no effects on future reproductive life, after informed  written consent from parents, proper analgesia and antibiotic cover a cruciate incision will be put on buldging membrane to allow drainage of collected menstrual blood. The remaining portions can be excised or can remain as such. It should be explained to the girl that menstrual blood will drain within few days.  Follow up after few weeks to see any remaining collection or re occlusion.

KRA

A

 Hx  I would find out about the impact on her quality of life, progress at school, her studies and absence from school due to pain.

I would sensitively explore to find out if she is sexually active because she could be pregnant.

I would find out about any family history of delayed menarche in her siblings if any and when her mother had her menarche.

I would find out about any family history of diabetes and herself.

I would find out if she has any frequency or urgency which could be due to an abdominal mass.

I would find out about weight loss and any strenuous exercise.

I would find out any childhood cancer requiring chemotherapy or radiotherapy can cause amenorrhoea.

I would find out about any symptoms of diarrhoea alternating with constipation that could be a gastrointestinal problem.

EXAM

I would check her weight and height to calculate her body mass index.

I would take her consent perform a physical examination to assess the development of secondary sexual characteristics, breast development, axillary and pubic hair development.

I would perform an abdominal examination to exclude and pelvic masses.

I would inspect the vulva to heck for any bluish bulging membrane suggesting an imperforate hymen.

I would look for the presence of webb neck and a wide carrying angle.

INVESTIGATION

I would request an ultrasound scan or magnetic resonance imaging to assess the pelvis, confirm the presence of uterus and exclude renal tract abnormalities.

I would perform karyotyping to determine whether she is XY or XX.

B

An imperforate hymen is a thin membrane at the junction of the sinovaginal bulb and urogenital sinus.  This results in menstrual blood accumulating behind the membrane and because there is no exit for the blood this results in vaginal distention and the feeling of pelvic mass.

C

I would counsel her and explain to her she needs surgical intervention.

I would consent her and explain that it involves making a cruciate incision to relieve the obstruction.

I would tell her she should make a good recovery and resume normal menstrual flow.

Dear Paul,  Could you please check my answer Thanks      Vinivee S

a)      Detailed history is obtained regarding site,nature of pain, whether it occurs at regular intervals,severity and impact on QOL.If she has to take time off school.

Associated symptoms such as nausea or vomiting  or diarrhea could be due to gastroenteritis. Recent change in frequency or form of stool and relief of pain on defecation may be due to IBS.

Urinary symptoms i.e dysuria and frequency are suggestive of UTI.

H/o childhood sexual abuse and psychological stress at home should be enquired,as it may contribute to her recurrent pain.

H/o difficulty in defecation or micturition with lower abdominal pain ,may be due to collection of  blood in case of Imperforate hymen.

With regards to her not starting her menstruation ,hx is taken about development of secondary sexual  characters. Whether she has developed breast , axillary and pubic hair and growth spurt, presence of these in chronological order would indicate normal hypothalamo-ovarian function.

             H/o psychological stress,at home or poor performance at school should be enquired .

h/o  anorexia nervosa or bulimia , h/o head trauma or infections ,h/o anosmia

should be taken as these may suggest hypothalamic causes,

h/o chemotherapy and radiotherapy may cause ovarian dysfunction.

h/o galactorrhea,headache or visual disturbance may be due to hyperprolactinemia

symptoms of hypothyroidism as weight gain ,increased sensitivity to cold and lethargy should be enquired

     Family h/o delayed onset of menstruation in mother and siblings may point to familial cause.

    Opportunity should be taken to enquire in privacy if she is sexually active, to rule out pregnancy.

    Examination ,height weight and BMI is checked.Dysmorphic features noted.Short height with short            webbed neck with wide carrying angle is due to Turner’s syndrome .

   Neck is examined for thyroid enlargement,Breast examined  and presence of axillary and

pubic hair is noted and staged according to Tanner’s staging,presense of galactorrhea is noted.

  Abdominal examination is done to look for any abdominopelvic mass,Groins palpated for any

  masses,which may be seen in case of primary ammenorrhea

  Pelvic examination should be restricted to inspection ,if she is not sexually active.

 Clitoral size,and configuration of hymen is noted.Degree of estrogenisation and perineal         hygiene is noted,Vestibule is examined by gentle lateral spread of labia majora   .Imperforate  hymen will be seen bulging with bluish discoloration.

b)Hymen is thin membrane that occurs at the junction of sinovaginal bulb and urogenital sinus.Failure of this membrane  to perforate during embryonic life causes  blood to collect behind the membrane and leading to primary amenorrhea.

c)Management of  imperforate hymen includes two important aspects,Psychological couselling and Surgical correction.She should be counseled that this is correctable and and her sexual life and fertility will not be affected.A cruciate incision will release the collected blood completely in 3-5days with no sequele.

Dear Paul,

Sorry there is error,sentence reads as,-----Groins should be palpated for masses ,that are found in cases of primary ammenorrhea due to androgen insensitivity syndrome

a)clinical assessment

Detailed history including nature & severity of abdominal pain whether cyclic or not& its effect on quality of life. I will ask about eating disorder as it may point to anorexia nervosa, smell & ouder recognition which is absent in kallman syndrome & history of blurred vision &headache which may point to pituitary adenoma. History of strenuous exercise may be a cause of hypothalamic amenorrhea also history of past head injury. I will ask about development of secondary sexual charachters such as breast development, growth spurt & pubic, axillary hair. Sexual history whether sexually active or not. I will check if any medical or psychological disorders or intake of any medications e.g. antipsychotic drugs. Also family history of 1ry amenorrhea as some cases are due to hereditary syndromes e.g. kallman syndrome or due to constitutional cause.

Examination of the girl to check body built, stature & low body mass index as in anorexia nervosa.I will check the development of 2ry sexual characters e.g. breast development, axillary & pubic hair. I will check the presence of hirsutism, temporal baldness. Abdominal examination to check any tenderness or pelviabdominal mass e.g. hematometria in case of imperforate hymen. Local examination may reveale infantile genitalia as in turner syndrome or bluish bulging membrane as in imperforate hymen. Also I will check for clitorimegaly as in congenital adrenal hyperplasia.

b)The lower 2 thirds of vagina develop from the urogenital sinus while the upper one third develops from lower end of fused mullarian ducts. Failure of canalization of vagina may results in imperforate hymen or trasverse vaginal septum.

c)I will explain the condition to the girl & her mother, written information can be provided. Incision of the imperforate hymen & drainage of collected blood is curative.  

This young girl has primary amenorrhoea.   Normal FSH, LH , PRL rules out a hypothalamic pituitary cause. Normal thyroid function and androgen levels suggests that thyroid disease and hyperandrogenism are not contributory. 

I will ask about the frequency of the pain. Cyclical pain occurring every month suggests that she might be menstruating. but the blood flow is obstructed. I will also ask if she has difficulty passing urine or defecating which she may experience if the blood is accumulating in the vagina.

I will ask if breast development has occurred and the age that it started. Breast begins about two years prior to onset of menses. I will also ask about growth of pubic hair which also occurs prior to the start of periods.

I will enquire of any family history of delayed menarche . If positive , a genetic cause , for example Mayer Rokitansky Kuster Hauser syndrome may be the cause.

I will enquire about her general health. Chronic illnesses may cause delayed menarche. 

I will ask about any involvement in strenuous sports which will contribute to a hypothalamic cause of delayed menses. Her endocrine function tests will however not be expected to be normal.

I will sensitively enquire about intimate relationships and any attempt at sexual intercourse and whether this was successful.

I will do a general examination looking especially for signs of chronic illness. I will measure weight and height and calculate BMI. I will assess breast and pubic hair and assess her Tanner stage.

The abdomen will be examined for any distention especially in the lower abdomen. 

I will palpate for a mass arising out of the pelvis.This may be due to accumulation of blood within the uterus causing it to distend. A distended bladder may also be palpable.

I will get verbal consent to examine the vulva to exclude abnormalities of the external genitalia, like cliteromegaly. I will examine for an imperforate hymen which will appear as a bulging membrane with a bluish tinge or a low transverse vaginal septum which will be pinkish and may be bulging.

If the vulva is normal I will do a pregnancy test. I will also request an ultrasound of the pelvis to examine for a normal uterus ovaries and to exclude haematocolpos, haematometra or haematosalpinx.

1. Two solid evaginations called the sinovaginal bulbs grow out of the pelvic part of the  urogenital sinus. The sinovaginal bulbs proliferate to form the vaginal plate. The vaginal plate develops a lumen by the breakdown of the central cells to form the vagina. The hymen develops at the junction of the sinovaginal bulbs and urogenital sinus . This becomes perforated during fetal development. Failure to become perforated leads to imperforate hymen.
2. The girl and her mother will be counseled about the diagnosis . They will be told this condition developed because the hymen did not perforate during fetal development. 

They will be reassured that the ovaries tubes  uterus and vagina are present. She is expected to have normal menses after treatment.Sexual and reproductive function are also likely to be normal after treatment

 A cruciate incision will be made to the imperforate hymen . The flaps may be left in situ or incised. The blood collected behind the hymen is expected to drain off within three to five days. Regular monthly periods is expected unless there is another cause for menstuation to be irregular.

Assessment of this girl should be carried out in sensitive way; Rapport   needs to be built with patient & her mother to obtain optimal assessment.

History should include the nature of the pain (constant, cyclical), severity & effect of the quality of life, for example ,ask about school absence as a result of the pain.

Any  associated urinary or bowel symptoms, ask about the age of development of secondary sexual characters such as breast , axillary &pubic hair development. I will ask about any abnormal eating habit, exercise pattern & stressful situation.  h/o of any headache , abnormal discharge from the breast(galactorrohea),clarify any unusual symptoms such as inability to smell

Clarify any significant medical problem such as DM, cystic fibrosis, renal disease or significant psychotic problem ,also I will ask about any previous  surgery , trauma , previous infection(meningitis).I will ask if the patient on any regular medication ,or h/o receiving chemotherapy.

Family H/o of delayed menstruation , renal or gynecological disorder.

By the end of the history taken stage you will be able to comment if the patient is farsear  comptent

Examination should be carried out in a very sensitive way as patient at this age will be embarrassing & shy. General examination  will include any obvious  dyshmorphic feature, also BMI & arm span should be taken , Breast development should be assessed according to Tanner stages as well as Axillary & pubic haie development.

Abdominal examinations include ant palpable abdomino plelvicmass  or  any tenderness

Vulva inspection for any abnormalities, any palpable mass in the groin region

in case of imperforated Hymen vulval examination will reveled membrane which is blue in appearance with dark blood translluminating through the thin membrane, in cases of the transverse vaginal septum the membrane will be Pink & the membrane will be much thicker.

B) The embryology of Imperforated Hymen : Hymen is the thin membrane at the junction between the urogenital sinus & sinovaginal bulb , this membrane usually perforated during the fetal life, failure for the membranes to perforate & being intact at puberty causing the menstrual blood to collect behind the membrane & the vagina begin to distended, increased distention causing cyclical pain because of haemtocolpos & may cause urinary or bowel symptoms.

C) Diagnosis can be confirmed with ultrasound or MRI imaging with the appearance of haematocolpos

Dianosis should be explained clearly using non-medical terms, patient should be advised that  surgical approcach is the main stay treatment & should be reassured regarding the simplicity & the safety of the procedure & that will be no long term complication associated with this condition

ACruciate incision on the hymen to relieve the obstruction &the remaining quadrants of the hymen could be left or incised , the haematocolps will completely drained with 3-5 days.

Assessment of this girl should be carried out in sensitive way; Rapport   needs to be built with patient & her mother to obtain optimal assessment.

History should include the nature of the pain (constant, cyclical), severity & effect of the quality of life, for example ,ask about school absence as a result of the pain.

Any  associated urinary or bowel symptoms, ask about the age of development of secondary sexual characters such as breast , axillary &pubic hair development. I will ask about any abnormal eating habit, exercise pattern & stressful situation.  h/o of any headache , abnormal discharge from the breast(galactorrohea),clarify any unusual symptoms such as inability to smell

Clarify any significant medical problem such as DM, cystic fibrosis, renal disease or significant psychotic problem ,also I will ask about any previous  surgery , trauma , previous infection(meningitis).I will ask if the patient on any regular medication ,or h/o receiving chemotherapy.

Family H/o of delayed menstruation , renal or gynecological disorder.

By the end of the history taken stage you will be able to comment if the patient is farsear  comptent

Examination should be carried out in a very sensitive way as patient at this age will be embarrassing & shy. General examination  will include any obvious  dyshmorphic feature, also BMI & arm span should be taken , Breast development should be assessed according to Tanner stages as well as Axillary & pubic haie development.

Abdominal examinations include ant palpable abdomino plelvicmass  or  any tenderness

Vulva inspection for any abnormalities, any palpable mass in the groin region

in case of imperforated Hymen vulval examination will reveled membrane which is blue in appearance with dark blood translluminating through the thin membrane, in cases of the transverse vaginal septum the membrane will be Pink & the membrane will be much thicker.

B) The embryology of Imperforated Hymen : Hymen is the thin membrane at the junction between the urogenital sinus & sinovaginal bulb , this membrane usually perforated during the fetal life, failure for the membranes to perforate & being intact at puberty causing the menstrual blood to collect behind the membrane & the vagina begin to distended, increased distention causing cyclical pain because of haemtocolpos & may cause urinary or bowel symptoms.

C) Diagnosis can be confirmed with ultrasound or MRI imaging with the appearance of haematocolpos

Dianosis should be explained clearly using non-medical terms, patient should be advised that  surgical approcach is the main stay treatment & should be reassured regarding the simplicity & the safety of the procedure & that will be no long term complication associated with this condition

ACruciate incision on the hymen to relieve the obstruction &the remaining quadrants of the hymen could be left or incised , the haematocolps will completely drained with 3-5 days.

Assessment of this girl should be carried out in sensitive way; Rapport   needs to be built with patient & her mother to obtain optimal assessment.

History should include the nature of the pain (constant, cyclical), severity & effect of the quality of life, for example ,ask about school absence as a result of the pain.

Any  associated urinary or bowel symptoms, ask about the age of development of secondary sexual characters such as breast , axillary &pubic hair development. I will ask about any abnormal eating habit, exercise pattern & stressful situation.  h/o of any headache , abnormal discharge from the breast(galactorrohea),clarify any unusual symptoms such as inability to smell

Clarify any significant medical problem such as DM, cystic fibrosis, renal disease or significant psychotic problem ,also I will ask about any previous  surgery , trauma , previous infection(meningitis).I will ask if the patient on any regular medication ,or h/o receiving chemotherapy.

Family H/o of delayed menstruation , renal or gynecological disorder.

By the end of the history taken stage you will be able to comment if the patient is farsear  comptent

Examination should be carried out in a very sensitive way as patient at this age will be embarrassing & shy. General examination  will include any obvious  dyshmorphic feature, also BMI & arm span should be taken , Breast development should be assessed according to Tanner stages as well as Axillary & pubic haie development.

Abdominal examinations include ant palpable abdomino plelvicmass  or  any tenderness

Vulva inspection for any abnormalities, any palpable mass in the groin region

in case of imperforated Hymen vulval examination will reveled membrane which is blue in appearance with dark blood translluminating through the thin membrane, in cases of the transverse vaginal septum the membrane will be Pink & the membrane will be much thicker.

B) The embryology of Imperforated Hymen : Hymen is the thin membrane at the junction between the urogenital sinus & sinovaginal bulb , this membrane usually perforated during the fetal life, failure for the membranes to perforate & being intact at puberty causing the menstrual blood to collect behind the membrane & the vagina begin to distended, increased distention causing cyclical pain because of haemtocolpos & may cause urinary or bowel symptoms.

C) Diagnosis can be confirmed with ultrasound or MRI imaging with the appearance of haematocolpos

Dianosis should be explained clearly using non-medical terms, patient should be advised that  surgical approcach is the main stay treatment & should be reassured regarding the simplicity & the safety of the procedure & that will be no long term complication associated with this condition

ACruciate incision on the hymen to relieve the obstruction &the remaining quadrants of the hymen could be left or incised , the haematocolps will completely drained with 3-5 days.