Thursday, 24 December 2009

Congenital adrenal hyperplasia in Pregnancy

Information on Congenital adrenal hyperplasia for Public

Congenital adrenal hyperplasia occurs as a result of gene mutation.

This results in defective glucocorticoid & mineralocorticoid synthesis.

Excellent Power point explaining congenital adrenal hyperplasia is here

Diagram explaining adrenal hormone synthesis

Mode of inheritance is autosomal recessive.

Clinical features

3 catergories of diseasse - depending on the type of mutation

(I)Salt Wasting(SW)

(II) Simple Virilising(SV)

(III) Non-Classic(NC)

The condition is usually diagnosed in infancy

(A) Female infant-ambigious genetalia

(B)Acute illness due to hyponatraemia (salt losing crisis due to mineralocorticoid defeciency)

90% due to 21 hydroxylase enzyme deficiency.These patients have both glucocorticoid & mineralocorticoid defeciency.

5-8% due to 11 hydroxylase deficiency.These patients have excess deoxycortisol, that shows mineralocorticoid activity,leading to hypertension.

How the disease affect pregnancy?
(A)Reduction in fertility , especially in patients with salt wasting type disease.

Reasons: (i) inadequate control of hyperandrogenism
(ii)Inadequate introitus due to poor surgical repair

(B) Disease inheritance by fetus

(C) Risk of Miscarriage,pre-eclampsia & IUGR increased

(D) Masculinization can lead to android type pelvis that may lead to failure to progress in labour.



2 Types of Clical problems

(I) Pregnant women with Congenital adrenal hyperplasia

(II)Women who is carrier for Congenital adrenal hyperplasia with previously affected child( or with a heterozygous partner)

Genetic counselling

(I) This is a autosomal recessive disorder.

(II)Prenatal diagnosis is possible with a battery of gene probes through chorionic villus sampling at 10 weeks

(III) In the past diagnosis made by estimation of 17 hydroxy progestrone & androgen levels in amniotic fluid.

(IV)Zygosity of partner for Congenital adrenal hyperplasia gene can be done.In case gene deletion ,30% of heterozygous , diagnosis can be made accurately.The other 70 % has gene mutation which can be mimicked by pseudo genes of normal individuals. So negative gene testing in the partner reduces the probability of heterozygosity from 1:50 to 1:70.

Differentiation of female genitalia occurs between 9-10 weeks,so even a diagnosis at 10weeks would be late in preventing masculinization of a female fetus with congenital adrenal hyperplasia.

Dexamethasone,the steroid which is capable of crossing the placenta, can be used to suppress the fetal adrenal gland to avoid masculinization. The therapy should be started at least by the fifth week.It may even be started preconceptually. This treatment regime isn't always successful in preventing masculinisation.

Maternal compliance is shown by reduced urinary cortisol or oestriol level.

If the chorionic villus sampling shows a female fetus with congenital adrenal hyperplasia(CAH), the treatment should be continued till delivery.In case of female fetus with congenital adrenal hyperplasia ,termination is also an option.

In a pregnancy with CAH in a male fetus or unaffected fetus the treatment should be discontinued.

Fetal sex determination by ultra sound scan is helpful as maternal androgen excess has minimal effects on male fetus.

Pregnant women with congenital adrenal hyperplasia
On the other hand some steroids can cause masculinization but patients need steroid to suppress their disease activity.However the placental aromatase activity is sufficient to prevent masculinization of fetus.

Hydrocortisone, cortisone acetate, prednisone, methylprednisolone are inactivated in the placenta but dexamethasone crosses the placenta and causes neonatal adrenal suppression.

Regular assessment of clinical status,serum electrolytes and serum androgen levels to adjust the glucocorticoid and mineralocorticoid therapy.

Generally the patients with 21-hydroxylase deficiency doesn't require alteration in dosage during pregancy.

Serum testosterone & free testosterone levels should me measured every 6 weeks in first trimester & every 8 weeks thereafter.Aim is to maintain free testosterone levels at high to normal levels for pregnancy.

Labour & delivery

Elective caesarean is indicated in patients who had genital reconstructive surgery.

Caesaren section rate among these patients is increased and this could be attributed to android type pelvis resulting from masculinization.

Stress dose glucocorticoid therapy ( Hydrocortisone 100 mg IM ) is indicated for patients undergoing labour.

Newborn should be examined for ambiguous genitalia.Female pseudohermaphroditism is either due to maternal hyperandrogenism or fetal 21 hydroxylase deficiency(if father is a carrier).

Affected fetuses would require steroid treatment but even the unaffted fetus would require short term steroid support when its adrenal activity is suppressed in utero as the result of treatment of mother.


1.Nelson-Piercy C. Handbook of obstetric medicine. Taylor & Francis; 2002.

2.Swiet MD. Medical disorders in obstetric practice. Wiley-Blackwell; 2002.


4.K. Hagenfeldt , P.O. Janson , G. Holmdahl , H. Falhammar , H. Filipsson , L. Frisén , M. Thorén , and A. Nordenskjöld
Fertility and pregnancy outcome in women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency Hum. Reprod. Advance Access published on July 1, 2008, DOI 10.1093/humrep/den118.Hum. Reprod. 23: 1607-1613.

Tuesday, 1 December 2009

Artificial heart valve in pregnancy

Preconceptual evaluation

cardiac function should be assessed-Clinical/Echo/Exercise testing
moderately or severely symptomatic (class III and IV) should be advised against pregnancy

(B)Fetal risks-abortion/prematurity/intrauterine growth restriction/congenital abnormalities-due to warfarin/inheritance in patient's with congenital heart disease

Inheritance of congenital heart disease

(C)Anticoagulation should be discussed-if anticoagulation is altered there is an increased risk thromboembolism. If thromboembolism occurs during pregnancy the risk to the fetus increased again.

(D) management planned by multi disciplinary team(Obstetrician/cardiologist/Cardiothoracic surgeon)

2 major types of valves

1.mechanical- Suggested INR is 3-4.Failure to anti coagulate could result in valve thrombosis and stroke.Subcutaneous heparin anti coagulation may inadequate in patients with artificial valves.
three categories : caged-ball tilting-disc bi leaflet valves prosthetic or homo graft-
three categories hetero grafts homo grafts auto grafts.

They don't require anti coagulation but shorter lives than mechanical valve.Patients may need anti coagulation if they develop Atrial fibrillation.

Opinion varies as to whether pregnancy accelerate homo graft valve deterioration.

Fetal risks of Warfarin
Chodrodysplasia punctata
Optic atropy

Factors determining the choice of anticoagulation
1.Site of valve replacement(mitral more thrombogenic aortic)

2.Type of valve ( ball & cage more thrombogenic than bi leaflet valves)

3.Past history of thromboembolic events

4.No of mechanical valves

5.patient choice

Anticoagulation regimens-3 broad possibilities

1.Warfarin throughout the pregnancy
2.Heparin & warfarin alternatively Heparin between 6-12 weeks & after 36 weeks
3.Heparin throughout the pregnancy

Aspirin is a useful adjunct when heparin is used.

If LMWH is used anti Xa levels should be monitored 4-6 hrs post injection.LMWH doesn't cross placenta

Advantages of LMWH

1.fewer bleeding complications
2.lower frequency of thrombocytopenia
3.lower incidence of osteoporosis
4.longer half life
5.more predictable dose response

The following is the commonly used regime

Conception is difficult to time & risk of congenital malformation is likely to be minimal in the first 4 weeks, so patient could conceive on warfarin.

Then patients are given intravenous heparin aiming to double APTT during 6-12 weeks.

Patient can be converted to warfarin from 12 weeks.

At 37 weeks patient us converted to continuous intravenous heparin.

If patients goes into labour while on warfarin vitamin K & FFP should be given & heparin should be commenced.
Heparin & LMWH can be reversed with protamine sulphate.

Postpartum patient continues on heparin for 3-7 days.Patient can breast feed while in heparin or warfarin.

Patients on dindevan shouldn't breast feed.

Antibiotic prophylaxis is indicated in patient with artificial valve.
Current recommendations amoxicillin 2g i.v & gentamicin 1.5 mg/kg i.v

Heart failure in pregnancy

medicine that can be used:digoxin, diuretics, nitrates, hydralazine, and
beta blockers.

medicine that should be avoided:angiotensin-converting enzyme
inhibitors and angiotensin receptor antagonist amiodarone sodium nirtotroprusside

Valve thrombosis in pregnancy

Thrombolysis is the first line approach
Surgery is reserved for patients in thrombolysis is contraindicated.

Patient information leaflet :Breast feeding while on anticoagulation


1.Nelson-Piercy C. Handbook of obstetric medicine. Taylor & Francis; 2002.

2.Swiet MD. Medical disorders in obstetric practice. Wiley-Blackwell; 2002.

3.Elkayam U, Bitar F. Valvular Heart Disease and Pregnancy: Part II: Prosthetic Valves. J Am Coll Cardiol. 2005 Aug 2;46(3):403-410.

4.The Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy

Evidence-Based Guidelines

Thursday, 26 November 2009

Mnemonic for Obstetric Haemorrhage

Management of Atonic Obstetric Haemorrhage-"HAEMOSTASIS"

General medical management

H-call for help

A-assess (vital signs/blood loss) & resuscitate

E-Establish aeitiology(4T-Tone/Tissue/ Trauma/Thrombin)

E-Ecbolics(syntometrine/ergometrine/bolus syntocinon)

E-Ensure availability of blood products

M-Massage the uterus

O-Oxytocin infusion,prostaglandins(intravenous,rectal,intramuscular,intramyometrial)

Specific surgical management

S-shift to operating theatre(bimanual compression anti-shock garment,especially if tranfer is required

T-Tissue & trauma to be exculded & balloon tamponade or uterine packing

A-apply compression sutures

S-sytematic pelvic devascularisation(uterine,ovarian,quadruple,internal iliac)

I-interventional radiology,uterine artery embolisation

S-Subtotal/Total hysterectomy


Mukherjee S, Arulkumaran S. Post-partum haemorrhage. Obstetrics, Gynaecology & Reproductive Medicine. 2009 5;19(5):121-126.

More Mnemoinics

Tuesday, 17 November 2009

Cystic fibrosis in Pregnancy

This is a multisystem disorder with impaired cellular secretion.
Commonest autosomal recessive disorder in U.K.
In Carrier rate 1:25 in Caucasians.In USA 1:30 American is a symptomless carrier.

Most common single mutation in Nonhispanic Caucasians is deltaF508.This occurs in approximately 65-70% of cases.DNA probes exist to detect 95% of mutations.If the couple already had a affected child,the defective gene could be identified and then prenatal diagnosis can be done to exclude cystic fibrosis.

Clinical features
1.recurrent lung infection/bronchiectasis/respiratory failure

2.pancreatic deficiency-malnutrition & diabetes.

Effect of Cystic fibrosis in Pregnancy
Female: Fertility is reduced due to 1.thick cervical mucous 2.low lean body mass 3. voluntary infertility due to fear of producing affected children.

Most are infertile due to atresia of the vas & epididymis.

Pre pregnancy counselling

Factors to consider

1.cor pulmonlae

2.uncontrollable recurrent respiratory tract infection.

3.vital capacity - Patients with vital capacity less than 50% recommended termination.Many cystic fibrosis patients and patients with kyphosis with such vital capacity have normal pregnancy.

4.Right ventricular cavity size at end diastole is a good prognosticator of clinical outcome.

5.Patients may have diabetes and/or liver disease.Screening for diabetes need to be done.

6.Presence of Burkholderia cepacia is also an important risk factor.
Women's life span is reduced,so implications for parenting should be discussed.

Contraindication to pregnancy

1.Pulmonary hypertension
2.Cor pulmonale
3.FEV1 <30-40 class="ii gt">
If father is a carrier-50% of babies wold be born with cystic fibrosis,other 50% would be carriers.

If father's carrier status unknown, the risk of CF baby is 2-2.5% as the carrier status is UK general population is 1:25.

1.risk of spontaneous abortion not increased.
2.complication in pregnancy
(B)IUGR-due to chronic hypoxia

Principles of antenatal management

1.antenatal screening- Power point on Cystic Fibrosis screening maternal nutrition
3.regular physio therapy
4.prevention & early treatment of infections.
5.avoid prolonged hypoxia
6.regular growth monitoring-Growth restriction can be managed by bed rest, nutritional supplements & oxygen.

Antibiotic treatment

Generally risk of infection is greater than the side effects of antibiotics.
Penicillin,cephalosporins and erythromycin are safe in pregnancy.
Tetracycline should be avoided.
Monitoring of drug levels should be done when intravenous aminoglycosides are used.
Data on new drugs like imipenam is limited.

The patient is managed by joined care between cystic fibrosis center & specialized obstetric center.

Screen for diabetes mellitus around 20 weeks

Presence of resting hypoxia(oxygen saturation 80-90%) is an indication for admission for bed rest & oxygen therapy.

Intrapartum with fluid & electrolyte as patients may easily become hyponatraemic & fluid overload should be avoided in corpulmonle.

2. epidural or caudal anaesthesia is preferred as there is risk post anaesthetic atelectasis.

3.risk of pneumothorax- be wary of chest pain

4.instrumental delivery could be used avoid prolonged second stage.

Breast feeding isn't contraindicated.Sodium & protein content of breast milk is normal.

Most medications used in the management of cystic fibrosis are safe in pregnancy.

Effect of pregnancy on disease

Pregnancy didn't affect the rate of annual decline in FEV1.

1.Nelson-Piercy C. Handbook of Obstetric Medicine, Second Edition. 2nd ed. Taylor & Francis; 2001.

2.Swiet MD. Medical Disorders in Obstetric Practice. 4th ed. Wiley-Blackwell; 2002.

Other sources

Article from Chest Journal

Another article

Wednesday, 26 August 2009

Ulcerative Colitis in pregnancy

Inflammatory bowel disease in pregnancy

Effects of disease on pregnancy

Fertility-fertility isn't affected in quiescent disease.
Ulcerative colitis- Fertility is little affected in active disease except for severe disease
Crohn's - infertility correlates with disease activity.

Fetal outcome:
First trimester-rates of abortion in patient with pre-existing IBD is similar to general population.

Fetal outcome is generally good (80%) in quiescent disease but increased risk of preterm labour & small for gestational birth could occur, especially in active disease.

Drug Therapy on fertility

Female-no effect
Male-Sulphasalazine reversible azoospermia

Effect of pregnancy on IBD

Pregnancy has little effect on the course of IBD.

Risk of flare up minimized if disease is quiescent at the time of pregnancy.

Clinical features of exacerbation are:abdominal pain/diarrhoea/passage of rectal mucus & blood.

Risk of exacerbation similar to non pregnant patients(50%).

Exacerbation occur in the first two trimesters & mostly mild.

Crohn's-remains quiescent in 75%.Most exacerbations in first trimester & postpartum.


1.advise to embark on pregnancy when disease is quiescent.
2.Folic acid supplementation. Sulphasalazine is a dihydrofolate reductase inhibitor.


Multidisciplinary team-Obstetrician,Surgeon,Gastroenterologist

1.Management of acute episodes & chronic disease is similar to non pregnant patients.

Drugs-Oral or rectal sulphasalazine, mesalazine and other 5-aminosalicylic acid drugs can be safely used in pregnancy & breast feeding.

Folic acid given for the reasons explained above.

Oral or Rectal steroids can be used to induce remission.

Azathioprine,6-mercaptopurine & ciclosporine may be needed to maintain remission.There is no evidence of excess congenital abnormalities but refractory disease as well as treatment are associated with IUGR & preterm birth.

2.if surgery is indicated (obstruction/haemorrhage/
perforation),it shouldn't be delayed because of pregnancy.

Vit B12 is essential for ileal Chron's disease or following ileal resection.

Antimotility agents & stool thickeners usage should be avoided because they contain anticholinergic component which is associated with congenital anomalies.

Patients with previous surgery for Inflammatory bowel disease

They do well if there disease has been quiescent preconceptionally.
Intestinal obstruction is a complication in patients with ileostomy.
Patients with ileal pouch-anal anastomosis can go for vaginal delivery unless there is cephalopelvic disproportion or marginal anal incontinence.

Mode of Delivery

Vaginal delivery is aimed unless there is perianal Crohn's disease as this may make the perineum inelastic & also there is delaying in healing of episiotomy.

If caesarean section is considered in patients with previous surgery for Crohn's disease,It is important to liaise with the surgeon.

Intrapartum hydrocortisone is considered if Steroids have been used antenatally.


Inflammatory bowel disease is an additional risk factor for deep vein thrombosis.
Mother can breastfeed.Risk of kernicterus due sulphapyridine is minimal.

Prednisolone doses upto 30 mg/day hasn't been shown to cause fetal adrenal suppression.

Azathioprine and breast feeding

Sources :

Swiet MD. Medical disorders in obstetric practice. Wiley-Blackwell; 2002.

Nelson-Piercy C. Handbook of obstetric medicine. Taylor & Francis; 2002.

Parangi S, Levine D, Henry A, Isakovich N, Pories S. Surgical gastrointestinal disorders during pregnancy. Am J Surg. 2007 Feb;193(2):223-32. Review. PubMed PMID: 17236852.

Friday, 27 February 2009

Genital cancer needs editing

Multiple leisons in 70%
99.7%. Cervical ca related to HPV

HPV double DNA

Early gene E late gene L. Vaccine L1 gene
HPV vaccine 16 18 responsible 70% Ca cervix

Quadrivalent vaccine Gardasil 6 11 16 18
9-26 yrs

How long does immunization work?
Should men be vaccinated?

>120 types HPV

E6/E7 gene

Smoking cessation engouages regression of CIN

Barrier methods regression of CIN

circumcision ?

Recall system reduction in incidence 60%

Genital cancer needs editing

Multiple leisons in 70%
99.7%. Cervical ca related to HPV

HPV double DNA

Early gene E late gene L. Vaccine L1 gene
HPV vaccine 16 18 responsible 70% Ca cervix

Quadrivalent vaccine Gardasil 6 11 16 18
9-26 yrs

How long does immunization work?
Should men be vaccinated?

>120 types HPV

E6/E7 gene

Smoking cessation engouages regression of CIN

Barrier methods regression of CIN

circumcision ?

Recall system reduction in incidence 60%

Amenorrhora(Needs edit)

With secondary sexual characteristic
With out secondary sexual charecterstic
Excess androgen
Absence of uterus

Rockintasy 30-40% urinary tract abnormalities
Can have their children-surrogate mother

Adolescent menstrual dysfuction may be worries about pregnancy STD contraception


XY females
Pure gonadal dysfenesis
Enzymatic failure
End organ insensitivity
Exogenous hormones-CAH

Teen age pregnancy (needs editing)

I'M highest teenage pregnancy rate in Western world.
Definition 13 yrs-below 18 yrs in gov publication includes 18-19 yrs.

Teen age pregnancy is cause and consequence of social exclusion.

Problems-medical social education

Medical hypertension anaemia placental abruption prematurity sudden infant death syndrome

Social deprivation low self esteem low education

Friday, 23 January 2009


Definitions unable to conceive >12 months but epidemiologist tend to define as >24 months

Fertility depends on frequency of intercourse
Ovulation induction kit decreases the fertility

Use of lubricants decreases the sperm motility
Risk of Infertility
PID * 1 7
PID*3 19

Semen analysis if poor repeat in 72 days

Chlamydia antibodies

Basal body temperature not recommended.Marital dysharmony

Female genital mutilation

WHO definitions whole or part
Type 1-4
Type 3 partial closing of vagina
Type 4 corrosives

Africa Asia not faith related Christian Muslim

Immediate complications

Urinary retention

Post op positioning leg finding

Longterm consequences

Type 2 &3
Obstetrics labour


Shock amongst carers
Painful examinations
Analgesia regional
Episiotomy anteriorly not laterally

Female Genital Multilation Act 2003

Reproductive Medicine

Learning points from regional teaching
Hypothalamus-GnRH that acts on anterior pitutitary
Pitutitary FSH/LH act on granulosa
Ovary granulosa cells secretes inhibin

3types of oestrogen :17b estradiol oestriol oestrone

Oestrogen helps in development of secondary sexual charecteristics and preserves bone density.

Ovary's dimension 4*1.5*1.5 cm



USS-ovarian volume >10ml
2-12 mm cysts 10 or more

Polygenic disorder of steroid biosynthesis or insulin metabolism.

LH acts theca cell

2 cell theory

SHBG reduced by androgen & insulin.SHBG produced in liver .

Free androgen index =total testerone*100/SHBG. Normal <4.5

Impact of Obesity insulin resistance and increase in insulin like growth factors


Endocannabiods regulate multiple endocrine system including HPO axis.

Intrauterine life has an impact


Anovulation with PCOS

Group 2 Anovulation

Consensus on infertility treatments ESRE
1-preconceptual-weight loss
2-ovulation induction
Multiple pregnancy clomiphene 10%


Tamoxifen less trials

Clomiphene + Dexamethasone is one option
Metformin Doses 500. mg or 850 mg bd
ESRE only indicated glucose intolerance or type 2 DM

Gonadotropins cumulative success rate 40-50%

1-2% severe OHSS. Possible tx

Rotterdam crieteria

Ovarian adhesion

Laparoscopic diathermy free of risk multiple
Pregnancy , no ovarian HSS

LOMNI new technique.

Wednesday, 14 January 2009

Learning points from Audit meeting

Obstetrics Cholestasis

If LFT is elevated needs
2.Hepatitis B serology
4.Autoimmune Screen

Do we have to fasting bile acids?

Postnatal care
1.contraception-avoids Combined Oral Contraceptive pills.
2.ensure resolution of LFT

1000 lives campaign

Mechanical compression Elective LSCS (TEDS)/Emergency section(Flowtron)

Elective Gynaesurgey Prophylactic antibiotic one hour prior to surgical incision

Debriefing after EmLSCS especially after EmLSCS,third degree tear is important.

Thursday, 8 January 2009

Flashcards for Obstetrics & Gynaecology

Flash cards are used as memory aid.One writes question on one side & answer overleaf.
Here are some resources.

Flashcard exchange


Basic terms in Obs & Gynae

My web site

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Thursday, 1 January 2009

Guidelines in Obstetrics & Gynaecology

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My website