CASE STUDY 23 Facilitator Pawin Puapornpong Gravid Year

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CASE STUDY 23 Facilitator: Pawin Puapornpong

CASE STUDY 23 Facilitator: Pawin Puapornpong

รายละเอยดการตงครรภครงกอน Gravid/ Year GA (term/ preterm) G 1/2551 Term G 2/2552 3 mo G

รายละเอยดการตงครรภครงกอน Gravid/ Year GA (term/ preterm) G 1/2551 Term G 2/2552 3 mo G 3/2553 Term G 4/2554 7 mo Route/comp/ abortion Birth weight Sex/ health NL 2, 800 g Male /healthy Spontaneous abortion with D&C C/S due to arrest of dilatation - - 3, 200 g Female /healthy - - Hydrop fetalis : aboerion by Vg delivery (cytotec)

Date GA Fu nd al Ht 13/8/57 7+6 28/8/57 Pre sen tati on FH

Date GA Fu nd al Ht 13/8/57 7+6 28/8/57 Pre sen tati on FH S F Wt M BP Uri ne (pr o/s ug) Abn. sy mtomp s /Edem a Treatment คล ำไม ได - 68. 2 118/66 -/- - Lab ANC 9+6 คล ำไม ได - 69. 7 115/65 -/- - 24/9/57 13+6 1/3 SP - 70. 9 115/73 -/- - 21/10/57 17+5 =u mb Tra nsv. - 72. 6 125/68 -/- - Ob US 2 nd to 3 rd trimester 28/10/57 18+6 - Ca uda l - 74. 9 120/73 /3+ - Ob US 2 nd to 3 rd trimester 11/11/57 20+6 - Ca uda l - 75. 9 107/76 -/- - Cordocen tesis for Hb typing 19/11/57 21+6 - Ca uda l - 75. 5 104/58 -/- - ผลCordocen tesis : Hb Bart’s

INVESTIGATION

INVESTIGATION

Lab investigation • • CBC Blood group Hb typing PCR for Alpha-thal 1 gene

Lab investigation • • CBC Blood group Hb typing PCR for Alpha-thal 1 gene HBT cord blood Chorionic villi sampling at GA 10 – 14 weeks Ultrasound

Lab and Investigation • ������� Hb/Hct 11. 7/37 MCV 61. 5 OF : Decrease

Lab and Investigation • ������� Hb/Hct 11. 7/37 MCV 61. 5 OF : Decrease DCIP : Negative Blood group : O Rh : Positive Hb typing : Hb. F 0. 4% Hb. A 2 2. 4% Hb. A 88. 6% VDRL : Non reactive Anti-HIV : Negative HBs. AG : Negative • ���� Hb/Hct 11. 2/33. 7 MCV 57. 6 OF : Decrese DCIP : Negative Hb typing : Hb. F 0. 0% Hb. A 2 2. 6% Hb. A 88. 3%

Cordorentesis (HBT cord blood) • WBC count 64. 1 x 10 3 /mm 3

Cordorentesis (HBT cord blood) • WBC count 64. 1 x 10 3 /mm 3 • MCHC 24. 4 g/d. L • Hb 5. 3 g/d. L • RDW 2 8. 5 % • Hct 21. 7 % • Platelets 84 x 10 3 /mm 3 • RBC count 2. 18 x 10 6 /mm 3 • Hb Bart’s Present • MCV 99. 5 f. L • Hb. H • MCH 24. 3 pg Present Interpretation Hb Bart’s hydrop fetalis

Ultrasound • 1 st at GA 17+5 wk by date / 16+5 wk by

Ultrasound • 1 st at GA 17+5 wk by date / 16+5 wk by ultrasound (21/10/57) Fetal heart sound : positive Fetal movement : positive Amniotic fluid : normal Fetal abnormality : cardiothoracic ratio > 0. 5; no hydropic change Placental grading : 0

Ultrasound • 2 nd at GA 18+4 wk by date / 17+3 wk by

Ultrasound • 2 nd at GA 18+4 wk by date / 17+3 wk by ultrasound (18/10/57) Fetal heart sound : positive Fetal movement : positive Fetal abnormality : cardiothoracic ratio > 0. 5 Placental thickness : 3. 7 cm

Ultrasound • 3 rd at GA 20+9 wk by date / 19+2 wk by

Ultrasound • 3 rd at GA 20+9 wk by date / 19+2 wk by ultrasound (11/11/57) Fetal heart sound : positive Fetal movement : positive Fetal abnormality : cardiomegaly Placental thickness : 4. 07 cm

Ultrasound • 4 th at GA 25+4 wk by date / 23+2 wk by

Ultrasound • 4 th at GA 25+4 wk by date / 23+2 wk by ultrasound (15/12/57) Fetal heart sound : positive Fetal movement : positive Fetal abnormality : cardiomegaly, minimal pericardial effusion and ascites, placentomegaly

DISCUSSION

DISCUSSION

ANC risks Hb Bart’s hydrop fetalis with alpha-1 trait 2. Previous cesarian section 3.

ANC risks Hb Bart’s hydrop fetalis with alpha-1 trait 2. Previous cesarian section 3. Previous abortions 4. Grand multiparity 1.

Problem list 1. 2. 3. 4. 5. Fetal anomalies : cardiomegaly with minimal pericardial

Problem list 1. 2. 3. 4. 5. Fetal anomalies : cardiomegaly with minimal pericardial effusion, ascites and placentomegaly detected by ultrasound at 25 week (by ultrasound) Alpha-thal 1 trait in both parents Previous abortions - Spontaneous abortion (2552) - Bart’s hydrop fetalis (2554) Previous cesarian section due to arrest of dilatation (2553) History of severe pre-eclampsia (2554)

Impression: Hydrop fetalis • Definition : accumulation of fluid in at least two fetal

Impression: Hydrop fetalis • Definition : accumulation of fluid in at least two fetal compartment • Immune vs non-immune

Hydrop fetalis Immune (10 -20% of cases): Alloimmune hemolytic disease/Rh isoimmunization Non-immune (80 -90%

Hydrop fetalis Immune (10 -20% of cases): Alloimmune hemolytic disease/Rh isoimmunization Non-immune (80 -90% of cases): -Twinnning -Fetal anemia -Tumors -Congenital infection -Miscellaneous : Cystic -Cardiac : hygromas, inheritable structural/arrhythmia disorders of -Pulmonary metabolism, malformations -Chromosomal/Genetic maternal thyroid syndromes disease, -Thoracic Abnormalities congenital nephrotic syndrome.

MANAGEMENT OF THALASSEMIA IN PREGNANCY

MANAGEMENT OF THALASSEMIA IN PREGNANCY

Bart’s hydrops fetalis • Counselling for termination of pregnancy • Induction / Cesarian section

Bart’s hydrops fetalis • Counselling for termination of pregnancy • Induction / Cesarian section • Induction by • Medication • Oxytocin, Misoprostol • Medical procedure • Amniotomy, ARM

Management in this patient. • ควร Early prenatal diagnosis ������� ANC GA 13+6 wk

Management in this patient. • ควร Early prenatal diagnosis ������� ANC GA 13+6 wk ����� CVS • Early terminate of pregnancy ���������� • Medical induction by misoprostol(200) ½ tab q 6 hr. • Plan Normal labor • ����������

Thank you for your attention

Thank you for your attention