Obstetrics (#3)

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Obstetrics (#3)
2012-11-20 00:38:47

Fetal Skeletal Dysplasias Face and Neck Fetal Heart Multiple Gestation Amniotic Fluid Hydrops
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  1. - endochondral ossification begins at 10 weeks LMP
    - mandible and maxilla (8-10 wks LMP)
    -all long bones ossified by end of 1st Trimester
    - fetal spine 18wks LMP
    Normal Fetal Skeletal development (embryology)
  2. indications for skeletal dysplasias (4)
    • specific genetic risk(previous)
    • apparent limb abnormality
    • associated anomaly
    • polyhydramnios
  3. errors of Morphogenesis (etiologies)
    • Malformation: genetic/teratogenic abnormal formation
    • Deformation: abnormal force alters structure (ex. oligohydramnios)
    • Disruption: abnormal interference destroys tissues (ex. ABS)
  4. Patterns of shortening?
    • Rhizomelia: proximal shortening
    • Mesomelia: middle shortening
    • Acromelia: distal shortening (hands/feet)
    • Micromelia: all portion short
    • Phocomelia: flippers (no limbs)

  5. Abnormal femur length is defined as?
    -4 SD GA for skeletal dysplasias (-2SD for chromosomal anomalies)
  6. T or F: Dysplasias that manifest later with less severity will have better prognosis?
  7. what to assess when considering skeletal anomaly?
    • patterns of shortening of long bones
    • shape/contour/density of bone
    • spine (ossification) - Radiograph
    • thoracic circumference/shape
    • hands/feet
    • calvarium and facial features
    • skin folds
    • amniotic fluid
  8. What are the three Common Lethal Dysplasias?
    • Thanatophoric Dysplasia
    • Achondrogenesis
    • Osteogenesis Imperfecta II
  9. What are the three Rare Lethal Dysplasias?
    • Congenital Hypophosphatasia
    • Camptomelic dysplasia
    • Homozygous Achondroplasia
  10. Dysplasias are considered lethal when?
    • severe micromelia (all shortened) >4SD
    • decrease thoracic circumference <5th %
    • pulmonary hypoplasia
    • long bone fractures
    • Kleeblattchadel
  11. severe micromelia (rhizomelia)
    macrocrania with frontal bossing
    narrow, bell shaped chest
    thickened redundant skin folds
    bowed limbs ("telephone appearance)
    Platyspondyly (flattened vertebral bodies)
    Kleeblattschadel (Cloverleaf skull)
    • Thanatophoric Dysplasia (common lethal)
    • **most common lethal skeletal dysplasia
    • AD mutation
  12. severe micromelia
    macrocrania and frontal bossing
    narrow chest
    thick redundant skin folds
    bowing limbs (fractures may be present)
    decreased mineralization (vertebral bodies, ischium, public bones)
    severe hydrops
    • Achondrogenesis (common lethal)
    • **2nd most common dysplasia
    • AR mutation

    • Type I (20% most severe) abnormal cranial and spinal ossification
    • Type II (80%) abnormal vertebral body but normal cranium
  13. defective collagen "brittle bones"
    Type I, III, IV
    Type II (lethal) severe micromelia, with multiple fractures "accordian bones", decreased mineralization, platyspodyly
    • Osteogenesis Imperfecta (common lethal)
    • AD or AR
  14. profound hypomineralization (skull may look like acrania)
    severe micromelia
    bowed, thin long bones/absent (+/-) fractures
    shortened ribs, small circumference
    • Congenital Hypophosphatasia (rare lethal)
    • AR mutation
  15. bent limbs (no fractures)
    narrow thorax
    severe clubbed foot
    absent/hypoplastic fibulae
    • Camptomelic Dysplasia (rare lethal)
    • "bent bone"
  16. rhizomelia
    narrow thorax
    large cranium
    narrow spine
    • Homozygous Achondroplasia (rare lethal)
    • One parent must be affected but usually both carry gene
  17. Non-lethal Skeletal dysplasias
    • Heterozygous Achondroplasia
    • Asphyxiating Thoracic Dysplasia
    • Diastrophic Dysplasia
  18. normal lifespan and normal intelligence
    late onset rhizomelia (27 wks)
    macrocrania and frontal bossing
    normal thoracic circumference
    • Heterozygous Achondroplasia
    • **most common form of non lethal dysplasias
  19. --> absent or short radius
    Radial Ray Abnormalities
  20. 1) Holt-Oram Syndrome (skeletal/cardiac anolamies)
    2) Thrombocytopenia-absent radius syndrome (blood disorer with RR)
    3) Roberts-Phocomelia (pseudothalidomide syndrome)
    4) VACTERL sequence
           are associated with what focal limb anomaly?
    Radial Ray Abnormalities
  21. --> medial deviation and inversion of sole of the foot

    due to: chromosomal, NTD, skeletal dysplasias, decreased fluid
    Talipes Equinovarus "Clubfoot"
  22. --> absent arch due to large calaneus

    associated with T18
    Rockerbottom Foot
  23. --> presence of extra digits (+/- bone)

    associated with T13
  24. --> three digits
  25. --> fusion of digits (webbed)
  26. --> marked shortening of hand digits with trident (pitchfork) configuration

    associated with homozygous achondroplasia
  27. --> permanent incurvature of digits (may seen clenched fists with overlapping fingers)
    bilateral or unilateral

    associated with T18
  28. --> abducted thumb

    associated with diastrophic dwarfism
    Hitchhiker's Thumb
  29. --> from RR, or absent ulnar

    isolated Karyotype abnormality
    Talipomanus "Clubhand"
  30. --> amnionic bands extend from chorionic surface to fetal tissue resulting in organ dissruption, amputations, clefting, or constriction rings

    **occurs from 1st trimester rupture of amnion
    Amniotic Band Syndrome (ABS)
  31. --> believed to result form early amnion rupture, vascular disruption or error in development resulting in lethal complex malformaitons
    - short cord/abdomen attached to placenta
    - evisceration of organs (omphalocele/open abdominal defect)
    - limb and spine defects (scoliosis, etc)
    Limb-Body Wall Complex (LBWC) or Body Stalk Anomaly
  32. embryology of this begings at 5 wks LMP and is complete by 10 wks LMP?
    Embryology of the face and neck
  33. 5 main processes of the face and next migrate and fuse together?
    • 1) 1 Nasal Frontal Process
    • 2) 2 Maxillary Prominences
    • 3) 2 Mandibular Prominences
  34. Nasal Frontal Process starts superior; move inferior (form mid forehead, nose, central upper lip, central maxilla, anterior palate)

    Maxillary Prominences move medially (form lateral upper lip, maxilla, hard palate)

    Mandibular Prominences move medially (form mandible)
    • Embrylogy of Face and Neck
  35. Migration of nose, eyes, and ears?
    • Nose start above orbits; move medially and inferiorly
    • Eyes move medially
    • Ears start below mandible; move laterally and superiorly
  36. Facial anomalies occur in ? births?
    Polyhydramnious occurs with what % of those facial anomalies
    ?% of the time other nonfacial anomalies occur due to chromosomal abnormalities
    • 1/600
    • 60%
    • 50%
  37. List the drugs that can affect the development of the face
    • alcohol
    • codeine
    • valium
    • anti-epileptic drugs
  38. Axial view through face (orbits and cranium are in view) assesses what?
    • binocular distance
    • orbital size
    • presence of hard palate
  39. Tangential coronal view of nose lip assesses?
    • cleft lip
    • presence of 2 nostrils
  40. Profile view assess?
    **limited by: oligohydramnious and obesity
    • prominence of chin and forehead
    • protruding tongue
    • flattened nose
    • nasal bone
  41. What is the orbital "Rule of Thumb"?
    • orbits should be separated by about the distance of an orbital globe (eye)
    • "Eyeballing It"

    * Outer orbital distance can be used to date pregnancy (if no BPD)
  42. --> congenital absence/sever hypoplasia of the eye
    • Anophthalmia
    • associated with T13
  43. --> small eyes (interocular measurement)
    • Micropthalmia
    • associated with T13
  44. --> wide spread eyes

    - Commonly due to mass blocking anterior migration (ex. encephalocele)
    - also due to craniosynostoses
  45. --> closely spaced eyes

    associated with T13
  46. --> fusion of eyes into one; typically with supraorbital proboscis
    • Cyclopia
    • associated with T13
  47. two closely spaced, but separate eyes, with supraorbital proboscis
  48. --> two closely spaced, but separate eyes, with centrally placed nose having one blind ended nostril
  49. --> tuft of tissue (usually above eyes; absence of abnormal nose)
  50. --> cleft in middle of nose (nasal and maxillary fail to fuse)
    ** also have cleft lip
    Median cleft face
  51. occurs with 40% of T18 and 60% of T13

    isolated cleft palate is likely associated with other anomalies
    Cleft palate is associated with polyhydramnios and small stomach
    • Cleft lip and palate (5 Types)
  52. --> abnormally large protruding tongue beyond lips

    associated with T21 and Beckwith-Wiedemeann Syndrome
  53. -macroglossia
    -macrosomia (large baby)
    -renal hyperplasia/dysplasia (increased risk for Wilm's tumor or hepatoblastoma)
    Beckwith-Wiedemann Syndrome
  54. --> receding chin
  55. --> protruding forehead ( + hydrocephaly or skeletal dysplasia)
    Frontal Bossing
  56. If ? are placed low or small, can be associated with Trisomies
  57. Fetal thyroid begins function at ? weeks?
  58. hyperthyroidism or hypothyroidism will cause thyroid to enlarge in fetus true or false?

    • can be caused by Maternal Graves disease
    • may see neck mass/hyperextended neck on u/s
    • **due to Thyroid Dysfunction see IUGR, oligohydramnios, and tachycardia
    • **due to obstructing mass see polyhydramnios

    may be treated in utero with drugs
  59. --> cord looped 2+ times around the neck
    Nuchal Cord
  60. --> septated nuchal fluid (multiocular lymphatic fluid limed by endothelium lymphatic endothelium

    thought to arise from failure of lymphatic system to develop a communication to venous system in neck
    • Cystic Hygroma
    • "Lymphandiectasia"
    • *all cystic hygromas are NOT lymphatic in origin
  61. associations of Cystic Hygromas
    • often associated with Hydrops
    • 60% of hygromas are chromosomal abnormalities (Turner's, Down's, Noonan's, FAS and cardiac abn)
    • poor prognosis

  62. DDx of Cystic Hygromas *seen as early as 10wks
    posterior encephalocele (brain and skill and spine are normal tho)
  63. 11-14 wks
    < 3mm thick normal
    associated with trisomies if thickened
    Nuchal Translucency
  64. --> thickening due to subcutaneous edema
    16-20 wks
    <6mm normal
    associated with Down's
    Nuchal Fold Thickening
  65. --> solid mass composed of ectoderm, mesoderm, endoderm (may see hyperextension of neck)

    may cause polyhydramious, or dystocia
  66. -begins to develop at 5wks
    - paired heart tubes form and fuse to single heart by 10 wks
    Embryology of the fetal heart
  67. Describe fetal circulation?
    Placenta> Umb V > LPV> ductus venosum> IVC> RA> foramen ovale> LA> LV> aorto-iliac a> IIA (hypogastric)> Umb A> Placenta

    or RV> PA> ductus arteriosus> dsc AO>IIA> Umb a

  68. clinical indications for Fetal Echo?
    • Abnormal previous u/s
    • Family Hx
    • Previous Pregnancy
    • Maternal Disease associated with defects (Type 1 DM)
  69. u/s findings Associated with Heart Defects?
    • 2 vessel cord (also associated with renal anomalies)
    • CDH
    • Omphalocele
    • Thick Nuchal Fold/Lucency
    • Hydrops
    • Chromosomal Abnormalities
    • Bradycardia
  70. Fetal Echo Routine
    • *Establish situs (heart/stomach)
    • *4ch Heart and outflows
    • aortic arch&ductal arch
    • SAX ventricles and ao
    • SVC/IVC
    • Pulmonary veins
    • Doppler (inflows/outflows; ductus art/foram ov)
    • MMode through atrium and ventricle
  71. 4ch view r/o ? cardiac defects
    outflows r/o ? cardiac defects
    • 1/3
    • 2/3
  72. 120-160 normal
    <110 bpm (bradycardia) heart blocks
    >200bpm (tachy) decreased SV (treated with Digoxin)
  73. --> partial or complete absense of IAS (3rd most common cardiac anomaly)

    SAX best views
    **Foramen of Ovale should be no larger than the aortic root
    Atrial Septal Defect (ASD)
  74. --> Complete or partial absense of IVS ( most common cardiac anomaly)

    SUBC & SAX best views
    ** may close on own
    • Ventricular Septal Defect (VSD)
    • ** 50% are associated with other anomalies
  75. --> occurs when endocardial cushions fail to fuse resulting in one common valve  with 5 leaflets

    *heart begins as one common ventricle/atrium that communicate through AV valve
    • Atrioventricular Septal Defect (AVSD)
    • "AV canal" "endocardial cushion defect"

  76. 50% of fetuses with AVSD have?
  77. --> inferior displacement of tricuspid valve
    associated with hydrops, pulmonary stenosis, lithium injestion (bipolar meds)
    Ebstein's Anomaly

    • Large RA
    • Apical displacement of TV
    • dysplastic RV
  78. --> secondary to pulmonary atresia with no VSD
    Hypoplastic Right Ventricle

    • small RV
    • small/absent pulmonary artery
  79. --> due to increased flow into/out of the left ventricle

    associated with coarction of aorta
    Hypoplastic Left Ventricle

    • small LV
    • Aortic stenosis/atresia
    • MV atresia
    • EFE as pregnancy progresses
  80. --> occurs from muscle hypertrophy; replaced by collagen and elastic tissue causing decreased CO and CHF

    associated with hypoplastic LT heart
    Endocardial Fibroelastosis (EFE)

    echogenic and focally thick myometrium
  81. VSD
    Overriding aorta
    RVH (postnatally)
    Pulmonary stenosis
    • Tetralogy of Fallot
  82. --> single large vessel arising from base of heart (supplies CA, Pulmonary and systemic)

    Large overriding ao
    pulmonary artery origin off aorta
    • Truncus Arteriosus
  83. --> aorta and pulmonary arteries arise from RV

    Maternal DM and alcohol abuse
    • Double Outlet Right Ventricle
  84. closed circut (80%)
    RA to RV
    Great vessels are parallel
    seen in SAX
    may have VSD
    • D loop Transposition of Great Vessels (Complete)
  85. RV to LA
    normal pulmonary to systemic circulation
    parallel great vessels
    may be asymptomatic throughout life
    • L Loop Transposition of Great Vessels (Corrected)
  86. --> narrowing of aortic lumen (@ ductus arteriosus, arch, after aortic branches)

    prominent RV
    Small Lv
    narrowed portion of Aortic Arch
    associated with AVSD and VSD (dm)
    Coarction of Aorta
  87. Rhabdomyoma (cardiac hamartoma) most common

    solid echogenic tumors (usually of the IVS)
    *may obstruct outflows
    • Cardiac Tumors
    • DDx: cardiac fibroma, hemangioma, myxoma or teratoma
  88. --> damage to heart muscle (viral, bacterial, metabolic, maternal DM)
  89. --> heart outside thoracic cavity

    associated with Pentalogy of Cantrell
    Ectopia Cordis
  90. --> PM calcification (usually LV)

    associated with T21
    • Echogenic Intracardiac Foci (EIF)
    • *more echogenic than bone
  91. Isomerism
    Situs Ambiguous
    Cardiosplenic Syndromes --> symmetric development or normally asymmetric organs or organ systems (2 left or 2 right sides)
  92. --> bilateral left sidedness

    multiple spleens
    2 LT lungs
    midline liver
    IVC intrahepatic interruption (to azygous vein>SVC)
    Dextro or mesocardia
    • Polysplenia "Left isomerism"
    • (Cardiosplenic Syndromes)
  93. --> bilateral right sidedness
    associated with AVSD

    no spleen
    2 RA
    2 RT lings
    midline liver
    bilateral SVC
    • Asplenia "Right isomerism"
    • AKA: Ivenmark's syndrome

  94. T or F: heart defects with polysplenia are much worse?
    False: they are worse with asplenia
  95. commonly associated defects with cardiosplenic syndromes?
    • TGA
    • Pulmonary stenosis/atresia
    • common atrium
    • total anomalous pulmonary venous return with asplenia (2 RA)
    • complete heart block
  96. clues to diagnose Cardiosplenic Syndromes in fetus?
    difficult to diagnose
    • Interruption of IVC
    • Large azygous vein
    • abnormal liver, stomach or heart position
  97. Multiple gestation accounts for ? % of natural pregnancies?
  98. --> fertilization of two separate ova
    70% of all natural twins
    1/80 births
    hereditary (maternal side)
    own genetic mix
    separate or fused placentas ()
    Dizygotic Twins "fraternal"
  99. Frequency of Dizygotic Twins depend on?
    • Maternal age/parity
    • hereditary
    • Racial Background (nigerian, african american)
    • Pharmaceutical Agents (clomide and pergonal)
  100. --> single fertilized ovum replicates
    30% of all natural twims
    1/250 births
    random occurance
    genetically the same
    Monozygotic Twins "identical"
  101. What is the most important predictor of pregnancy outcome in twins?
    • Chorionicity
    • best determined between 10-14 weeks
    • Lambda (or peak) = dicorionic (fused)
    • T sign = monochorionic/DA
  102. if 2 placentas implant close together they may fuse

    some placenta may grow up between membranes

    this is known as?
    Twin Peak Sign/ Lambda Sign
  103. when division of ova occurs after implantation

    one placenta and membrane creates what?
    T sign
  104. Types of Monozygotic Twinning
    • Dichorionic-Diamniotic
    • Monochorionic-Diamniotic
    • Monochorionic-Monoamniotic
    • Conjoined
  105. -occurs with all dizygotic twins
    -may occur with monozygotic twins (18-30%)
    -Morula splits before implant (2 days post fertilization)
    --implants separately (2 chorion+2amnion=4 layer membrane)
    -2 placentas (may be fused )
      (may be ant/post placenta)

    •  (fused=lambda sign)
    • Dichorionic-Diamniotic
  106. -occurs only with monozygotic twins (70%)
    - division occurs at blastocyst stage (4-8 post fertilization)
    - 1 chorion; 2 amions (2 layer membrane)
    - 1 placenta (T sign)

    Monochorionic- Diamniotic
  107. - occurs only with monozygotic twins (4%)
    - division occurs at embryonic disc stage after amnionc sac hs formed (8-12 days post fertilization)
    - no membrane
    - 1 placenta
    *increased risk of mortality
    Monochorionic - Monoamniotic
  108. Which Twinning variation is impossible?
  109. --> incomplete divion at embryonic disc stage
    occurs on day 13
    no membranes
    Conjoined Twins
  110. Indications for Multiple Gestation scan?
    • strong family hx
    • LGA
    • increased maternal hCG
    • two or more heartbeats heard
  111. Multiple Gestation Protocol
    • 1. identify number of fetuses
    • 2. position of fetuses
    • 3. label Twin A/Twin B and side
    • 4. presence of membrane, placenta(s), twin peak sign
    • 5. treat each fetus as singleton
    • 6. show similar fetal parts in same image
    • 7. r/o  polyhydramnios (5-10%)
    • 8. r/o fetal demise "papyraceous"
  112. - MC/MA twins
    - Thoracopagus (chest) and Omphalopagus (abdomen) are most common join sites
    - "Diprosopus" localized anatomic duplication of anatomy (nose, face, head)
    - 70% are female
    - 1/3 are stillborn; 1/3 die day 1
    • Conjoined Twins
    • - lack of separating membranes
    • - more than three vessel cord
    • - complex fetal anomalies
  113. --> Twin reversed arterial profusion (A-A or V-V shunts in placenta)
    --> acardiac twin possesses no heart or very abn heart and completely profused by donor twin & only develops legs and trunk
    --> donor twin has high risk of developing hydrops due to high output cardiac failure
    Monochorionic Twins only

    TRAP Syndrome
    "Acardiac Parabolic Twin" "Acardiac Monster"
  114. --> parasitic twin within abdomen of its sibling (looks like teratoma)
    Fetus in Fetu
  115. --> fertilization of two separate ova
  116. Complications with Twins
    • twin to twin transfusion Syndrome (TTTS)
    • twin embolization syndrome
    • premature delivery
    • congenital anomalies
    • cord accidents
  117. Other abnormal Twinning variations
    • Hydatidiform mole with normal twin
    • heterotopic pregnancies
    • fetus in fetu
    • superfetation
  118. --> arteriocenous fistulas in the placenta (A-A or V-V or A-V) blood is not divided equally
    **A-V is the worst (difference in pressure gradient)
    --> anemic donor (small, hypotensive, oligohydramnios) "Stuck Twin"
     --> fluid overload recipient (large, edematous, hypertensive, polyhydramnios)
    Monochorionic Twins only

    Twin to Twin Transfusion Syndrome

    • - >20% discrepancy in size
    • - disparity in amniotic fluid
    • - single placenta
    • - thin or no membrane
    • - hydrops of 1 twin
  119. --> donor twin may have little or no fluid aroud it (the amniotic membrane holds baby tightly to uterine wall)
    Stuck Twin

    • --> occurs in dichorionic twins due to placental insufficiency
    • --> occurs in monochorionic twins due to TTTS
  120. demise of twin in monochorionic twins may result in renal, hepatic, and cerebral damage in surviving twin (clots pass causing infarct)

    demise of twin in di-di results in papyraceous fetus (resorbed twin)
    Twin Embolization Syndrome

    • - ventriculomegaly
    • - porencephalic cysts
    • - cerebral atrophy
    • - mocrocephaly
  121. uterus unable to accommofate two fetuses
    Premature Delivery
  122. genetic and developmental anomalies are more commin in monozygotic twins
    Congenital Anomalies
  123. tangled, prolapsed or wrap around neck and strangled cord
    Cord Accidents
  124. Functionc of Amniotic Fluid
    • protect from physical trauma
    • allows for growth
    • stable temperature
    • enables normal development of bones and muscles
    • matures lungs and GI
    • may provide nutrients
  125. Amniotic Fluid Production
    • 1st Trim: chorion frondosum, membranes, skin, GU tract & lungs
    • 2-3rd Trim: kidneys & lungs
  126. - 1cm pocket required for fetal breathing
    - max level reached at 22 weeks
    - begins to deminish at 34 weeks
  127. Estimating Amniotic Fluid Volume
    • Subjective: experience
    • Max Verticle Depth: deepest pocket free of cord or fetal parts (<8cm)
    • AFI: largest pocket in each quadrant summed (5-25cm)
  128. >8cm pocket
    >25cm AFI
    "fetus floating"
    Polyhyramnios (son pres)
  129. may cause maternal abdominal pain, SOB, PROM, preterm delivery

    Tx: Amniocentisis/drugs to reduce fluid
    (PROM, preterm delivery, infecton, premature closure of ductus arteriosus are complicaitons)
  130. Causes of Polyhydramnios
    • DM
    • CNS anomalies
    • High GI obstruction
    • Hydrops
    • TTTS
    • Musculoskeletal dysplasia
    • Idiopathis
  131. Causes of Oligohyramnios
    • PROM
    • IUGR
    • Renal anomaly
    • post dates
    • abruption (placental tearing away)
    • TTTS (donor)
    • Idiopathic
  132. <5cm AFI
    <2x2 cm pocket
    limb anomalies (clubfoot)
    pulmonary hypoplasia
    restricted growth
  133. --> abnormal accumuation of serous fliud in at least 2 body cavities or tissues
    • Hydrops
    • *common; but each etiology is rare
    • based in Immune or non-immune etiologies
    • terminal stage for many conditions (signifies fetal decompensation - 24-48hrs demise)
  134. Son Pres of Hydrops
    • Ascites
    • Pleural effusion
    • Pericardial effusion
    • Subcutaneous Edema
    • Placental Edema
    • A or V doppler abnormalities
    • low BPP scores
  135. --> fluid collecting in fetal abdomen (seen in pelvis first)
    can track down to scrotal sac causing hydrocele
    • Ascites (w Hydrops)
    • Pseudoascites - <2cm hypo ring
    • DDx: baby's fat layer under skin
  136. --> fluid in pleural space around lungs
    (>pleural effusion > pressure on mediastinum causing upper body edema, polyhydramnios, pulm hypo)
    Pleural Effusion
  137. --> fluid in sac surrounding heart (causes pressures on heart and great vessles)
    Pericardial Effusions
  138. --> general, local or limited to upper/lower body depending on etiology
    (generally first seen at fetal scalp adn face, then abd and limbs)
    • Subcutaneous Edema (Hydrops)
    • "Anacarca" --> general edema
  139. --> ground glass appearance >4cm placenta; late sign of hydrops
    Placenta Edema

    • If hydrops is of fetal etiology (whole thickened placenta)
    • IF hydrops is due to placental vascular malformation (partial hydropic placenta)
  140. with Immune Hydrops: Ascites>edema>pleural/pericardial effusions

    with Thoracic abn (chylothorax/heart defects) pleural/pericardial first
    Patterns Of Hydrops
  141. --> occurs when Rhesus sensitized mother has antibodies to fetal RBC
    Hemolysis occurs when maternal immunoglobin antibodies cross placenta and attack antigen positive fetal RBC
    • Immune Hydrops
    • "alloimmune Hydrops"
    • "erythroblastosis fetalis"
  142. 85% of pop are Rh+ (have Rhesus factor)
    80% of IH is due to anti D antibodies
    Rhesus Factor - red cell protein
  143. Attach on fetal RBC in IH causes?
    • anemia
    • hepatosplenomegaly
    • Erythroblastosis fetalis (outpouring of immature RBC which can not carry oxygen)>>causes: tissue hypoxia, hydrops, cardiac failure, demise)
  144. Assessment of Immune Hydrops
    • assess maternal antibody titers
    • sonographic assessment for signs of hydrops
    • MCA doppler (increase in velocities due to decreased viscosity of blood)
    • Optical Density Determination (ODD)- amnio to assess bilirubin in AF
  145. Treatment for Immune Hydrops
    Fetal Blood Sampling/Transfusion (PUBS)

    • with transfusion:
    • --- 70-85% hydropic fetuses survive
    • --- 85-90% nonhydropic fetuses survive
  146. commonly seen in 1st/2nd trim spontaneous abortions
    etiology varies geographically
    Non-Immune Hydrops
  147. --> blood disorder not compatible with life (if both parents pass gene, fetus has profound anemia>death)
    Homozygous Thalassaemia
  148. --> only one parent passes gene then fetus has relatively mild red cell anomalies
    Heterozygous Thalassaemia
  149. Maternal CAuses of NOn-Immune Hydrops
    • DM
    • Severe anemia
    • severe hypoproteinemia
    • TORCH
  150. Placental causes for Non-Immune Hydrops
    • Chorioangioma (shunting)
    • venous thrombosis
    • cord torsion
  151. Fetal causes for Non-Immune Hydrops
    • Cardiac (malfor, arrhythmias, output failure, TTTS)
    • Thorax/Neck (anything that causes compression)
    • Urinary (prune belly)
    • Chromosomal
    • Infection (TORCH)
    • skeletal dysplasias (achondroplasia/genesis, thanatophoric)
    • Fetal hypokinesis
    • idiopathic
  152. Investigation of NIH
    • Hx: etiology?
    • detailed scan for markers
    • Karyotype [FISH (fluorescent if situ hybridization) count chromosomes (24-48 hrs)]
    • Fetal Blood Sampling 48hrs
    • Cavity aspiration (sample fluid for chylothorax/theraputic reasons)
    • Pathology (placenta assessed)
    • Autopsy
  153. U/S assessment of NIH
    • assess change in degree of hydrops
    • cardiothoracic ratios
    • doppler for cardiac function (TR)
  154. Therapy for NIH
    *depends on etiology
    • Arrhtythmia: digoxin
    • Aneuploidy: none
    • Non immune anemia: transfusion
    • Chylothorax/CCAM 1: pleural drainage
    • Infections: antibiotics
    • Thoracentesis/Paracentesis pre delivery
  155. (Therapy for TTTS and NIH
    • serial therapeutic amniocentesis for recipient
    • laser ablation of communicating vessels
    • cord occlusion with TRAP syn (to prevent cardiac failure of donor)