Chapter 68=Congenital Malformations of The Skeletal System
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Transcript of Chapter 68=Congenital Malformations of The Skeletal System
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8/10/2019 Chapter 68=Congenital Malformations of The Skeletal System
http://slidepdf.com/reader/full/chapter-68congenital-malformations-of-the-skeletal-system 1/3
1/19/2015 Chapter 68. Congenital Malformations of The Skeletal System - Review of Medical Embryology Book - LifeMap Discovery
http://discovery.l ifemapsc.com/library/review-of-medical-embryology/chapter-68-congenital-malformations-of-the-skeletal-system
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EMBRYONIC DEVELOPMENT & STEM CELL COMPENDIUM
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I. V ertebral column
A. SPINA BIFIDA OCCULTA results from failure of fusion of t he halves of
the vertebral arch, most often in the lumber and sacral regions
1. It is a common defect but usually of no significance
2. The skin over the b ifid spine is usually intact, and there may be
no visible signs of the defec t exce pt for a "dimple" or tuft of
hair
3. Severe types do exist and are described under Nervous system
development
B. KLIPPEL-FElL SYNDROME (brevicollis): very rare; extreme shortening of
the neck due to a reduced number of cervical vertebrae. The rest of
the cervical vertebrae are usually abnormal in shape and may be fused.
Associated with othe r abnormalities
C. ASY MMETRICALLY FUSED VERTEBRAE or parts of vertebrae missing;
an increase or decrease in vertebral number is not uncommon due to
the complicated process of formation and rearrangement of the
segmental sclerotomes in development
II. Ribs: defe cts are mostly secondary to malf ormations of the vertebral column
A. IF PART OR ALL OF A VERTEBRA is missing, t he corresponding ribs aregenerally gone
B. IN SEVERE CONGENITAL SCOLIOSIS, the ribs on the concave side of
the chest are often fused or branched
C. ACCESSORY RIBS: usually the cervical rib (lumbar ribs are less
common)
1. Att ached to t he seventh cervical vertebra; may be unilateral or
bilateral
2. Pressure effec ts on the brachial plexus or subclavian vessels
may produce symptoms
3. From retention and development of costal processes of cervical
or lumbar vertebrae
D. FUSED RIBS: this may occur poste riorly when 2 or more ribs arise from
a single vertebra
1. Often associated w ith a hemivertebra which may produce
scoliosis
III. Sternum
A. CLEFT STERNUM
1. Minor clefts or notches are common and are seen as isolated
anomalies
2. Major clefts are usually associated w ith severe malformations of
the chest
3. Large clefts are rare ( heart); associated w ith herniation of
thoracic viscera
IV. The clavicles
A. CLEIDODYSOST OSIS: absence o f all or part of t he c lavicle
1. Usually bilateral and the shoulde rs are drawn forward to meet
under the chin
68. Congenital Malformations of The Skeletal System
Review of MEDICAL EMBRYOLOGY Book by BEN PANSKY, Ph.D, M.D.
PREV NEXTTABLE OF CONTENT67. Appendicular Skeleton And Skull Development 69. Development Of The Limbs
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8/10/2019 Chapter 68=Congenital Malformations of The Skeletal System
http://slidepdf.com/reader/full/chapter-68congenital-malformations-of-the-skeletal-system 2/3
1/19/2015 Chapter 68. Congenital Malformations of The Skeletal System - Review of Medical Embryology Book - LifeMap Discovery
http://discovery.l ifemapsc.com/library/review-of-medical-embryology/chapter-68-congenital-malformations-of-the-skeletal-system
2. Often associated w ith skull defe cts (cleidocranial dysostosis)
V. Skull malformations range from major defects incompatible w ith life to those
that are minor and relatively unimportant. The abnormalities are manifold, and
either all or part of the skull may be involved. They are frequently associated
with brain defect s
A. CRANIOSCHISIS OR ACRANIA: the cranial vault is almost absent and a
large spinal defect is often present. Also associated with anencephaly
1. Due to a failure of the cranial end of the neural tube t o close
during we ek 4, thus the cranial vault does not form
B. CRANIOSYNOSTOSIS OR CRANIOSTENOSIS: due to premature
closure of skull sutures
1. More common in male than female; associated with ot her
skeletal abnormalities
2. Type of de formed skull depends on which sutures close
prematurely
a. If sagittal suture: a long, narrow, wedge-shaped skull
(scaphocephaly)
b. If the coronal suture: a high, towerlike skull (oxyce phaly
or acrocephaly)
c. If coronal or lambdoid suture closes on one side:
twisted and asymmetric skull (plagiocephaly)
C. MICROCEPHALY : cranium is normal size or slight ly small, but the re is no
abnormal closure of the sutures. It is primarily an abnormality of the
CNS in which the brain and skull both fail to grow
Related Pages
Related Organs
Bone
Cartilage
Related Anatomical Compartments
Cartilage > Annulus Fibrosus
Bone > Central Neural Arch
Bone > Distal Rib
Bone > Dorsal Neural Arch
Somite > Dorsomedial Sclerotome
Cartilage > Intervertebral Disc
Bone > Lateral Neural Arch
Cartilage > Nucleus Pulposus
Bone > Proximal Rib
Bone > Rib
Bone > Spinous Process Of Verteb rae
Somite > Ventrolateral Sclerotome
Somite > Ventromedial Sclerotome
Bone > Vertebrae
Bone > Vertebral Body
Related Cells
Bone > Spinous Process Of Vertebrae > Prechondrocytic Mesenchymal
Cells
Bone > Dorsal Neural Arch > Prechondrocytic Mesenchymal Cells
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8/10/2019 Chapter 68=Congenital Malformations of The Skeletal System
http://slidepdf.com/reader/full/chapter-68congenital-malformations-of-the-skeletal-system 3/3
1/19/2015 Chapter 68. Congenital Malformations of The Skeletal System - Review of Medical Embryology Book - LifeMap Discovery
http://discovery.l ifemapsc.com/library/review-of-medical-embryology/chapter-68-congenital-malformations-of-the-skeletal-system
Bone > Central Neural Arch > Prechondrocytic Mesenchymal Cells
Bone > Proximal Rib > Prechondrocytic Mesenchymal Cells
Bone > Distal Rib > Prechondrocytic Mesenchymal Cells
Cartilage > Annulus Fibrosus > Prechondrocytic Mesenchymal Cells
Bone > Ve rtebral Body > Prechondrocytic Mesenchymal Cells
Bone > Lateral Neural Arch > Prechondrocytic Mesenchymal Cells
Somite > Cervical Mesenchymal Sclerotome > Sclerotome Cells
Somite > Lumbar Mesenchymal Sclerotome > Sclerotome Cells
Somite > Thoracic Mesenchymal Sclerotome > Sclerotome Cells
Somite > Sacral Mesenchymal Sclerotome > Sclerotome Cells
PREV NEXTTABLE OF CONTENT67. Appendicular Skeleton And Skull Development 69. Development Of The Limbs
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