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Iatrogenic (eg, after shunt placement for hydrocephalus)
Diagnosis, Workup, and Consultations
History
Abnormal head contour
Sleep disturbances
Regression or failure to meet developmental
milestones
Physical exam
Palpable ridge along synostotic sutures
Lack of movement along sutures with palpation
May have dysmorphic facial features or facial
asymmetry
Abnormal head circumference when compared with
age-predicted norms
Poorly defined, absent or bulging fontanelles
Evaluate for elevated intracranial pressure (ICP)
Approximately 10% of single suture synostosis and
40% of patients with multisuture synostosis have
elevated ICP.
Irritability, growth impairment, inconsolability, vomiting,
bulging fontanelles.
Fundoscopic examination for papilledema.
Requisite neurosurgical consultation in all confirmed
patients.
Imaging: CT scan
Routinely used in diagnosis.
Three-dimensional reformatting for preoperative
planning.
Evidence of elevated ICP may be manifested as
hydrocephalus or Lückenschädel (“copper beaten”)
skull.
Genetics evaluation.
Neuropsychological evaluation to determine baseline
cognitive functioning.
Speech and audiology assessment should be performed
to ensure ongoing language acquisition during
development.
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NONSYNDROMIC
CRANIOSYNOSTOSIS
Metopic Synostosis—Deformity: Trigonocephaly
Relatively uncommon: <10% of craniosynostosis
Keel-shaped skull with pointed forehead, frontal bossing,
bitemporal narrowing, hypotelorism, and recessed superior
orbital rims
Sagittal Synostosis—Deformity: Scaphocephaly
(Dolichocephaly)
*Most common: >50% of craniosynostoses
Male predominance: 4:1 male/female ratio
Sporadic with 2% genetic predisposition
Associated findings: increased AP length of skull, “boatlike”
appearance, decreased biparietal width, and frontal and
occipital bossing
Unilateral Coronal (Unicoronal) Synostosis—Deformity:
Anterior Plagiocephaly
Second most common: 20% of craniosynostosis
Ipsilateral frontal bone flattening, contralateral frontal
bossing, shortened AP dimension on affected side, anterior
displacement of ipsilateral ear, and deviation of nasal tip to
contralateral side
*Harlequin eye deformity
Lack of ipsilateral descent of greater wing of sphenoid
during development
Pathognomonic for unicoronal synostosis
Bilateral Coronal (Bicoronal) Synostosis—Deformity—
Brachycephaly
*Most commonly associated with syndromic
craniosynostosis (such as Crouzon and Apert
syndromes)
Frontal bossing, vertical elongation of frontal bones,
widening of anterior cranial base, shortened AP skull
dimension, occipital flattening, shallow orbits, and
hypertelorism
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Lambdoid Synostosis—Deformity: Posterior Plagiocephaly
Least common: <3% of craniosynostosis
May be unilateral or bilateral
Flattening of ipsilateral occiput, posterior/inferior
displacement of ipsilateral ear, and contralateral
occipitoparietal bossing
Multiple Suture Synostosis
Usually occurs as a feature of syndromic craniosynostosis.
Dysmorphic features depend on the pattern of involved
sutures.
Pansynostosis: fusion of all cranial sutures.
Nonsyndromic pansynostosis
Inadequate volume expansion secondary to
impaired brain growth.
Skull is normocephalic in contour but
microcephalic in volume.
Syndromic pansynostosis
Normal brain growth, which is constrained by the
fusion of all sutures
Kleeblattschädel deformity: clover leaf skull
Ballooning of cranial vertex and squamosal
sutures.
Immediate surgical decompression at birth to
avert neurologic compromise.
May require cerebrospinal fluid (CSF) shunt.
C-spine must be evaluated for additional
abnormalities.
DEFORMATIONAL PLAGIOCEPHALY
(POSITIONAL HEAD DEFORMITY, OR
POSITIONAL PLAGIOCEPHALY)
Results from external pressure applied to the pliable fetal or
infant skull
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Must be differentiated from lambdoid or coronal synostosis
(Fig. 16-2)
Figure 16-2 Distinguishing features of
deformational and synostotic
plagiocephaly.
Causes:
Supine sleeping position, which is recommended to
decrease the risk of sudden infant death syndrome. This is
the most common cause of positional plagiocephaly.
In utero compression.
Vertebral abnormalities.
Congenital muscular torticollis (often occurs with
deformational plagiocephaly).
Ocular torticollis: visual field deficits causing preferential
head positioning.
Treatment Specific to the Underlying Cause
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Supervised prone positioning (“tummy time”), physical
therapy, stretching and possible muscle release for
torticollis, head and neck rotation while feeding,
encouraging looking to affected side by positioning infant
Shaping helmets
Fitted to widest skull dimension.
Compensatory growth occurs due to external forces
applied by helmet.
Must be worn for >23 hours per day, for 2-3 months or
longer.
Less effective after 18 months of age; early helmeting
is much more effective.
Multiple helmet fittings required as cranial contour
improves.
SYNDROMIC CRANIOSYNOSTOSIS
Apert Syndrome (Fig. 16-3A)
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Figure 16-3 A. Apert syndrome. B.
Crouzon syndrome. C. Treacher Collins
syndrome. D. Goldenhar-Gorlin syndrome.
(D, from Gold DH, Weingeist TA. Color
Atlas of the Eye in Systemic Disease.
Lippincott Williams & Wilkins; 2001. Figure
122.1.)
Genetics
Autosomal dominant inheritance but vast majority of
cases represent sporadic mutations
*FGFR2 mutation (chromosome 10)
Craniofacial features
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