Craniosynostosis Flashcards
- Features demonstrated in the picture below are most likely the result of:
a. Anterior plagiocephaly
b. Deformational posterior plagiocephaly
c. Kleeblattschadel deformity
d. Oxycephaly
e. Turricephaly
c. Kleeblattschadel deformity
The picture demonstrates a newborn infant with cloverleaf skull deformity-frontal towering, bitemporal expansion, bilateral supraorbital recession (with proptosis), hypertelorism, and midface hypoplasia. One should also examine for broad great toe/ thumb (Pfeiffer syndrome) and syndactyly (Apert’s syndrome). It is caused by premature closure of sagittal, coronal, and lambdoid sutures and can occur in any severe craniosynostosis.
What is the most likely craniosynostosis depicted in this image?
a. Bilateral Coronal synostosis
b. Cloverleaf (Kleeblattschadel)
c. Lambdoid synostosis
d. Metopic synostosis
e. Sagittal synostosis
d. Metopic synostosis
Metopic synostosis. Features demon- strated are trigonocephaly, significant ridg- ing over the metopic suture, supraorbital recession and hypotelorism (see CT below). Due to reduced anterior cranial volume resulting in back pressure into the posterior fossa, there is an association with Type 1 Chiari malformations (30%). It is also associ- ated with frontal dysmorphology-corpus cal- losum dysgenesis, holoproscencephaly.
Which one of the following most likely to cause the appearances shown below?
a. Bicoronal synostosis
b. Crouzon’s syndrome
c. Metopic synostosis
d. Pierre-Robin sequence
e. Sagittal synostosis
e. Sagittal synostosis
Given the clinical features demonstrated in this 6-month-old child which one of the following is most likely:
a. Right anterior plagiocephaly due to left coronal synostosis
b. Left anterior plagiocephaly due to left coronal synostosis
c. Right anterior plagiocephaly due to right coronal synostosis
d. Left anterior plagiocephaly due to right coronal synostosis
e. Left anterior plagiocephaly due to bilateral coronal synostosis
f. Right anterior plagiocephaly due to bilateral coronal synostosis
b. Left anterior plagiocephaly due to left coronal synostosis
Left anterior plagiocephaly due to left cor- onal synostosis. Features seen are significant left supraorbital retrusion, left forehead flat- tening, and compensatory right frontal bos- sing. Other features to look for in unicoronal synostosis are ipsilateral perisutural ridging, ipsilateral nasal root displacement, anterior displacement of ipsilateral ear, contralateral chin deviation, and the pathognomonic feature of ipsilateral orbital elevation (harlequin sign). Strabismus is common (50-60%) due to mechanical effect on superior oblique, and anterior plagiocephaly is commoner on the right side (3:2).
Features demonstrated in the bird’s eye diagram below are most consistent with:
a. Right posterior plagiocephaly due to lambdoid synostosis
b. Left posterior plagiocephaly due to lambdoid synostosis
c. Right posterior plagiocephaly due to bilateral lambdoid synostosis
d. Right posterior deformation alplagiocephaly
e. Left posterior deformational plagiocephaly
f. Left posterior plagiocephaly due to bilateral lambdoid synostosis
d. Right posterior deformation alplagiocephaly
Right posterior deformational plagioce- phaly. Posterior deformational plagiocephaly is characterized by a parallelogram shaped head, anterior displacement of ipsilateral ear, and ipsilateral frontal bossing. In con- trast, unilateral lambdoid synostosis is marked by a trapezoid shaped skull, posterior displacement of ipsilateral ear and contralat- eral occipital bossing.
Craniosynostosis occurs in approximately how many live births?
a. 1/1000 to 1/1500
b. 1/1500 to 1/2000
c. 1/2000 to 1/2500
d. 1/2500 to 1/3000
e. 1/3000 to 1/3500
c. 1/2000 to 1/2500
1/2000 to 1/2500
Frequency of different types of single suture synostoses is approximately:
a. Lambdoid 18%, Metopic 25%, Sagittal 60%, Unicoronal 3%
b. Lambdoid 18%, Metopic 60%, Sagittal 25%, Unicoronal 3%
c. Lambdoid 25%, Metopic 18%, Sagittal 3%, Unicoronal 60%
d. Lambdoid 3%, Metopic 25%, Sagittal 60%, Unicoronal 18%
e. Lambdoid 60%, Metopic 18%, Sagittal 3%, Unicoronal 25%
d. Lambdoid 3%, Metopic 25%, Sagittal 60%, Unicoronal 18%
Lambdoid 3%, Metopic 25%, Sagittal 60%, Unicoronal 18%. Sagittal (3.5-7:1) and metopic (75%) synostosis is commoner in boys, while unicoronal is commoner in girls (3:2). True lambdoid synostosis is rare, and must be distinguished from posterior deformational plagiocephaly (positional molding) where there is occipital flattening without suture fusion, possibly due to supine sleeping position instituted to reduce sudden infant death syndrome.
Virchow’s law states
a. Skull growth is arrested in the direction perpendicular to the fused suture and reduced at the sites of unaffected sutures
b. Skull growth is arrested in the direction parallel to the fused suture and expanded at the sites of unaffected sutures
c. Skull growth is arrested in the direction perpendicular to the fused suture and expanded at the sites of affected sutures
d. Skull growth is arrested in the direction parallel to the fused suture and expanded at the sites of affected sutures
e. Skull growth is arrested in the direction perpendicular to the fused suture and expanded at the sites of unaffected sutures
e. Skull growth is arrested in the direction perpendicular to the fused suture and expanded at the sites of unaffected sutures
Skull growth is arrested in the direction perpendicular to the fused suture and expanded at the sites of unaffected sutures, leading to characteristic calvarial deformations
Features of raised intracranial pressure on plain radiograph show:
a. Bilateral Harlequin sign
b. Copper beating
c. Frontal bossing
d. Temporoparietal bossing
e. Open fontanelle
b. Copper beating
Copper beating. The thinned out skull is usually an indicator of chronic hydrocephalus.
Fronto-orbital advancement surgery is most likely considered in which one of the following scenarios
a. Apert’s syndrome
b. Fibrous dysplasia
c. Paget’s disease
d. Sagittal synostosis
a. Apert’s syndrome
Apert’s syndrome
Which one of the following most likely to cause the appearances shown below?
a. Lambdoid synostosis
b. Metopic synostosis
c. Positional plagiocephaly
d. Sagittal synostosis
e. Unicoronal suture synostosis
e. Unicoronal suture synostosis
Unicoronal suture synostosis. The skull radiograph exhibits the classic ‘harlequin’ sign.
Which one of the following most likely to cause the appearances shown below?
a. Aqueduct stenosis
b. Fibrous dysplasia
c. Hyperparathyroidism
d. Langerhan’s histiocytosis
e. Multiple myeloma
a. Aqueduct stenosis
Aqueduct stenosis. The appearances above are termed “copper beaten skull” asso- ciated with raised intracranial pressure in children. Common causes are craniosynosto- sis, obstructive hydrocephalus, intracranial masses and hypophosphatasia.
Which one of the following most likely to cause the appearances shown below?
a. Hyperparathyroidism
b. Leukemia
c. Neurofibromatosis (NF)
d. Pfeiffer’s syndrome
e. Saethre-Chotzen
d. Pfeiffer’s syndrome
Pfeiffer’s syndrome (Cloverleaf skull).
Which one of the following most likely to cause the appearances shown below?
a. Cerebral abscess
b. Fibrous dysplasia
c. Multiple myeloma
d. Neurofibromatosis type 1
e. Raised intracranial pressure
d. Neurofibromatosis type 1
Angiomas and neurofibromas of the scalp may affect the underlying skull and cause deformities, bony defects, and regional hyper- ostoses. Plain film findings of NF include lytic defect in the lambdoid suture, absence of the orbital roof and floor, elevated lesser sphenoid wing, enlarged middle cranial fossa, enlarged cranial nerve foramina, unilateral orbital enlargement, and J-shaped sella turcica.
Which one of the following most likely to cause the appearances shown below?
a. Arachnoid cyst
b. Epidermoid
c. Fibrous dysplasia
d. Leukemia
e. Raised intracranial pressure
b. Epidermoid
A small oval defect in the parietal bone with a sharply defined sclerotic border. Epidermoids are ectodermal rests or inclusions that may be located in the scalp, in the diploic spaces, or between the internal surface of the inner table and the dura. Epidermoids are usually benign and grow slowly. If they protrude into the cranial cavity, they may be the source of cerebral symp- toms. When epidermoids grow within the bone or impinge on it, they produce local destruction of bone that appears radiographically as a sharply demarcated lucency surrounded by a smooth scle- rotic margin, which sometimes may be scalloped. The margin is due to flaring of the edge of the bone into a marginal ridge. Most cases are found in children younger than 3 years. The lesions usu- ally disappear within a few years of discovery.
Which one of the following most likely to cause the appearances shown below?
a. Dermoid
b. Epidermoid
c. Raised intracranial pressure
d. Saethre-Chotzen syndrome
e. Tuberculosis
e. Tuberculosis
Multiple destructive tuberculosis foci in the calvaria of a 3-year-old girl. Tuberculosis of the calvaria usually manifests as a painless subgaleal scalp swelling with a discharging sinus. The lesions are usually either small, circumscribed, punched-out lytic areas or spreading, circumscribed sclerotic areas; or a combination of the two. The differential diagnosis of multiple lytic lesions in the skull—TB, multiple myeloma, Langerhans cell histiocytosis.
