Pediatric General Surgery Part 4 Flashcards
What is craniosynostosis?
Congenital anomaly in which one or more cranial sutures close prematurely
Name the type of Craniosynostosis
Unilateral Coronal Synostosis
Name the type of Craniosynostosis
Unilateral Lambdoid Synostosis
Name the type of Craniosynostosis
Sagittal Synostosis
Name the type of Craniosynostosis
Metopic Synostosis
Which gender is more likely to have Craniosynostosis
Male
What is Apert Syndrome
- Also known as acrocephalosyndactyly, is a rare genetic disorder that causes malformations of the skull, face, hands, and feet
- Sporadic mutation/can occur w/ autosomal dominance FGFR2 gene on chromosome 10
What are the physical features of someone with Apert’s Syndrome?
- “Cloverleaf Skull” Craniosynostosis
- Hypertelorism (unusually long distance b/w the eyes)
- Proptosis (protruding eyeballs)
- Midface hypoplasia → Increase ICP and OSA
- Syndactyly - webbed fingers/toes
Which skull sutures are prematurely closed with Apert’s Syndrome?
Bicoronal Sutures
What are the physical features of someone with Crouzon Syndrome?
- Similar to Apert w/ differing ophthalmic defects
- Midface hypoplasia
- Severe Proptosis
- Craniosynostosis
- No hand/foot deformities
Up to _____% of patients with Crouzon Syndrome will develop optic atrophy.
20%
What is midface hypoplasia?
Midface hypoplasia is a developmental anomaly that causes the upper jaw, cheekbones, and eye sockets to grow more slowly than the rest of the face.
Indications for Craniosynostosis Surgery
- Increase ICP
- Severe exophthalmos
- OSA
- Craniofacial deformity
- Psychosocial reasons
Anesthesia Considerations for Craniofacial Reconstruction
- Airway management, difficult intubation/mask ventilation
- Eye protection
- ↑ ICP (avoid ketamine, blunt SNS stimulation)
- Type/Cross
- A-line
- TXA
How is a patient positioned for a craniosynostosis surgery?
- Prone, Swan Dive position
What is another name for Hemifacial Microsomia?
Otomandibular Dysostosis
What is the most common facial defect in pediatrics?
Cleft lip and cleft palate
Hemifacial Microsomia is the 2nd most common
What acronym is used to classify Hemifacial Microsomia?
OMENS
Orbital distortion
Mandibular hypoplasia
Ear anomaly
Nerve involvement
Soft tissue deficiency
Hemifacial microsomia is a result of malformation of this brachial arch.
1st and 2nd brachial arches
Which brachial arch do Apert syndrome and Crouzon syndrome affect?
1st brachial arch (pharyngeal arch)
What are defects that may be included with Hemifacial Microsomia?
- Mandibular hypoplasia
- Temporomandibular joint dysostosis
- Cleft palate
- Auricular, ophthalmologic, facial nerve defects
Difficulty intubating Hemifacial Microsomia patients is related to these factors.
- Mid-facial hypoplasia
- Asymmetry of mouth opening
- Retrognathic mandible (recessed jaw)
What is the most common form of Hemifacial Microsomia?
Goldenhar syndrome
How many percent of patients with Goldenhar syndrome will have vertebral anomalies?
How many percent of patients with Goldenhar syndrome will have CHD?
- 40% will have vertebral anomalies
- 35% will have congenital heart defect
