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
Bilateral mandibular hypoplasia predisposes patients to _______ and can be a difficult airway.
OSA
Signs and symptoms of Treacher Collins Syndrome
- Mandibular hypoplasia
- Microstomia
- Cleft palate
- Colobomas of the eye, notched lower lids, sloping palpebral fissures
- Hypoplastic zygomatic arches
- Microtia
- Choanal atresia
- Cardiovascular defects
- Renal defects
Hemifacial Microsomia, Goldenhar, and Treacher Collins Syndrome Anesthetic Considerations
- Upper airway obstruction during induction and emergence
- Difficult airway cart, ready to go
- Inhalational induction or IV induction in older children (precedex or propofol with topical lidocaine for airway)
- LMAs can be used successfully
Treacher Collins Syndrome is associated with a difficult airway that increases with ______.
Age
What is the triad of symptoms involved with Pierre Robin syndrome?
- Micrognathia
- Glossoptosis (caudally displaced tongue)
- Respiratory Distress in the first 48 hours of life
With Pierre Robin, what airway device may be used for induction and intubation?
Consider LMA for induction, then nasal fiberoptic intubation
Why are tongue sutures placed in Pierre Robin patients for retraction?
Caudally displaced down can cause respiratory distress
Cervical spine anomalies in Pierre Robin patients may limit _______ and ___________.
Flexion, Extension
With Pierre Robin pt, difficult airway improves with _______.
Age
Easier to intubate the older the patient gets.
