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Mar: Sem 6 - Adv. Principles B > Pediatric General Surgery Part 4 > Flashcards

Pediatric General Surgery Part 4 Flashcards

1
Q

What is craniosynostosis?

A

Congenital anomaly in which one or more cranial sutures close prematurely

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2
Q

Name the type of Craniosynostosis

A

Unilateral Coronal Synostosis

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3
Q

Name the type of Craniosynostosis

A

Unilateral Lambdoid Synostosis

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4
Q

Name the type of Craniosynostosis

A

Sagittal Synostosis

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5
Q

Name the type of Craniosynostosis

A

Metopic Synostosis

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6
Q

Which gender is more likely to have Craniosynostosis

A

Male

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7
Q

What is Apert Syndrome

A
  • 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
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8
Q

What are the physical features of someone with Apert’s Syndrome?

A
  • “Cloverleaf Skull” Craniosynostosis
  • Hypertelorism (unusually long distance b/w the eyes)
  • Proptosis (protruding eyeballs)
  • Midface hypoplasia → Increase ICP and OSA
  • Syndactyly - webbed fingers/toes
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9
Q

Which skull sutures are prematurely closed with Apert’s Syndrome?

A

Bicoronal Sutures

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10
Q

What are the physical features of someone with Crouzon Syndrome?

A
  • Similar to Apert w/ differing ophthalmic defects
  • Midface hypoplasia
  • Severe Proptosis
  • Craniosynostosis
  • No hand/foot deformities
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11
Q

Up to _____% of patients with Crouzon Syndrome will develop optic atrophy.

A

20%

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12
Q

What is midface hypoplasia?

A

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.

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13
Q

Indications for Craniosynostosis Surgery

A
  • Increase ICP
  • Severe exophthalmos
  • OSA
  • Craniofacial deformity
  • Psychosocial reasons
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14
Q

Anesthesia Considerations for Craniofacial Reconstruction

A
  • Airway management, difficult intubation/mask ventilation
  • Eye protection
  • ↑ ICP (avoid ketamine, blunt SNS stimulation)
  • Type/Cross
  • A-line
  • TXA
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15
Q

How is a patient positioned for a craniosynostosis surgery?

A
  • Prone, Swan Dive position
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16
Q

What is another name for Hemifacial Microsomia?

A

Otomandibular Dysostosis

17
Q

What is the most common facial defect in pediatrics?

A

Cleft lip and cleft palate

Hemifacial Microsomia is the 2nd most common

18
Q

What acronym is used to classify Hemifacial Microsomia?

A

OMENS

Orbital distortion
Mandibular hypoplasia
Ear anomaly
Nerve involvement
Soft tissue deficiency

19
Q

Hemifacial microsomia is a result of malformation of this brachial arch.

A

1st and 2nd brachial arches

20
Q

Which brachial arch do Apert syndrome and Crouzon syndrome affect?

A

1st brachial arch (pharyngeal arch)

21
Q

What are defects that may be included with Hemifacial Microsomia?

A
  • Mandibular hypoplasia
  • Temporomandibular joint dysostosis
  • Cleft palate
  • Auricular, ophthalmologic, facial nerve defects
22
Q

Difficulty intubating Hemifacial Microsomia patients is related to these factors.

A
  • Mid-facial hypoplasia
  • Asymmetry of mouth opening
  • Retrognathic mandible (recessed jaw)
23
Q

What is the most common form of Hemifacial Microsomia?

A

Goldenhar syndrome

24
Q

How many percent of patients with Goldenhar syndrome will have vertebral anomalies?

How many percent of patients with Goldenhar syndrome will have CHD?

A
  • 40% will have vertebral anomalies
  • 35% will have congenital heart defect
25
Q

Bilateral mandibular hypoplasia predisposes patients to _______ and can be a difficult airway.

A

OSA

26
Q

Signs and symptoms of Treacher Collins Syndrome

A
  • 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
27
Q

Hemifacial Microsomia, Goldenhar, and Treacher Collins Syndrome Anesthetic Considerations

A
  • 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
28
Q

Treacher Collins Syndrome is associated with a difficult airway that increases with ______.

A

Age

29
Q

What is the triad of symptoms involved with Pierre Robin syndrome?

A
  • Micrognathia
  • Glossoptosis (caudally displaced tongue)
  • Respiratory Distress in the first 48 hours of life
30
Q

With Pierre Robin, what airway device may be used for induction and intubation?

A

Consider LMA for induction, then nasal fiberoptic intubation

31
Q

Why are tongue sutures placed in Pierre Robin patients for retraction?

A

Caudally displaced down can cause respiratory distress

32
Q

Cervical spine anomalies in Pierre Robin patients may limit _______ and ___________.

A

Flexion, Extension

33
Q

With Pierre Robin pt, difficult airway improves with _______.

A

Age

Easier to intubate the older the patient gets.

Mar: Sem 6 - Adv. Principles B (14 decks)

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