Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Principles B - Summer '25 > Pediatric Surgery - Module 3 > Flashcards

Pediatric Surgery - Module 3 Flashcards

(89 cards)

Start Studying
1
Q

Define craniosynostosis and give its incidence.

A

Premature closure of one or more cranial sutures; occurs in roughly 1 in 3 000 live births.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What proportion of craniosynostosis cases are isolated versus syndromic?

A

≈ 80 % are isolated single‑suture cases; ≈ 20 % involve multiple sutures and are part of >400 described syndromes.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the 4 typical types of craniosynostosis

A
  • Unilateral coronal
  • Unilateral lamboid
  • Sagittal
  • Metopic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

List four surgical indications for craniofacial reconstruction in craniosynostosis.

A

Raised intracranial pressure, severe exophthalmos, obstructive sleep apnea, significant craniofacial deformity/psychosocial concerns.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Apert syndrome incidence and genetic mutation?

A

≈ 1 in 100 000 live births; activating FGFR2 mutation on chromosome 10.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Five classic craniofacial features of Apert syndrome.

A
  • Cloverleaf skull craniosynostosis
  • Hypertelorism - eyes are spaced too far apart
  • Proptosis - protrusion/bulging of 1 or both eyes.
  • Midface hypoplasia
  • Syndactyly of hands/feet.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is Apert syndrome classified as?

A

Branchial arch syndrome - afftects the first btrnachial or pharyngeal arch, the precursur to the maxilla and mandible

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Crouzon syndrome key differences from Apert.

A
  • Similar FGFR2 mutation and midface hypoplasia but NO hand/foot anomalies
  • Optic atrophy in up to 20 %.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Inheritance pattern split for Crouzon syndrome.

A

≈ 50 % sporadic mutations, ≈ 50 % autosomal‑dominant familial cases.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Cranofacial Reconstruction and Considerations

A
  • Anticipate a difficult a intubation
  • Positioning for long surgery
  • Arterial line
  • ICP
  • 2 large IVs
  • Eye protection
  • Type and cross
  • TXA
  • Prevent hypothermia
  • Foley for UOP monitoring
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Explain Hemifacial Microsomia (HFM). Also known as…

A
  • Otomandibular dysostosis (group of genetic disorders that affect the development of bones, particularly the formation of bone (ossification))
  • Second‑most‑common facial defect after cleft lip/palate; malformation of 1st/2nd branchial arches causing asymmetric mandibular and auricular hypoplasia.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What does the OMENS mnemonic classify in HFM?

A

O‑orbital distortion, M‑mandibular hypoplasia, E‑ear anomaly, N‑nerve involvement, S‑soft‑tissue deficiency.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Airway challenges typical in HFM.

A

Mid‑facial hypoplasia, asymmetric mouth opening, retrognathic mandible make laryngoscopy and mask fit difficult.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Goldenhar syndrome hallmark airway issue.

A

Bilateral mandibular hypoplasia leading to OSA and potential difficult intubation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Two systemic anomalies frequent in Goldenhar.

A
  • Vertebral anomalies (~40 %)
  • Congenital heart disease (~35 %)
  • Bilateral mandibular hypoplasia - predisposes to OSA (may be a difficult airway)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Treacher Collins syndrome characteristics.

A
  • Mandibular hypoplasia
  • Microstomia,
  • Cleft palate ±
  • Zygomatic hypoplasia
  • Colobomas (notched lower lids & sloping palebral fissures)
  • Microtia (underdeveloped external ear)
  • Choanal atresia (defect where the back of the nasal passage (choanae) is blocked, either by bone, soft tissue, or both, preventing airflow from the nose to the throat)
  • CV defects
  • Renal defects
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

How does Treacher Collins airway difficulty change with age?

A

Becomes more challenging as the child grows due to progressive mandibular discrepancy.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Recommended airway equipment for craniofacial syndromes (per slide).

A

Full difficult‑airway cart: fiberoptic scope, Glidescope, various LMAs; plan inhalational induction or IV with propofol/precedex plus topical lidocaine.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Pierre Robin sequence defining triad.

A
  • Micrognathia
  • Glossoptosis
  • Respiratory distress in first 48 h of life.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Initial airway approach for Pierre Robin.

A
  • Consider LMA for induction then fiberoptic intubation
  • Tongue‑lip suture may be needed for postoperative airway.
  • May have limited neck flexion d/t cervical anomalies
  • Cleft lip or palate may be present
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Why should cervical spine be assessed in Pierre Robin?

A

Associated anomalies may limit flexion/extension and affect positioning.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Difficult airway worsens with age in Pierre Robin. T/F?

A

False. It improves with age.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Incidence of cleft lip ± palate and demographic pattern. When does it begin.

A

≈ 1 in 600 births; more common in males and in Asian/Latin American populations.
- Begins in first trimester of pregnancy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Typical ages for primary cleft repairs.

A

Lip at 2–3 months; palate at 6–10 months; alveolar graft around 10 years.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Predictor of difficult airway in cleft palate repair that improves with age.
Micrognathia is an independent predictor but lessens as mandible grows.
26
Preferred endotracheal tube and fixation for cleft surgery.
Oral RAE tube secured midline to the chin.
27
Blade choice and approach to avoid palatal cleft.
Straight blade using right paraglossal approach.
28
Induction drug combo often used for cleft repair.
Fentanyl 1–2 µg kg⁻¹ plus propofol 2 mg kg⁻¹; maintain with volatile agent.
29
Medications to reduce swelling and emergence agitation in cleft cases.
Dexamethasone 0.5 mg kg⁻¹ and dexmedetomidine 0.3–0.5 µg kg⁻¹ h⁻¹ infusion.
30
Multimodal Analgesia for Cleft lip/palate
- IV tylenol 10-15 mg/kg or rectal tylenol 30-40 mg/kg - Block done by surgeon (infraorbitral and/or external nasal nerve/palatal/bilateral suprazygomatic maxillary)
31
Post‑extubation airway support frequently used after palatoplasty.
Surgeon‑placed nasal trumpets to maintain patency; awake extubation mandatory.
32
Down syndrome (Trisomy 21) live‑birth incidence.
≈ 1 in 800 live births.
33
Three airway features in Down syndrome relevant to anesthesia.
Macroglossia, short neck with atlanto‑axial instability, subglottic stenosis.
34
Cardiac anomaly prevalence in Down syndrome.
≈ 40–50 % have congenital heart disease (e.g., AV canal, VSD, TOF).
35
Why is bradycardia common on induction in Down syndrome?
- Enhanced vagal tone; atropine 0.01–0.02 mg kg⁻¹ or glycopyrrolate 0.01 mg kg⁻¹ should be ready. - Give before HR actually decreases to ensure prompt onset.
36
Describe subglottic stenosis implication for Down syndrome intubation.
May require 0.5–1.0 mm smaller ETT than age‑predicted size and increases risk of post‑extubation stridor.
37
Microtia and mandibular hypoplasia impact on airway in craniofacial syndromes.
Distorted landmarks and limited mouth opening complicate mask seal and laryngoscope blade insertion.
38
Syndrome with autosomal‑dominant FGFR2 mutation causing cloverleaf skull.
Apert syndrome.
39
Optic complication unique to Crouzon compared with Apert.
Optic nerve atrophy in up to 20 % of cases.
40
Explain why LMAs can be successful in Treacher Collins.
Despite difficult laryngoscopy, supraglottic placement often bypasses retrognathic jaw and provides stable airway.
41
OSA risk factor common to Goldenhar patients.
Bilateral mandibular hypoplasia narrows airway during sleep.
42
Describe glossoptosis.
Posterior displacement of the tongue base causing airway obstruction, central to Pierre Robin sequence.
43
Why might cleft palate patients need cricoid pressure during RSI?
Helps prevent gastric insufflation due to associated airway anomalies.
44
Risk of blood transfusion in primary cleft lip/palate repair?
Low; usually no transfusion required if pre‑op hematocrit > 30 %.
45
Drug of choice for emergence agitation reduction in cleft surgeries.
Intra‑operative dexmedetomidine infusion 0.3–0.5 µg kg⁻¹ h⁻¹.
46
Atlanto‑axial instability screening in Down syndrome.
Pre‑op cervical spine X‑ray if neurologic symptoms or before major head/neck manipulation.
47
Reason micrognathia airway improves with age in Pierre Robin.
Mandible catches up postnatally, reducing tongue prolapse.
48
Anesthetic plan for craniosynostosis repair blood loss.
Two large‑bore IVs, arterial line, type & cross; anticipate large blood loss and use cell saver.
49
Explain hypertelorism.
Increased distance between the orbits; prominent feature in Apert syndrome.
50
Describe proptosis and its relevance.
Anterior displacement of the globe; increases risk of corneal injury during anesthesia in Apert/Crouzon.
51
Key pre‑op cardio evaluation for Goldenhar.
Echocardiogram to detect congenital heart defects (present in ~35 %).
52
Why nasal trumpets helpful post cleft surgery.
Maintain airway patency and allow suctioning despite nasal edema.
53
Difficult airway statistic for bilateral vs unilateral clefts.
Overall 3–23 %, higher in bilateral clefts.
54
Role of dexamethasone in craniofacial reconstruction anesthesia.
Reduces swelling and PONV; typical dose 0.5 mg kg⁻¹ up to 8 mg.
55
Common mutation chromosome for Apert/Crouzon.
FGFR2 on chromosome 10.
56
OSA screening importance in craniofacial syndromes pre‑op.
Predicts peri‑op airway obstruction and influences postoperative monitoring location.
57
Describe choanal atresia and its syndrome association.
Obstruction of posterior nasal aperture; seen in Treacher Collins and can worsen airway obstruction.
58
Syndrome with hypertelorism and syndactyly.
Apert.
59
Definition of syndactyly.
Fusion of digits of hands or feet.
60
OSA relation to maxillary hypoplasia in Crouzon.
Midface deficiency narrows nasopharyngeal airway increasing OSA risk.
61
Surgical timing general for craniosynostosis.
Ideally before 12 months to allow brain growth.
62
Explain glossoptosis airway management technique.
Tongue‑lip adhesion or jaw distraction if severe.
63
Key postoperative complication in cleft repair requiring awake extubation.
Immediate upper airway obstruction due to edema and reduced pharyngeal tone.
64
Name at least two nerve blocks for cleft lip surgery.
Infraorbital nerve block, external nasal nerve block, suprazygomatic maxillary block.
65
Controlled ventilation vs spontaneous for cleft palate repair.
Controlled ventilation preferred for surgical exposure and airway stability.
66
Why video laryngoscopy recommended in craniofacial syndromes.
Improves glottic visualization in patients with midface and mandibular anomalies.
67
Describe the 'cloverleaf skull' deformity.
Trigonocephaly-like trilobar skull shape due to multiple synostoses in Apert.
68
Why is laryngospasm risk higher in craniofacial anomalies?
Upper‑airway structural abnormalities cause reactive airway and difficult suctioning.
69
Appropriate age for alveolar bone graft in cleft patients.
Around 10 years when canine root half‑developed.
70
Microtia implication for IV access monitoring.
May indicate vascular anomalies; pulse oximeter placement on ear may be difficult.
71
Dose of atropine for bradycardia prophylaxis in Down syndrome noted.
0.01–0.02 mg kg⁻¹ IV before HR drop.
72
Describe optic atrophy relevance.
Potential visual impairment; careful eye protection under anesthesia.
73
Incidence of craniosynostosis in males vs females.
More common in males.
74
Define hypertelorism.
Abnormally increased interpupillary distance.
75
Describe glossoptosis impact on mask ventilation.
Posterior tongue collapses airway causing obstruction during induction.
76
Why is a straight laryngoscope blade recommended for cleft palate?
Allows right paraglossal approach avoiding cleft gap.
77
Benefit of LMA during induction in Pierre Robin.
Provides patent airway while planning fiberoptic intubation.
78
Describe mandibular distraction surgery timeline.
Often performed in early infancy to relieve airway obstruction, then hardware removal.
79
Recommended IV and rectal acetaminophen doses for cleft surgery analgesia.
IV Tylenol 10–15 mg kg⁻¹ every 6 h; rectal Tylenol 30–40 mg kg⁻¹ single dose
80
Dexmedetomidine infusion parameters to curb emergence agitation after cleft repair.
Intra‑operative drip 0.3–0.5 µg kg⁻¹ h⁻¹
81
Post‑palatoplasty airway adjunct and extubation strategy.
Surgeon places nasal trumpets; awake extubation needed due to high obstruction risk; use arm restraints (“no‑no’s”) post‑op
82
Incidence and predictors of difficult airway in cleft lip/palate.
Overall 3–23 %; higher in bilateral clefts; micrognathia predicts difficulty but improves with age
83
Down‑syndrome ETT sizing caveat and stridor risk.
Subglottic stenosis often requires 0.5–1 mm smaller tube; raises post‑extubation stridor risk
84
Common systemic issues in Down syndrome relevant to anesthesia.
Duodenal atresia (~GI obstruction), congenital heart disease 40‑50 %, hypotonia, hypothyroidism
85
Goldenhar vertebral and cardiac anomaly prevalence.
≈ 40 % vertebral defects and ≈ 35 % congenital heart disease
86
Treacher‑Collins airway tip despite difficult laryngoscopy.
LMAs often seat well and provide stable airway even when direct view is difficult; airway worsens with age
87
Pierre Robin operative airway adjunct.
Tongue–lip (tongue suture) adhesion may be needed to prevent postoperative glossoptosis obstruction
88
OMENS acronym elements for Hemifacial Microsomia classification.
O = Orbit, M = Mandible, E = Ear, N = Nerve, S = Soft‑tissue deficit
89
Blood transfusion likelihood in primary cleft repairs.
Usually none required if pre‑op hematocrit > 30 % fileciteturn1file1

Principles B - Summer '25 (6 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions