common carotid, most of internal carotid IX

Chapter 50 - Congenital Malformations H/N Flashcards by Ben Walters

Lymphatic malformations: tumors? do they expand?

not true tumors - grow with patient

can rapidly expand with infection, bleeding, trauma

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branchial cleft: deep to which arch? superficial to which?

anomalies track deep to structures of own arch, superficial to structures of next one

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most common congenital neck mass

thyroglossal duct cyst

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how does thyroglossal duct cyst form?

3 wk gestation thyroid gland forms from diverticulum at tongue (foramen cecum)
descends to fuse with 4th-5th pouch components
5-8wk: tract formed by descent obliterates, forming foramen cecum proximally and pyramidal lobe thyroid distally
Incomplete obliteration –> TDC

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Classic TDC PE sign

elevates with tongue protrusion

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Critical preop imaging prior to TDC removal

U/S to r/o ectopic thyroid

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DDx and defining characteristics of midline CONGENITAL neck masses

TDC - elevate with swallow
Dermoid - submental, move with skin
Teratoma - firm, CT with calcification, rare, larger/more sx than dermoid
Plunging ranula - midline, cystic, extends to FOM

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DDx and defining characteristics of lateral CONGENITAL neck masses

branchial cleft cyst - deep to ant border SCM, fluctuant
lymphangioma - soft, compressible, transilluminate
external laryngocele - air filled, protrude through thyrohyoid, hoarse, cough
SCM tumor - firm, painless, discrete, fusiform mass

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Recurrence rate of TDC after Sistrunk

2.6-5%

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Can TDC’s harbor malignancy?

Yes - well differentiated thyroid CA

Sistrunk removal offers excellent cure rate

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Describe sistrunk procedure objectives

remove TDC tract connecting it to the foramen cecum

remove central portion of hyoid

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Dermoid vs teratoma

Dermoid- ectoderm/mesoderm only, from entrapped epithelium along lines of embryonic fusion, enlarge over time, cheesy contents upon excision, fill with sebaceous material

Teratoma- all 3 germ layers, larger/typically more Sx, dx by prenatal u/s often, 30% associated with polyhydramnios (esophageal obstruction)

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Branchial cleft vs arch vs pouch

At 4wk geswtation, there are 4 pairs of arches, and two rudimentary ones
External lining ectoderm, internal endoderm, meso between
arches separated by pouches internally and clefts externally
clefts and pouches gradually replaced with mesenchyme
when clefts and pouches persist –> branchial anomaly

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3 types of branchial anomaly

remember this is when clefts/pouches persist
Cyst- remnant with no openings, squamous lining
Sinus- iexternal opening (often drain to skin), often lined with ciliated columnar
Fistula- internal (aerodigestive tract) and external (skin) opening

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Type 1 BA

1% (rare)
between EAC and submandibular area
lateral to VII
duplication of EAC
can terminate in EAC or middle ear
Do not contain mesoderm

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Type 2 BA

95%
MEDIAL to VII and has mesoderm
Anterior border SCM
Between ICA and ECA
Terminate in tonsillar fossa (but can terminate in EAC/middle ear)

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Type 3/4 BA

present lower than type 2 but also along anterior SCM border
terminate ipsilateral pyriform sinus
tract of type 4 loops under subclavian (R) or under aortic arch (L)

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Arch 1 artery, nerve

terminal br maxillary

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Arch 1 skeletal elements

incus, malleus, Meckel’s cartilage, upper portion auricle, maxilla, zygomatic, squamous portion of T bone, mandible

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Arch 1 mm

mastication, mylo, anterior dig, TT, TVP

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Arch 2 arteries, n

stapedius (embryologic), corticotympanic (adult)

VII

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Arch 2 skeletal elements

stapes, styloid, stylohyoid ligament, lesser horns and upper rim hyoid, lower auricle

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Arch 2 mm

facial expression, posterior dig, stylohyoid, stapedius

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Arch 3 a, n

common carotid, most of internal carotid

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Arch 3 skeletal

lower rim, greater horn hyoid

Arch 3 mm

stylopharyngeus

Arch 4 a/n

aortic arch, R subclavian original sprouts pulm aa SLN

Arch 4 skeletal

laryngeal cartilages

Arch 4 mm

pharynx constrictors, cricothyroid, levator veli palatini

Arch 6 a/n

ductus arteriosus, roots of definitive pulmonary aa | RLN

Arch 6 skeletal

laryngeal cartilage

Arch 6 mm

intrinsic mm larynx

Pseudotumor of infancy (fibromatosis coli)

firm rounded mass in SCM presents 2-3 wk after birth dense fibrous tissue, no striated muscle birth trauma, muscle ischemia, intrauterine position Tx: conservative, PT, >80% resolve by 1y/o

Midline nasal mass: dermoid vs glioma vs encephalocele

Dermoid: most common, noncompressible, glabella to columella, midline pit with hair. Surgery Glioma: portion of brain tissue isolated after suture closes, may have fibrous stalk maintaining CNS connection, but NO HERNIATED DURA Encephalocele: meninges and brain matter through SB defect, illuminates, enlarge with strain/cry (furstenberg sign)

Choanal atresia: incidence, syndromes, dx

1:8000 live births Usually unilateral right, F>M Choana is more often membranous than bony CHARGE, Crouzon, Treacher-Collins 5-6 French catheter cannot pass into nasopharynx at birth

Presentation of laryngomalacia

few to no sx at birth | gradually develop high pitch inspiratory stridor first few wk of life, usually gone by 12mo

Classic endoscopic findings of laryngomalacia

omega shaped epiglottis | foreshortened aryepiglottic folds

Which other condition is significantly associated with laryngomalacia

LPR | So acid-suppression is first line in non-surgical tx

Presentation of u/l VC paralysis vs b/l

weak cry, breathy cry, poor feeding, aspiration | b/l: biphasic stridor at birth

Causes of VC immobility

``` birth trauma hydrocephalus spina bifida CP Chiari ```

Congenital subglottic stenosis: definition, two types, presentation

subglottis <4mm term infant, <3mm premie only congenital if prior to any intubation membranous (more common, less severe) or cartilagenous first few mo biphasic/inspiratory stridor, recurrent croup

Tx of congenital subglottic stenosis

manage croup, anterior cricoid split, trach

Hemangioma subglottis: presentation, course

3-4 wk of life, inspiratory/biphasic stridor, harsh cough, abnormal cry, FTT 50% have associated congenital hemangiomas Spontaneous regression by 5 yo

Tx of subglottic hemangioma

BB, cryotherapy, sclerotherapy, steroids, open resection, laser ablation, trach

Presentation of laryngotracheal cleft

cyanosis, hoarse cry, cough, choke, stridor, aspirate, pneumonia

4 types of laryngeal cleft

1- absence of interarytenoid muscle, cleft above VC 2- into upper cricoid 3- involves entire cricoid, poss to cervical trachea 4- extend to thoracic trachea

5 types of tracheoesophageal anomalies

``` EA w/ distal TEF (85%) EA only (8%) TEF w/o atresia - H type (5%) EA w/ proximal TEF (1%) EA w/ prox & dist TEF (0.5-1%) ```

Presentation of TE anomalies

EA: copious oral secretions, cough/choke/respiratory distress with feeds first few d life, cannot pass NG tube H-type: may present later, chronic feeding diff, recurrent respiratory distress/pneumonia, Dx barium esophagram/endoscopy

Causes of intrinsic tracheal malformations

tracheal agenesis (die in utero), congenital tracheal stenosis (may have complete tracheal rings), tracheomalacia

Causes of extrinsic tracheal malformations

vascular (innominate, double arch, right arch w/ persistent ductus or ligamentum arteriosum), neck/mediastinal mass, thyromegaly

Lop (cup)ear

most frequent ext auricular deformity upper helix folded, wrinkled or tight, middle helix large and 90 deg from skin Tx mold fold of superior crus, early neonatal

Stahl's ear

abnormal fold in scapha | Tx press out this area

Protrudring ear

third most common ext auricular deformity tx fix posterior helical rim to posterior retruauricular region with surgical tape; or use dental wax to mold ear in addition to taping

Syndromes involving microtia

Treacher Collins | Goldenhar

Types of microtia

I- mild deformities, no need for additional skin/cartilage to reconstruct II- some structures recognizable III- no recognizable landmarks

Management of type III microtia

``` 2-4 stages of surgery Stage 1- cartilage implant (rib) 2- 2-3 mo later, transfer lobule 3- elevate cartilage graft, postauricular skin grafting 4- tragus ```

Optimal timing of microtia repair

6-8 yo

When should you place a BAHA when they have bilateral HL

Prior to 12 mo old

Protruding ear measurement

mastoid to ear angle of 40 degrees or distance of 20mm

Hemangioma vs vascular malformation

Hem: rarely present at birth, rapid postnatal proliferation then slow involution 10% incidence, most common neonatal tumor increased capillary tubule formation VM: always present at birth, abnormal morphogenesis of vascular channels, do not proliferate or involute, may grow larger with existing cell hypertrophy/fill cystic space with trauma/infection/hormones

Low flow vs high flow VMs

Low: capillary, venous, lymphatic, combined High: arterial, AVM

Klippel Trenaunay syndrome

cutaneous capillary malformation underlying venous/lymphatic malformation associated with skeletal overgrowth of limb

Sturge Weber

facial capillary malformation (port wine stain) in CN V, think about leptomeningeal VM on that side