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Superficial veins - EJV - from retromandibular v. and posterior auricular v. Passes over SCM to
pierce fascia over post. triangle and drain into subclavian v.
- AJV - from submand. and submental region
Veins are firmly attached to Cx fascia
Superficial nerves. - sensory, from Cx plexus, appear under post. belly of SCM 1/2 way down.
Greater auricular. - ascends to ear over SCM
Lesser occipital - ascends up the post. border of SCM.
Transverse cervical- C2 and 3. Passes forward.
Supraclavicular - C3 and 4.
.
DDx
Congenital Inflammatory Neoplasms Miscellaneous
Bacterial or viral Granulomatous Benign Malignant
Thyroglossal cyst Cx adenitis TB Haemangioma Primary SM fibrosis
Branchial cleft cyst Abscesses MAIS lymphangioma Secondary Normal structure
Foregut cyst Cat-scratch fever Sarcoidosis teratoma -neuroblastoma ranula
Dermoid cyst IM Actinomycosis Sal gland - rhabdo laryngocoele
Bronchogenic cyst Toxoplasmosis thyroid Lymphoma
brucellosis Paraganglioma
Schwannoma
Neurofibroma
CONGENITAL
Embryology:
During 4th to 8th week, 5 pairs (and 1 rudimentary pair) of well developed arches connect paired dorsal
and ventral aortas in lateral cervical area. Each consist of a mesodermal cartilagenous centre, an intervening
ectodermal lined cleft, an endodermal lined pouch internally, artery, nerve.
Incidence:
= all neck masses in kids. ~ 1/3 branchial, 1/3 thyroglossal, 20% dermoids
90% 2nd cleft, 74% cysts, 25% fistulas, 1% skin tags / cartilage
8% 1st, 2% 3rd.
Branchial cysts:
palpable anterior to the middle third of sternomastoid, upper 1/3 - lower 2/3 junction.
and may become more prominant after a respiratory tract infection. Probably arise from remnants of the
cervical sinus. They are not usually apparent during childhood.
Contain cholesterol crystals, lined by epithelium with much lymphoid tissue.
DDx :- LN
- CB tumour, Haemangioma, Neurofibroma, Cystic hygroma
Treatment is elective excision, b/c of infection risk. If infected, first control with antis if possible.
When excising, keep close to the cyst as X, XI and XII are often in the proximity. may be very close to IJV
or Carotid artery
Some authors (including those in Melbourne) have used this and the obvious lymphoid tissue in the wall as
evidence against these being branchial remnants at all and have suggested that they are derived from the
cystic degeneration in lymph nodes.
Sistrunk procedure.
Excision of cyst, central hyoid and duct up to the foramen caecum.
Extended neck, tv incision over cyst (excise sinus if present), platysma reflected, cyst dissected out.
Dissect track up to hyoid, excise central 1cm, continue dissection of a core of tissue up to f caecum
Assistant pushes f caecum downward to facilitate dissection.
Ligate duct at base of tongue by excising a conical area of tongue muscle. Close defect 3/0 vicryl
Leave hyoid unapproximated, close in layers over drain.
Recurrence rate 5-7%, even with hyoid excision. Risk - younger, skin involvement, cyst lobulation, rupture
of cyst at op, failure of hyoid removal and core above.
< 1% develop carcinoma, 80% papillary
CRANIOFACIAL DERMOIDS
= epidermis + appendages in cyst wall.
The soft tissues of the face are formed by the convergence of three facial processes (frontal, maxillary and
mandibular). lines of fusion where islands of ectodermal tissue may become submerged, later to secrete
sebaceous material and present as obvious cystic swellings known as dermoids.
commonest site is upper lateral part of the forehead (an external angular dermoid), other sites include
-upper medial part of the eye, or along the midline of the face and neck - nasal bridge, submental,
superficial to sternum. 23% occur in the floor of the mouth..
Rarely, communication through the calvarium with two dermoid elements occuring on either side of the
bone to resemble a dumb-bell tumour. Any suspicion that a dermoid may be fixed to the bone should
prompt an x-ray examination or even CT.
Malignant degeneration of dermoid cysts is possible but extremely rare.
difference between a dermoid cyst and a epidermoid cyst - epidermoid cyst is lined by epithelium without
any other of the skin adnexae (glandular structures etc).
congenital sinus on the anterior aspect of the helix, passes anteriorly and inferiorly through the skin to end
in a group of preauricular cysts. arise from imperfect fusion of the six fetal tubercles which form the pinna.
Symptoms are rare in infants. Infection commonly occurs in older children and is difficult to treat. If
untreated, recurrent infection results in a preauricular ulcer. The lesion may be unilateral or bilateral and
may be familial. Not associated with a syndromic hearing loss except in branchio-otorenal syndrome.
Most common in Chinese and least in Scots, it is an autosomal dominant condition.
only treat symptomatic ones.
Complete excision is necessary to effect a cure. Operative: raise a flap as an inverted L, tract and cysts
should come up with flap. After abscess drainage some of them scar up so much that they do not require
excision (Vipul). Martin operates using methylene blue and an elliptical incision.
Accessory auricles:
Situated on the line of fusion of the maxillary and mandibular processes of the first branchial arch and are
managed by surgical excision.
INFLAMMATORY
Acute infection.
Mostly viral. Bact = Staph, esp in infants, Group A Strep or both > Group B Strep, haemophilus. If cellulitis
/ toxic / infant. Failure to respond in 24h US / FNA. Either aspiration or formal drainage.
If neglected, can extend into lateral pharyngeal space
"Ludwig angina" - extension of a dental infection into floor of mouth. Pain with tongue movement.
Can cause airway obstruction.
Chronic infection.
MAIS :- not contagious, portal of entry = oropharynx, 1-5yrs, generally unilateral submental or
submandibular. Nodes firm and rubbery, non-tender. May have fistula after spontaneous discharge of collar
stud abscess. Mantoux ve or intermediate. AFBs in pus. Not gen responsive to Abs. Trial with
roxithromycin suggest may be as good as Sx. Sx excise affected skin as ellipse and affected nodes.
Consider nerve stimulator.
Cat Scratch disease: - Bartonella henselae = gram neg bacillus. Entry via skin, papule at site in 3-5d,
draining nodes up in 1-2w. Early systemic symptoms common. Most in limbs, but neck 25%. Org can be
Ided with Warthin-Starry stain of nodes. Most resolve in 6-8w. Suppuration unusual, but systemic
manifestations such as encephalitis, ocular in 12%. Excisional Bx may be needed if sinus develops or Dx
uncertain.
TB :- painless nodes with malaise, LoW, cough, fever. Esp in posterior triangle. Mantoux +ve.
Actinomycosis. :- involves soft tissues, not nodes. Woody mass, Sinuses often present.
Definition:
(Greek, hygros-moist)
A cystic lesion caused by a mass of dilated lymphatics which is usually found in the neck.
Mulliken says should not be called lymphangiomas because this signifies a tumour. He feels it should be
called a lymphatic malformation.
Incidence:
1/12,000 births , M=F
usually at birth or within the first year of life
may be identified in utero by ultrasound
Pathology:
Cystic hygromas begin to develop at about 40 days gestation and are due to the failure of the embryonic
lymphatics to connect with the venous system -> the lymphatics become dilated and continue to grow
forming cystic structures
the left-sided predominance may be due to a failure of the thoracic duct junction to connect with the left
subclavian vein
There are six primative lymph sacs: 2 jugular, 2 posterior in the sacral region, the cysterna chyli and the
thoracic duct, all of which eventually join up.
These are hamartomatous, lymphatic malformations that result in a multicystic mass which infiltrates tissue
planes and has no tendency to spontaneous regression (except occasionally antenatally, see later).
If diagnosed before 30/40 tend to be associated with a poor prognosis. They tend to be located in the
posterior cervical region and are associated with the presence of fetal hydrops and difuse
lymphangiomatosis. 1/2 will have abnormal karyotypes (Turner syn, trisomy 13, 21, 22) also Noonan
(Turner-like syndrome, webbing of neck, pectus excavatum, cryptorchidism, pulmonary stenosis), fetal
alcohol. Some with Turner and Noonan Syn will spontaneously regress in the second trimester and some
authors believe that it may be the aetiology behind the webbed neck seen in children with these syndromes.
After 30/40 it does not seem to be associated with hydrops or genetic anomalies.
Location:
75% in the neck, L:R 2:1
20% in the axilla
5% in the mediastinum, retroperitoneum, or pelvis
Lymphangioma is the most common cause of macroglossia in infancy. can also involve the buccal mucosa.
floor of mouth, salivary glands, including the parotid
Clinical Features:
diffuse, non-tender mass usually found in the neck at birth soft, well-demarcated, and irregular on
palpation
usually found in the posterior triangle of the neck
mass may be small -> large
easy to transilluminate
small and medium size hygromas are usually asymptomatic
larger hygromas may extend into the face, mouth, tongue, parotid, larynx, or chest (15% extend into the
mediastinum) and can be associated with complications
Complications:
upper airway obstruction (stridor, apneas, cyanosis) dysphagia
mandibular maldevelopment, and malocclusion.
nerve palsies
hemorrhage
infection
sinus formation
Investigations:
1. Ultrasound - unilocular or multilocular cyst with a thin or thick wall
2. Needle Aspiration - usually a clear or straw-colored fluid, may also be bloody, blood tinged, brown,
and/or purulent if complicated by hemorrhage or infection
Management:
Antenatal diagnosis by U/S is possible for larger masses; affected infants should be delivered in a centre
capable of expert ET intubation rare cause of obstructed labour, and elective C/S may be necessary in a
proportion.
Surgery:
surgical removal if large and/or symptomatic, may require multiple surgeries
In neck masses, the accessory nerve seems to be at most danger because it tends to get tented up by the
mass or become invested by it. The facial nerve usually lies deep to the masses and is less commonly
damaged (Pitkin).
Injection therapy:
OK-432 (an incubation of streptococcus and penicillin G) -has been very effective. Cannot give with
penicillin allergy, as made with penicillin G. Need pre and post op photographs, and preoperative CT scans.
Latest paper on how it works (Ogita et al 1996 31 477-480).
Bleomycin in a microsphere-in-oil fat emulsion (9mg/ml) given at a dose of 0.3-0.6 mg/kg has been used
with success in Japan (Tanaka et al 1990 PSI 5 270). Dr Shun uses 0.5mg/Kg.
Jill Orford (JPS 1995 30 1282). They treated 16 cases with an excellent response in 44%, good in 44% and
poor or none in 12%. 11 single treatments were used and 5 patients required multiple treatments.
no major side effects, and minor side effect occurred after 27% of injections (fever, vomiting, cellulitis,
skin discoloration. Recommends injection always under U/S control to miss the vessels.
Pulmonary toxicity resulting in pulmonary fibrosis is the main side effect of this drug- but has not been
reported after injection therapy and would not be expected with the low doses used.
Laser: - Has a use in airway lesions and is done in Sydney by the ENT Surgeons.
Radiotherapy: - Has been used in past but has side effects such as growth problems and thyroid
insufficiency.
Prognosis:
depends on size of cyst and if complications
unpredictable cystic growth and growth into surrounding tissues
some cysts may resolve spontaneously
recurrence is possible
normal life span and intelligence
Papers:
Langer et al looked at 29 fetuses diagnosed with cystic hygroma antenatally and found the majority
developed hydrops fetalis or diffuse lymphangiomatosis which led to fetal demise. There was usually either
an associated chromosomal abnormality (usually Turner syndrome, but many others have been reported)
spontaneous regression has occasionally been seen with Turner or Noonan syndrome. of the 27 fetuses
diagnosed before 30 weeks gestation only one survived. Twenty five of the 27 were aborted (21
therapeutic); severe hydrops was present in 21 of these 25. Two of the 27, both with stigmata of Noonan
syndrome, underwent spontaneous regression during the second trimester. Karyotype analysis appeared to
be important for genetic counselling, in that fetuses with abnormal chromosomes tended to be sporadic
cases, with a low risk of recurrence in future pregnancies. Normal chromosomes were correlated with a
history of consanguinity, multiple structural anomalies, polyhydramnios, and familial syndromes, with a
higher recurrence risk. The two fetuses diagnosed after 30 weeks gestation had a much different outcome.
Neither had hydrops, polyhydramnios, associated anomalies, or an abnormal karyotype. One had a
completely normal sonogram at 17/40, which suggests that the cystic hygroma had developed later in
gestation. Both survived after an operation at 4 days of life. They continue to say that there may be two
separate aetiologies for this condition with vastly different outcomes, ie those presenting before 30/40 and
those after which include the majority of cystic hygromas.
A ranula is a cystic swelling in the floor of the mouth containing mucoid salivary secretions, which most
commonly originate from a sublingual salivary gland. The extravasated mucous may track below the
myolohyoid (plunging ranula). A plunging ranula is not epithelially lined and are therefore pseudocysts.
This may occur without a ranula being present in the floor of the mouth, in which case its origin may not be
appreciated.
Diff diag: branchial cyst, thyroglossal cyst, epidermoid cyst, tetatoid cyst, cystic hygroma, cervical thymic
cyst, laryngocoele, AV-malformation, lymphadenopathy, abscess, or soft tissue tumour.
U/S or CT should give diagnosis.
Excision of the ipsilateral sublingual gland seems to be essential in order to cure the condition. The
recurrence rate for plunging ranula after excision of the cyst through a submandibular incision is 85%, 53%
after marsupialisation, and 2% after excisionn of the sublingual gland with transoral drainage of the cyst.
TORTICOLLIS
Causes inc:
SM tumour common, > 3/52
Abn in utero posture improves with age
Cx hemivertebrae structural plain x-ray
RPh abscess toxic
Ocular imbalance 2o to squint
Post fossa tumour rare, other neurol signs
Spasmodic with GOR Sandifers syndrome
Postural familial
Secondary effects plagiocephaly within 1st few months, tends to resolve when sitting. Sig facial
hemihypoplasia > 8/12, more often noticed at 3-4yrs. Elevate ipsilat shoulder, scoliosis to keep eyes level.
All improve with SMT resolved.
Treatment:
Value of manipulation not proven, ? value of something being done ? place toys etc on opp side
Indications for Sx: - persistent tightness with LOR >12-15/12
- progressive facial hemihypoplasia
- presentation > 1yr
Technique GA, supine, shoulder roll, head to opp side
3-4cm incision 1cm > clavicle, 2 heads dissected and div with diathermy. Investing fascia divided
ant and post to muscle, continue across post triangle to ant border of trapezius can feel tightness,
under vision!. Close platysma, skin
recurrence rare - <3%. Comps haematoma, nerve damage
follow patients until T resolved completely, full ROM, SM feels normal.