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158
CHAP TER5 The front oftheneck
Hodgkin’s disease in 80% of cases involves cer vical lymphnodes.
Some patients present with systemic symptoms such as weight loss,
fever, and night sweats (type B symptoms).
Diagnosis is conrmed by biopsy (often excision biopsy is necessary
after initial FNA cytology).
Secondary:metastatic disease
Metastatic cervical lymph nodes, secondar y to head and neck
malignancy are quite common.
In approximately 90% of cases spread to the cervical lymph nodes
occurs in a predictable fashion (superior neck nodes being involved
before inferior nodes).
Pattern and extent of cer vical lymph node involvement dictates the
nature of treatment.
Neck nodal involvement is the single most important prognostic
factor in determining patients’ survival.
Factor s of prognostic importance are the level, numbers, and size of
nodal involvement and presence (or absence) of extr acapsular spread.
Treat neck nodes seriously. Regard the solitary node as a malignancy until ruled out. Refer accordingly.
bThyroid/ thyroglossal cyst/ thymus
The enlarged thyroidgland
The thyroid gland lies in the midline of the lower third of the neck behind the pre- tr acheal fascia. It consists of two pear- shaped lobes connected by an isthmus in the middle. A normal thyroid gland weighs approxi­mately 20 g in adults. Posterior to the thyroid gland are superior and inferior parathyroid glands (calcium homeostasis).
Assessment of a thyroid lesion essentially involves answering the fol­lowing questions:
What is the patient’s thyroid status— normal, over- or under- active?
Is it a generalized enlargement of the gland or a solitary nodule?
Can this be malignant? Both solitary and multinodular goitres can be
benign or malignant.
bGoitres
A goitre is an enlarged thyroid gland. The term goitre is generally used to refer to any enlargement without reference to thecause.
Dierent types ofgoitres
Physiological (usually diuse goitre):seen during pregnancy, at
puberty and in conditions of iodine deciency (now uncommon).
Inammatory (usually diuse but can be multinodular):De Quervain’s
thyroiditis, Hashimoto’s thyroiditis, Riedel’s thyroiditis.
Toxic (usually diuse goitre):Gr aves’ disease.
Nodular:this is a simple benign enlargement of the thyroid gland.
Anodular goitre can be a solitary nodule or multinodular. This only
requires treatment if the patient is thyrotoxic, concerned with its
appearance, or has symptoms of compression of adjacent structures
(e.g. dysphagia or dyspnoea).
THYROID/THYROGLOSSAL CYST/THYMUS
Key questions
Rate of growth ofgoitre
Pressure symptoms:hoarseness, pain, dysphagia
Symptoms of hypo- or hyperthyroidism
Radiation exposure, family history of thyroid disorders/ cancers.
Investigations
Thyroid function tests:TSH, freeT4.
FNA.
Thyroid r adionucleotide scintigraphy:to determine function, or
identify ectopic thyroid tissue (retrosternal, lingual, and metastasis).
Ultrasound:denes lumps from cyst s and guidesFNA.
CT/ MRI:evaluates substernal goitre, nodal involvement, airway and
vascular displacement, tumour invasion.
CXR:metastasis work- up, tracheal displacement .
Indications forthyroidectomy
Excision may be required if there is suspicion of malignant change
(rapid increase in size of goitre, lymph node enlargement , or hoarseness from recurrent laryngeal ner ve involvement).
Compression symptoms (airway compromise, dysphagia).
Extension into the mediastinum (substernal goitre).
Cosmesis.
Failed medical management for Gr aves’ disease or hyperthyroidism.
bThe solitary thyroid lump (nodule)
These have several causes:
Cystic:this is usually a degenerative part of a nodular goitre although
true cysts are also seen. Haemorrhage into the cyst is common and will present with pain and rapid enlargement.
Adenoma:this may produce thyrotoxicosis if functioning. Subdivided
histologically:papillary, follicular, embryonal, and hurtlecell.
Carcinoma:4 – 10% of solitary nodules can be malignant.
Dierent types ofthyroid cancers
Papillary adenocarcinoma:seen in younger age groups; low grade and
rarelyfat al.
Follicular adenocarcinoma:a malignancy of middle age; bony
metastases are common.
Anaplastic carcinoma:an aggressive malignancy of the elderly;
metastatic disease at presentation is common.
Medullary carcinoma:seen in all age groups with equal sex incidence;
moderate malignant potential spreading to lymphnodes.
Malignant lymphoma:may occur in lymphatic tissue within the thyroid
gland or as secondar ies.
Secondary:direct spread from adjacent malignancies or metastatic
spread, most commonly from brea st, renal, colon, andlung.
Risks factors forthyroidcancer
Extremes of age, nodule >5cm, previous radiation therapy, autoimmune thyroiditis (develop lymphomas).
159
160
CHAP TER5 The front oftheneck
bThyroglossal ductcyst
Failure of complete obliteration of thyroglossal duct results in a midline painless neck mass. The cyst is attached to the hyoid bone so elevates with tongue protr usion. Patients may have dysphagia. Cysts can get infected. Malignant potential is rare. Refer for surgical excision
bThymiccysts
Pathophysiology:remnant of third pharyngeal pouch between angle
of mandible to midlineneck.
Symptoms:midline neck mass at lowerneck.
Diagnosis:biopsy, serum calcium (associated parathyroid disorders,
DiGeorge’s syndrome), CT, andMRI.
Treatment is surgical excision.
bCarotid bodytumour
Carotid body tumours (chemodectomas) are rare, slow- growing lesions arising in the carotid bifurcation, distorting and encasing the carotid ves­sels. Chronic hypoxia has been repor ted as a causal factor (high inci­dence in high- altitude areas). A familial tendency in 10%, and 10% are bilateral. Left untreated about 5 – 10% will develop metastases within 10years. May also become locally invasive.
Clinical features
These usually present in patients over 50years of age as solitary or
bilateral lumps at the level of carotid bifurcation (Figure5.5).
They transmit the carotid pulse rather than being pulsatile itself.
Figure5.5 CT showing bilateral carotid body tumours.
BRANCHIAL CYSTS/SINUSES/FISTULAE
Most can be moved laterally (displacing the carotid pulse) but not
vertically (Fontainesign).
Occasionally pressure on the carotid sinus by tumour may cause
fainting attacks.
Management
Surgical excision is the management of choice in the young patient. In elderly, frail patients, follow- up only is required.
bBranchial cysts/ sinuses/ fistulae
bBranchialcyst
After the thyroglossal cyst, the branchial (lateral cervical) cyst is the second most common congenital swelling in the neck. It is thought that branchial cyst s develop from remnants of the second branchial cleft. In some ca ses tract s are found running from the deep surface of the cyst to the pharyngeal wall. However, it has also been postulated that they are a result of cystic degeneration in cer vical lymphatic tissue (Figure5.6).
Clinical features
Most lesions present in the third decade of life. Patients complain of an enlarging mass arising along the junction of the upper and middle thirds of the SCM muscle. Frequently the cyst may appear as a swelling during an upper respiratory tract infection, which may be painful and persist after the infection has been treated. Recurrent infections can result in a rm, xed mass adherent to surrounding structures such as the jugular vein, proving dicult to excise surgically.
161
Figure5.6 Branchialcyst.
162
CHAP TER5 The front oftheneck
Investigations
Diagnosis is usually made on the ba sis of the history and the site of the swelling. However, FNA biopsy can prove useful by producing an opales­cent uid containing cholesterol cr ystals or frank pus. CT or MRI helps dene its size anddepth.
Management
Surgical excision of the lesion is the treatment of choice following man­agement of infections with appropriate antibiotics. All of the cyst lining should be removed as any remaining remnants may result in recurrence or a chronic discharging sinus from thewound.
bBranchial sinus/ branchial stula
A branchial sinus is a small opening found over the anterior border of the SCM, which may discharge a mucous secretion. They are generally pres­ent at birth but may present in later life as a consequence of a ruptured, chronically infected branchial cyst. The sinus can extend superomedially between the internal and external carotid ar teries to open onto the lat­eral wall of the pharynx forming a br anchial stula .
Summary ofbranchial cleft anomalies
Pathophysiology:developmental alterations of the branchial
appar atus results in cysts, sinuses (single opening to skin), or stulas
(opening to skin and digestive tract).
Symptoms:neck mass in anterior neck (anterior to SCM), stulas and
sinuses may express mucoid discharge; secondary infections cause
periodic uctuation of size, tenderness, and purulent drainage.
Diagnosis:CT with contrast (may consider injecting contrast into
stula), laryngoscopy to visualize internal opening.
Histopathology:lined by squamous epithelium.
bPlungingranula
Congenital ranulas arise secondary to an imperforate salivary duct . These are ver y rare and spontaneously resolve. More commonly, ranu­las arise from minor trauma to the sublingual gland, leading to mucus extravasation and formation of a pseudocyst. Partial obstruction of the sublingual duct leads to formation of an epithelial- lined retention cyst. Plunging ranulas can manifest as neck swellings usually in conjunction with swelling in the oor of themouth.
Investigations
CT scan may show a unilocular hypodense lesion within the sublingual space. Plunging ranulas are occasionally noted on CT scanning to have a small tail extending into the sublingual space and this nding is almost pathognomonic. Ultrasound and MRI may be useful but not routinely ne ce ssary.
DERMOID CYSTS AND TERATOMAS
Treatment
Observation for spontaneous resolution of congenit al ranulas is usually all that is required. Surgical treatment for acquired ranulas includes mar­supialization or excision via the mouth orneck.
bDermoid cysts and teratomas
Dermoidcysts
These are considered the most common for m of teratoma and are char­acterized by a predominance of ectodermal content. Most dermoids in the head and neck occur in the region of the oor of the mouth. They are believed to be caused by epithelial rests trapped during embr yologi­cal development. Clinically, they manifest as slow- growing lesions that produce symptoms when their cystic lumens become lled with keratin debris. Sebaceous material may also be found in the cyst, alluding to its ectodermal origin.
Management
The management of these cysts is surgical excision.
bCervical teratomas
These are extremely uncommon lesions of the head and neck with preva­lence of 1 in 16,000 bir ths. In the neck, they most often occur in the midline and may manifest clinically with severe respiratory distress and dysphagia due to compression of the trachea and oesophagus. These lesions are known to often grow into a large size (as far as 12 cm) and histologically, may contain any combination of tissues from the three germ layers. They are sometimes classied in relation to proximity to the thyroid gland as follows:(1)teratomas of the thyroid gland, deriving its blood supply from thyroid arteries; (2) teratomas adjacent to the thyroid gland, in which a denitive blood supply cannot be identied; and (3)teratomas of theneck.
Management
Cervical teratomas are managed with surgical excision. Untreated cases risk possible malignant degeneration. The prognosis in non- neoplastic cases is excellent . The ex utero intrapartum treatment (E XIT) procedure is a technique that allow par tial fetal delivery via Caesarean section, with establishment of a safe fetal airway. This may be necessary if there is fet al airway obstruction diagnosed by prenat al scanning.
163
When examining a salivar y gland swelling, assess the following:
Palpation (mobility, size, consistency)
Bimanual palpation, with gland massage and inspection of any saliva
expressed
Tenderness (inammatory process)
Facial nerve dysfunction (suggests malignancy)
Lingual and hypoglossal nerve dysfunction with submandibular
gland tumours
Parapharyngeal space (tonsil) displacement with parotidlumps
Cervical lymphadenopathy.
164
CHAP TER5 The front oftheneck
The salivaryglands
There are three pairs of major salivary glands and several hundred minor salivary glands distributed throughout the upper aerodigestive tract. The parotid is the largest gland and produces predominantly serous saliva. Submandibular and sublingual glands secrete mucoserous saliva. Salivary stones are more common in the submandibular gland because of its high mucinous content. Minor salivary glands are concentrated on palate, lips, and pharyngeal mucosa and secrete predominantly mucinous saliva which helps to keep mouthmoist.
Swellings of the glands may present as a swelling in the neck, mouth, or side of the face. It is unusual for the sublingual gland to produce a true neck swelling except with a plunging ranula. The causes of salivary gland swelling are in essence threefold:obstructive, infective, and neoplastic. These may occur in isolation although they may coexist, e.g. a stone may result in infection.
cObstruction
Obstruction of any par t of the duct system may result in a build- up of salivary secretions and swelling. With recurrent bouts of obstr uction, infection may supersede due to stagnation of secretions. The classic his­tory is of swelling associated with meal times— the patient may repor t that the swelling settles a few hours af ter the end of eating (Figure5.7).
Salivary calculi form as a result of calcium deposition around a nidus of organic material. 80% occur in the submandibular gland. Of the subman­dibular stones, 20% are radiolucent. In these cases sialography is indi­cated to locate them. Stones in the oor of the mouth may be removed through a local incision under local anaesthetic. However, recurrent damage to the gland may necessitate its removal.
Figure5.7 Acute submandibular obstruction.
THE SALIVARYGL ANDS
Duct strictures can also form as a result of chronic trauma or iatro-
genic injur y or may be caused by ulceration around a salivar y calculus. Fibrosis leads to duct stenosis and obstructive symptoms.
Neoplasia may occasionally present with obstructive symptoms.
cInfections
Infec tion in a major salivary gland usually presents with a painful, red, warm, tender swelling. The regional lymph nodes may be enlarged and tender, and pus may be seen to exude from the duct orice on gently massaging the gland. Infection of the submandibular gland may present as a painful lump in the neck . Infection of the parotid gland tends to pres­ent as a painful swelling on the side of the face. Infections of the sublin­gual gland and minor salivary glands are generally rare and present with symptoms within themouth.
Mumps
Both bilateral and unilateral painful parotid swelling is commonly due to a paramyxovirus infection. This is highly infectious, generally aecting children, with an incubation period of 21days. Immunity is long- lasting after an attack. Treatment is symptomatic (analgesia and applying a warm or cool compress to help relieve pain). Complications of mumps include pancreatitis, meningitis, oophoritis, and orchitis (especially in adults).
Suppurative parotitis
This is seen in debilitated patients, particularly following major surgery, as a result of xerostomia secondary to dehydration. Oral ora ascends along the duct into the gland which becomes infected. Management includes rehydration and appropriate antibiotic therapy.
Chronic sialadenitis
Usually a complication of recurrent duct obstruction.
bTumours
70– 80% of all salivary gland tumours arise in the parotid. Of these, approximately 80% are pleomorphic adenomas and 10– 15% are malig­nant (Figure5.8).
The classication of salivar y gland tumours is complex. This includes
(not an exhaustivelist):
Benign epithelial tumours (pleomorphic and monomorphic
adenomas, myoepitheliomas, and Warthin’s tumours)
Malignant epithelial tumour s (acinic cell, mucoepidermoid, and
adenoid cystic carcinomas, salivary duct carcinomas)
Soft tissue tumours (lymphangiomas, haemangiomas, and
lymphomas)
Metastatic tumours (skin cancers metastasizing to parotid nodes).
Lumps need urgent refer ral to a head and neck specialty (ma xillofacial/ ENT etc.). Imaging is usually required (CT/ MRI or ultrasound). The role of FNA is controversial. Management is surgical removal.
165
166
CHAP TER5 The front oftheneck
Figure5.8 Parotid tumour.
bSalivary gland dysfunction
Xerostomia (drymouth)
There are many causes for this including primary salivary disorders (Sjögren’s disea se), medication induced (especially tricyclic antidepres­sants, antihistamines, antimuscarinic drugs, antiepileptic drugs, anti­psychotics, beta- blockers, and diuretics), radiotherapy, dehydration, and mouth breathing from nasal obstruction. Treat with ar ticial saliva, sugar- free chewing gum, or pilocarpine. Ask about dry/ gritty eyes. If pres- ent, this could be Sjögren’s syndrome. Discuss with ophthalmology regarding ocular assessment.
Ptyalism (drooling)
True overproduction of saliva is very rare (causes include intraoral infections, foreign bodies such as new dentures, and mercury poison­ing). More commonly patients just don’t swallow normal amounts eec­tively. Causes include Parkinson’s disease, epilepsy, and other swallowing disorders. It also occurs during pregnancy. Treatment includes scoline patches or in severe cases chorda tympani transec tion, ductal rerouting, ligation of Stenson’s duct, or gland excision.
Some usefulfacts
Mumps is the commonest cause of acute parotid swelling, whether it
is unilateral or bilateral.
Most parotid lumps are benign. Malignancy is more likely in
submandibular/ sublinguallumps.
Alump associated with ner ve dysfunction (facial, lingual, hypoglossal)
suggests inltrative pathology (i.e. tumour).
Not all stones show up on plain X- rays.
Ask about dry grit ty eyes, joint symptoms, and ra shes in anyone
complaining of severe dry mouth or recurrent salivar y symptoms.
HOARS E VOICE (DYSPHON IA)/LOSS OFVO ICE
HIV can aect the salivary glands.
Heer fordt’s syndrome is sarcoidosis resulting in parotid enlargement,
fever, anterior uveitis, and facial nervepalsy.
Anxiet y and medication are the commonest causes of a drymouth.
cHoarse voice (dysphonia)/ loss ofvoice
Dysphonia is a common condition and in most cases the underlying cause is benign and self- limiting. However, persistence of symptoms requires care-
ful evaluation:it can be an early sign of malignancy.
Components ofspeech
Respiration:source of energy from inhalation/ exhalation
Phonation:production of voice by vocal fold vibration
Resonation:oral/ nasal speech balance determined by velopharyngeal
musculature and resonance of the sinuses, nasopharynx, nasal cavity, and oralcavity
Articulation:speech sounds using muscles of lips, tongue, andjaw
Assessment ofa patient withdysphonia (usually asan outpatient)
Character ofdysphonia
Onset and dur ation, time course (acute vs chronic), periodicit y (morning hoarseness with GORD and evening hoarseness with voice abuse).
Contributing factors
Voice abuse, recent URTI, fever, sore throat, cough, congestion; tobacco or alcohol abuse; PMH of neuromuscular disorders, hypothyroidism, psychological stressors, previous lar yngeal trauma, surgery, or airway manipulation.
Associated symptoms
Dysphagia, aspiration, weight loss, hearing loss, heartburn.
Assess perceptual quality ofvoice
Abnormally high pitched or low pitched, abnormally loud orsof t.
Examination
Indirect or direct laryngoscopy (mirror, exible nasopharyngoscopy, videostroboscopy); assess vocal fold motion, examine laryngeal lesions and appearance of vocal folds, assess glottic competence. Neck masses, thyroid masses, neurological examination.
Videostroboscopy
Examines the vocal fold mucosa for general health, vocal fold anatomical defects, vocal fold disturbances, and mucosal wave dynamics.
Aerodynamic studies and laryngealEMG
Measures airow, pressures, and pat ter n of vibration of the vocalfolds.
167