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Branchial Cleft Anomalies

- Male and female incidences are equal - Nearly all these lesions are recognized by the time the patients reaches 30 years of life - Small cysts may not be recognized until the second decade in life Clinical presentation: - Typically present as smooth, round, fluctuant, non-tender masses along the anterior border of SCM muscle, anywhere from the external auditory canal to the clavicle - During upper respiratory tract infections, a painful increase in size is common and occasionally associated with external drainage through an unrecognized fistula

TREATMENT: Preoperative assesment: USG, CTscan and/or MRI is essential; may be combined with a fistulogram or pharyngoesophagram Complete surgical treatment excision is the treatment of choice for branchial cleft anomalies and is indicated for recurrent infection, cosmetic deformity, and potential for malignant degeneration To avoid recurrence, combined endoscopic examination for a pharyngeal pouch and sinus tract with meticulous dissection of a sinus tract It is essential to excise enbloc any portion of the thyroid involved in the sinus tract

Branchiogenic carcinoma
Squamous cell carcinoma arises in a branchial cleft cyst - Cystic squamous cell carcinoma presenting in the neck without an apparent primary is almost universally secondary to metastasis from a neoplasm arising in the faucial or lingual tonsillar crypt epithelium or nasopharyngeal tissue - Malignant transformation with in situ branchial cyst carcinoma is a rarity - Management: wide excision of the tumor and ipsilateral radical neck dissection followed by adjuvant radiation therapy or chemoradiation

Lymphangiomas, Hemangiomas, and other Vascular Anomalies


Congenital lymphangiomatous malformations Result from abnormal development of the lymphatic system at sites of lymphatic-venous connection, with obstruction of lymph drainage from the affected area causing multicystic endothelium-lined spaces The neck is the most common site (25% of all cases) Over half present at birth, with 90% manifesting by 2 years of age Arise above the mylohyoid muscle => more infiltrative and tend to extend from skin to mucosa arise below the mylohyoid muscle => discreet and cystic

Cystic Hygroma
Cystic hygroma: large lymphangiomas; most commonly found in the posterior triagle of the neck and axilla in children Cervical cystic hygroma => appear before 30 weeks gestation => usually associated with karyotypic abnormalities, various malformation syndromes and several teratogenic agents => prognosis: poor Cystic hygroma developing late in pregnancy has a more favorable outcome and is more likely to be encountered by the head and neck surgeon 2/3 are asymptomatic Soft, painless, compressible masses that may increase when patient cries After an URI, sudden enlargement with infamation, infection, dysphagia, and stridor may develop (more commonly seen if the anterior triagle of the neck is involved or in patient with pharyngolaryngeal extension or intraoral involvement)

Vascular Anomalies (I)


=> Hemangiomas and arteriovenous malformations or lymphovenous lesions Hemangiomas are frequently multiple with the parotid gland, a common site of occurrence Hemangiomas proliferate during the first year of life, then involute at a variable rate over several years Treatment for hemangiomas in the proliferate phase:
Subcutaneous recombinant interferon alpha 2a or oral methylprednisolone administered over 6-12 months Cyclophospamide may induce fast regression in neonates with life threatening hemangiomas
Surgical excision is reserved for severe cosmetic deformity or life threatening aerodigestive tract impairment

Vascular Anomalies (II)


Diagnostic imaging of these anomalies is based on the need for surgical treatment (only those lesions that cause functional impairment or developmental disturbance are surgically addressed) Angiography + MRI => allows separation into low-flow lesions (hemangiomas, venous, lymphatic malformations) and high-flow lesions (arteriovenous malformations and invasive, combined lymphovascular malformations)

Vascular Anomalies (III)


Treatment for low-flow: macrocystic-type lymphangiomas less than 5 cm Sclerosant therapy with the agent OK-432, a lyophilized mixture of low-virulence group A Streptococcus pyogenes with penicillin G potassium Treatment for high-flow: Preoperative embolization at the time of selective angiography and surgical excision

Vascular Anomalies (IV)


Small Cystic Hygromas Treatment of small cystic hygromas that have not regressed but have enlarged is with surgical excision with staged debulking of larger cystic hygromas Neural structures should not be sacrified recurrence is uncommon when gross neoplasm is removed

Aberrant Thyroid Tissue (I)


Embryology: Primitive thyroid gland begins as a ventral diverticulum of endodermal origin, arising in the floor of pharynx between the tuberculum impar and copula. Ultimately, it becomes the foramen cecum and the copula become the posterior third of the tongue. The thyroid descends caudally through the primitive hyoid bone. The developing thyroglossal duct reaches its final position in the midline of the neck and develops into the median lobe of the thyroid. It normally persists as a hollow stalk for 6 weeks and then atrophies

Aberrant Thyroid Tissue (II)


Can occur anywhere from foramen cecum to the lower neck Most frequently occur as a thyroglossal duct cyst associated with a normal thyroid gland Less common is total ectopia, manifesting as a lingual thyroid The findings of abberant thyroid tissue should direct attention to the ipsilateral thyroid lobe Cervical metastases of papillary thyroid carcinoma (PTC) can mimic brachiogenic cysts clinically and histopathologically

Thyroglossal duct anomalies


Result from a failure of complete obliteration of the thyroglossal duct and located anywhere along the descent of the gland Thyroglossal duct cyst is the most common congenital neck mass and the second most common of all childhood cervical neck masses; most commonly are found below the hyoid bone and above the thyroid gland, displaying movement with anterior tongue protrusion and swallowing The cyst are usually 1-3 cm in diameter and are smooth, round, fluctuant Hereditary predisposition is suspected S&S: - cysts, sinuses, and fistulas of the thyroglossal duct manifest as anterior midline neck masses from the foramen cecum to the thyroid gland, most before 10 years - thyroglossal ducts and fistulas are often asymptomatic; may become recurrently infected during upper respiratory tract infections, which can cause cyst enlargement abscess development, and rupture wuth external sinus formation

Lingual Thyroid (I)


Present in the midline as a sessile, nontender reddish mass in the base of the tongue, anterior to the valleculae Female preponderance of 7:1 Most frequent benign mass encountered in the oropharyx Symptoms: dysphagia, cough, dysphonia, cough, and in extremely rare cases, symptoms of obstructive sleep apnea and hemorrhage Lingual thyroid may become apparent during pregnancy (increased of thyroid function) The mass consists of normal or immature thyroid tissue, which may be functional or dysfunctional

Lingual Thyroid (II)


Indications for surgival removal: uncontrollable hyper-thyroidism, hemorrhage, symptomatic enlargement or question of malignancy Preoperative => thyroid uptake and scan => to determine whether functioning thyroid tissue exists in its normal cervical location MRI may also be of value, because it allows multiplanar imaging and provides the best soft tissue definition

lingual thyroid: covered with squamos cell epithelium and often exhibits an abundant vascular supply thyroglossal duct cysts and fistulas: squamous, ciliated columnar or transitional cell epithelium the cyst or sinus tract is filled with mucoid or mucopurulent material

Histopathologic Features in lingual thyroid and thyroglossal duct cysts and fistulas

Treatment
LINGUAL THYROID Preoperative thyroid scan and uptake study are mandatory THYROGLOSSAL DUCT CYSTS AND FISTULAS Indications for surgical excision: malignant degeneration (predominantly of the papillary type; usually patients in their forties), recurrent infections, undesirable cosmetic appearance, intermitent upper airway obstruction Incision and drainage => if an abscess has developed high recurrence after excision => Sistrunk procedure to prevent recurrence treatment if papillary carcinoma is found in an abberant position => total thyroidectomy with central lymph node dissection Treatment of thyroglossal duct remnant carcinoma consists of the Sistrunk procedure, total thyroidectomy, central lymph node disection, ablative iodine131 therapy for residual metastatic thyroid carcinoma subsequent thyroid supression therapy and careful interval follow up with whole body thyroid scanning

Thymic Cyst
Extremelly rare, more common in male Most occuring asymptomatically in children and adults Only 10% have involved patients younger than 1 year of age Primarily located anterior and deep to the middle onethird of SCM muscle Ectopic servical thymus is best imaged by MRI whereas thymic cyst has a more consistent appearance on CT Surgical resection provides definitive dx and cure

Neurogenic neoplasm (I)


Congenital neurogenic lesions involving the head and neck include all neoplasms or anomalies originating in the neural tissue or its covering 2 groups: - heterotopic brain lesions with developmental defects - neoplasms of neurogenic origin including neuromas, neurofibromas, ganglioneuromas, and meningiomas schwannomas => neuromas originating from the connective tissue sheath of the nerve They may appear as multiple neurofibromas arising from cutaneous, visceral, and cranial nerves in neurofibromatosus type 1 (NF1) or type 2 (NF2) and have a predilection to arise in deep planes of the neck

Neurogenic neoplasm (II)


Neuromas => solitary, encapsulated lesions with elongated spindleshaped cells with an oval or flattened nucleus Ganglioneuromas => rarely seen in the head or neck, are characterized by ganglion and glial cells Meningiomas => usually benign and arise from embryonic arachnoid rests, may appear extracranially at the nasal root or in the sinuses Whorl-like fibroblastic nuclei with hyaline formations producing sand-like appearance may be present (psammoma body) Pharyngeal neuromas => rare, appear as smooth, firm, rounded, and yellow masses if multiple brown discolorations of the skin or caf au lait spots are present => neurofibroma may suggested treatment is surgical excision and must be complete because recurrence is common