HEAD AND NECK LESIONS
A. Cervical Lymphadenopathy
An enlarged lymph node is the most common neck mass in children. Most are anterior to the sternocleidomastoid muscle. Infection is the usual cause of enlargement; viral etiology and persist for months. Acute suppurative submandibular adenitis occur in early childhood (6 mo-3 yrs), is preceded by pharyngitis or URI, the child develops erythema, swelling and cellulitis, and management is antibiotics and drainage. Chronic adenitis: persistent node (> 3 wk., tonsillar), solitary, non-tender, mobile and soft. Generally no tx if < 1 cm, for nodes above 2 cm sizes with rapid growth, clustered, hard or matted do biopsy.
Other causes are: (1) Mycobacterial adenitis- atypical (MAIS complex), swollen, non-tender, nor‑inflamed, positive skin test, excision is curative, chemotx is of no value. (2) Cat-Scratch adenitis- caused by A. Fellis, transmitted by kittens, positive complement fixation test, minimally tender, fluctuant regional nodes, spontaneous resolution. (3) Hodgkin's disease mostly teenager and young adults, continuing growth, non-tender node, associated to weight loss, biopsy is diagnostic.
B. Congenital Torticollis
Congenital muscular torticollis is a disorder characterize by shortening of the cervical muscles, most commonly the sternocleidomastoid (SCM) muscle, and tilting of the head to the opposite side. This is the result of endomysial fibrosis of the SCM muscle. There is a relationship between birth position and the side affected by the contracture. Congenital torticollis causes: plagiocephaly (a craniofacial deformity), fascial asymmetry (hemihypoplasia), scoliosis and atrophy of the ipsilateral trapezius muscle if not corrected. Torticollis can develop at any age, although is more common during the first six months of life. The SCM muscle can be a fibrous mass, or a palpable tumor 1‑3 cm in diameter within the substance of the muscle is identified by two to three weeks of age. Management is conservative in most cases using early physiotherapy exercises a mean duration of three months to achieve full passive neck range of motion. The severity of restriction of motion is the strongest predictor of treatment duration. Those children with failed medical therapy or the development of fascial hemihypoplasia should undergo surgical transection of the SCM muscle.
C. Thyroglossal Duct Cysts
Thyroglossal duct cyst (TDC) is the most common congenital anterior midline neck mass usually (2/3 of cases) presenting before the second decade of life. Symptoms appear at an average age of four with the sudden appearance of a cystic mass at the angle of neck level moving with tongue protrusion and swallowing. Males are more commonly affected than females. TDC is an embryologic anomaly arising from epithelial remnant left after descent of the developing thyroid from the foramen cecum. The lining is cuboidal, columnar or pseudostratified epithelium. TDC is associated to discomfort, infection and a slight probability of malignancy. A legally protective requirement is to document that the mass is not ectopic thyroid gland. Diagnosis is physical. Sonograms will show a cyst between 0.4 and 4 cm in diameter, with variable sonographic appearance and no correlation with pathological findings of infection or inflammation. Once infected surgical excision is more difficult and recurrence will increase. Management is Sistrunk operation: Excision of cyst with resection of duct along with the central portion of hyoid bone (a minimum of 10‑15 mm of hyoid bone should be removed) and some muscle surrounding the proximal ductules (the length of single duct above the hyoid bone spreads into many ductuli as it approach the foramen cecum). Extensive dissection can cause pharyngodynia. The greatest opportunity for cure is surgery at initial non-inflamed presentation. Inadequate excision is a risk factor for further recurrence.
D. Branchial Cleft Fistulas
Branchial cleft fistulas (BCF) originate from the 1st to 3rd branchial apparatus during embryogenesis of the head and neck. Anomalies of the 2nd branchial cleft are by far the most commonly found. They can be a cyst, a sinus tract or fistulas. Fistulas (or sinus tract if they end blindly) display themselves as small cutaneous opening along the anterior lower third border of the sternocleidomastoid muscle, communicates proximally with the tonsillar fossae, and can drain saliva or a mucoid secretion. Management consists of excision since inefficient drainage may lead to infection. I have found that dissection along the tract (up to the tonsillar fossa!) can be safely and easily accomplished after probing the tract with a small guide wire in‑place. This will prevent injury to nerves, vessels and accomplish a pleasantly smaller scar. Occasionally a second stepladder incision in the neck will be required. 1st BCF are uncommon, located at the angle of the mandible, and communicating with the external auditory canal. They have a close association with the fascial nerve. 3rd BCF are very rare, run into the piriform sinus and may be a cause of acute thyroiditis or recurrent neck infections.
E. Cystic Hygroma
Cystic hygroma (CH) is an uncommon congenital lesion of the lymphatic system appearing as a multilocular fluid filled cavity most commonly in the back neck region, occasionally associated with extensive involvement of airway or vital structures. The etiology is intrauterine failure of lymphatics to communicate with the venous system. Prenatal diagnosis can be done during the first trimester of pregnancy as a huge neck tumor. Differential diagnosis includes teratomas, encephalocele, hemangiomas, etc. There is a strong correlation between prenatal dx and Turner's syndrome (> 50%), structural defects (Noonan's syndrome) and chromosomic anomalies (13, 18, 21). Early diagnosis (< 30 wk gestation) is commonly associated to those anomalies, non‑immune hydrops and dismal outcome (fetal death). Spontaneous regression is less likely but can explain webbed neck of Turner and Noonan's children. Prenatal dx should be followed by cytogenetic analysis: chorionic villous sampling, amniocentesis, or nuchal fluid cell obtained from the CH itself to determine fetal karyotype and provide counseling of pregnancy. Late diagnosis (>30 wks) should be delivered in tertiary center prepare to deal with dystocia and postnatal dyspnea of newborn. The airway should be secured before cord clamping in huge lesions. Intracystic injection of OK432 (lyophilized product of Streptococcus pyogenes) caused cystic (hygromas) lymphangiomas to become inflamed and led to subsequent cure of the lesion without side effects.
F. Cat Scratch Disease
Cat Scratch Disease (CSD) is a self‑limited condition transmitted by a Bartonella species (Rochalimaea henselae) present in unaffected kitten paws. Following inoculation by a scratch and one to two weeks of incubation period, malaise, fever, headache, anorexia and swelling of the regional lymph nodes follow. The adenopathy generally develops in the upper extremity (epitrochlear, axilla) or head/neck areas, is minimally tender and can develop fluctuation. Median age is 14 years with highest attack rate in children less than ten years of age. The diagnosis relies on the presence of symptoms, signs, physical exam (characteristic papule at the site of the scratch), history of exposure to a cat, and a positive immunofluorescent assay for Bartonella antibodies. Most patients with clinically diagnosed CSD developed an immunologic response to Bartonella species. Conservative symptomatic management is recommended for most children since the node will eventually disappear spontaneously. In other cases' aspiration of fluctuant nodes is alleviating. Antibiotics are recommended during severe cases. Overall prognosis is good.
G. Parotid Mass
A parotid mass in a child creates great concern and should be managed promptly since a high percentage of cases will harbor a malignant tumor. The more common benign parotid tumor in children includes hemangioma, pleomorphic adenoma and lymphangiomas. Infants with a rapidly enlarging violaceous or soft tumor in the parotid region harbor a hemangioendothelioma. Hemangioendothelioma is the most common parotid gland tumor of childhood. They seldom need excision as spontaneous regression is the norm. An asymptomatic, slow growing solid mass is the most common presentation in older children. Tenderness is associated with an infectious process. Diagnosis includes the use of Doppler ultrasound, CT-Scan, MRI and fine needle aspiration (FNA) biopsy. Salivary gland carcinoma is rare in children. Most common histological type is mucoepidermoid either as a primary neoplasm or secondary malignancy after neck irradiation. Management consists of superficial or total parotidectomy extent which is selected during the surgical procedure depending on deep gland or fascial nerve involvement. The tumor must be widely excised. Recurrence is managed with postoperative irradiation for high or intermediate grade malignancies. Rhabdomyosarcoma arising in the parotid gland area is another locally invasive aggressive tumor that presents early with swelling and symptoms of seventh nerve deficit. This tumor needs surgical excision followed by chemotherapy and irradiation.
Acute parotitis is a self-limiting disease most commonly associated with mumps (epidemic parotitis) in children. Other times the parotitis is associated with bacterial infection progressing to frank suppuration. Recurrent parotitis, also known as juvenile recurrent parotitis, is characterized by a cyclic swelling of the parotid glands associated with discomfort and/or pain in the absence of external inflammatory changes during a period of several years. The condition mainly affects children between the ages of three and six, males being more commonly affected. The symptoms peak in the first year of school and usually begin to subside after puberty. Retrograde infection induced by the mumps virus and upper respiratory infection play a major role in the etiology of recurrent parotitis. Sialography demonstrates sialectasia. Children with recurrent parotid swelling needs to be screened for underlying systemic immune disorders such as Sjögren’s syndrome. With time the recurrent episodes reduce salivary flow, while increasing the chloride, sodium, copper, albumin, IgA and lactoferrin concentration. Etiology of juvenile recurrent parotitis is a combination of congenital malformation of portions of the salivary ducts and a set-in infection. Treatment is conservative.
H. Hashimoto Thyroiditis
Hashimoto thyroiditis (HT) is a chronic lymphocytic autoimmune thyroiditis seen with some frequency in adolescent females and children. Most common cause of asymptomatic enlargement of the thyroid gland in children in iodine-sufficient geographic regions. Thyroid cell damage in HT is caused by antithyroid antibody-dependent cell-mediated direct toxicity linked to deficiency in antigen-specific suppressor T lymphocytes. The gland shows lymphocyte infiltration with follicular cell hyperplasia. Thyroid antibodies are elevated. Radionuclear scans show absent uptake. Initially the child develops elevated thyroid hormones (T3 and T4) followed by symptomatic hypothyroidism. Following the hypothyroid phase there is final recovery in most patients. Indications for surgery in HT include: 1- firm enlargement of the gland causing tracheal compression with dyspnea, hoarseness or swallowing difficulties, 2- failure to respond to suppressive therapy and development of symptomatic hyperthyroid goiter, and 3- development and enlargement of a solitary thyroid nodule. The incidence of malignancy in HT is low. Differentiating a hyperplastic follicular cell nodule from a follicular neoplasm is very difficult using fine needle aspiration biopsy. Patient with malignant nodules in Hashimoto glands are most commonly papillary, females, low frequency of extrathyroidal invasion and nodal metastasis with absent distal metastasis. It is believed the lymphocytic infiltration of HT causes a form of immune reaction to control tumor growth and proliferation.
I. Parotid Hemangioma
Parotid hemangioma (or hemangioendothelioma) is by far the most common tumor of the parotid gland seen in infants and children. Initially the infant presents with non-tender swelling of the cheek during the first weeks of life. The swelling is generally confined to the superficial lobe of the parotid gland, but it can involve the masseter muscle. With capillary and bluish involvement of the skin and subcutaneous tissue the diagnosis is easier to establish. MRI is the investigation of choice because of picture quality, definition of soft tissues and lack of exposure to ionizing radiation. MRI allows a definite diagnosis to be made without any invasive procedure being required. When in doubt a fine-needle biopsy will establish the histologic nature of the mass. US with Doppler imaging (lobular internal structure, fine echogenic internal septations, mildly lobulated contour and extremely high vascularity), and labeled red cell scintigraphy (well-defined area of intense activity) can also sustain the diagnosis of parotid hemangioma. Management is conservative since most lesions involute spontaneously. During involution ulceration and calcification can occur. Medical management (intralesional injection of steroids, systemic steroids or interferon) is given when the tumor is large, deforming, ulcerated, or involves nearby structures with functional consequences. The overall response rate is very high.
J. Congenital Tracheal Stenosis
Congenital tracheal stenosis (CTS) is a rare condition seen immediately after birth or in early infancy that is uniformly life-threatening. Infants with CTS presents with stridor, respiratory distress, recurrent pulmonary infections or failure to thrive. Inflammation of the mucosa or mucous accumulation can easily obstruct the already stenotic airway. The stenosis includes a short or long segment of circular cartilaginous ring. Diagnosis is established using bronchoscopy, MRI (assessment of vascular structures and relation to the stenosis), or CT-scan (good anatomic delineation of the airway). Each individual malformation is studied using rigid tracheo-broncho- esophagoscopy. Associated cardiac defects should rule out with echocardiogram. Management of CTS is surgical. Selection of the type of treatment depends on the patient's clinical status and the anatomic pattern of the stenosis. Resection of a short stenosis with anastomosis can be possible with a length that does not exceed half of the trachea. For longer stenosis the most useful technique consists of enlargement tracheoplasty with cartilaginous or a pericardial graft or the more recent and slide-tracheoplasty. The latter technique is preferable because it preserves native tracheal tissue with fewer postop complications. For CTS one should always look for other associated thoracic malformations, such as a pulmonary sling which may compromise the results of the surgical correction of the tracheal stenosis. Best prognosis is obtained with simultaneous correction of the respiratory and cardiovascular malformation. Overall survival of these children is 75%.
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