Congenital Disorders Congenital anomalies of the salivary glands are very uncommon. Aplasia of the major salivary glands and atresia of their ducts has been described, as has polycystic disease of the parotid glands. Polycystic disease may be unilateral or bilateral and presents with intermittent swelling of the parotid gland, particularly at meal times, only to subside shortly afterward. There is rarely any need for acute treatment of polycystic parotid disease and most cases have little impact on the child’s life. The more common developmental salivary disorder is a mucous retention cyst of the floor of the mouth called a ranula. Occasionally, these cysts are found in the newborn but more typically in toddlers and preschool children. They appear as a superficial translucent swelling in the floor of the mouth with a frog’s belly appearance, hence their name. Ranulae usually lie just to one side of the midline. If sufficiently large, a ranula can extend well into the anterior triangle of the neck, displace the tongue upward, and interfere with swallowing (cervical or “plunging” ranula). Occasionally, these cysts rupture spontaneously or, as a result of unnoticed trauma, release thick viscid mucus. Recurrence is then common. The diagnosis is clinical but imaging (ultrasound, magnetic resonance imaging [MRI], if needed) can demonstrate the limits of the swelling and help to plan treatment, particularly if there is extension into the neck. Surgical management was traditionally by simple generous marsupialization, opening the sac and suturing the edges to the adjacent normal mucosa, but recurrence was not uncommon and most surgeons now advocate a more thorough dissection with delivery and complete excision of the ranula. Many advise excision of the sublingual gland as well to further reduce the risk of recurrence. Hemangiomas may be present at birth, in the first year of life, and certainly before the age of 10 years. Girls are affected more frequently than boys. The tumor presents as a diffuse soft swelling of the parotid gland and, if particularly large, appears bluish. Other salivary glands are hardly ever involved. Spontaneous regression sometimes with phlebolith formation is well recognized. These hemangiomas are partly or predominantly cavernous or are mixed hemangiomas. They follow the expected natural history of hemangiomas in that they grow to a peak in size and then regress, so the long-term prognosis is excellent. Often these tumors progress rapidly in the first year of life and can cause extreme anxiety. Both parents and doctors may fear that the child has an aggressive malignancy. Imaging—ideally with MRI scanning under the supervision of an experienced pediatric radiologist—is essential and has greatly improved management and has reduced the need for biopsy as the diagnosis can usually be made with near certainty on MRI scanning. Parents can then be reassured that this is a benign condition with an excellent prognosis. The discovery that childhood hemangiomas respond to propranolol has transformed the management of this condition (see Chapters ▶ 21 and ▶ 24). Children are now typically managed medically at least until the proliferative phase is over. Many will not require any further intervention, and if surgical treatment is needed at all, it should be delayed for as long as possible and certainly until the child is at least 5 years old. Total excision of parotid hemangiomas is curative, but almost never needed nowadays. The surgeon who undertakes this type of work must have significant expertise. It is not difficult to imagine the difficulties that might be encountered operating on such a vascular tumor in close proximity to the facial nerve. It is important not to confuse parotid hemangiomas with lymphangiomas as the management and expectations in terms of natural history are very different. Local sclerosants are not appropriate for hemangiomas. Systemic corticosteroids have been reported to control rapidly growing hemangiomas, but medical management with propranolol to reduce the proliferative phase of development of these lesions is now widely accepted as best practice. 1 Propranolol is best given under the supervision of a pediatrician with careful monitoring of the cardiovascular effects, particularly in the early stages of treatment. In very aggressive hemangiomas, with particularly aggressive histology and if the lesion is encroaching on important structures (e.g., the orbit), a course of treatment with cytotoxic agents such as vincristine or methotrexate can be considered. This would be under the supervision of a pediatric oncologist. These congenital, often multiloculated, lymphatic malformations (formerly known as cystic hygroma) are commonly found in the neck and frequently involve or infiltrate the parotid and submandibular salivary glands. Two main types, macrocystic and microcystic, are recognized, but mixed types are also seen. The cysts contain lymph and are now recognized as part of a spectrum of vascular malformations. They are not true neoplasms (see ▶ 24). Lymphangiomas tend to increase in size over time and are subject to repeated episodes of infection that require antibiotic therapy and take a considerable time to respond. Management is discussed in ▶ 24. Essentially, it is either by expectant treatment, surgery, or sclerotherapy, often under the supervision of an interventional radiologist, or by a combination of any of these strategies. A number of injectable chemical agents, for example, biological products such as OK432 (see Giguere et al) 2 or alcohol, bleomycin, and doxycycline, have been used in attempts to cause sclerosis. They can be quite successful in macrocystic but less so in microcystic disease, presumably because they do not disperse evenly throughout the often multiple small cysts. Injection is best given under the careful supervision of an interventional radiologist with good quality imaging and will usually require general anesthesia. For large lesions, a vigorous inflammatory reaction can ensue in the early postoperative period and the child may need to be managed in a high dependency unit or on occasion in a pediatric intensive care unit with endotracheal intubation. Surgery can be extremely challenging. These lesions do not respect tissue planes and it is easy to inflict cranial nerve palsies ( ▶ Fig. 26.1). Fig. 26.1 An extensive macrocystic lymphangioma in the deep lobe of the right parotid gland. The facial nerve needed to be mobilized to remove the lesion. Congenital anomalies of the head and neck, including lymphangiomas, are sometimes diagnosed by prenatal maternal ultrasonography, supplemented as needed by MRI scanning. They can be extremely large and may threaten the integrity of the airway. Mothers of babies with large lymphangiomas detected at prenatal scanning are best delivered at major tertiary centers. The newborn baby’s airway can be secured while the placental circulation is still functioning, either by endotracheal intubation or, in extreme cases, by tracheotomy. This technique, ex utero intrapartum treatment (EXIT), can permit definitive treatment of babies who would otherwise not survive much beyond birth. 3 First pharyngeal or “branchial” arch anomalies are uncommon and may present with a cutaneous sinus or fistula on the face or neck beneath the pinna overlying the parotid gland, usually between the tragus and the angle of the jaw. The superior opening, if present, may be either in front of the tragus or in the floor of the external auditory canal, usually at the junction of cartilaginous and bony part. While the superficial pits are noticeable at birth, they become more prominent and noticeable with each and every subsequent episode of infection. Aural discharge synchronous with purulent discharge from the cutaneous opening indicates that there is an opening within the external ear canal (see ▶ 24). As recurrent infections become more frequent, surgical resection of the fistulous track or sinus becomes inevitable. A lazy “S” parotidectomy incision and approach may be used as the track runs a variable course often deep to the inferior division and branches of the facial nerve. Frequent infections can make dissection from the nerve difficult and increase the likelihood of facial nerve morbidity. As with all parotid surgery, intraoperative facial nerve monitoring should be mandatory. The internal opening of the fistula must be removed as well and occasionally is found to open into a duplicate cartilaginous canal lying inferior to the external auditory canal ( ▶ Fig. 26.2). Fig. 26.2 Resection of first branchial arch anomalies. The fistulous external opening has been marked. The scissors point to the internal opening within the external auditory canal that has been excised in continuity with the anomaly. The lower division of the main trunk of the facial nerve can be seen running immediately posterior and deep to the track. Like other exocrine mucus-secreting glands, salivary glands are affected by this autosomal recessive disease. Abnormal mucus produced by the glands obstructs their ducts and causes progressive damage to the related parenchyma. The parotid gland, which mainly produces serous secretion, is less affected than the submandibular gland. These mucoceles are relatively common and usually caused by trauma that tears the duct of a minor salivary gland allowing mucus to extravasate into the surrounding tissues. The commonest site is the lower lip, but these cysts are sometimes found in buccal mucosa or in the floor of the mouth. They present as soft, dome-shaped, swellings with a bluish hue. They are rarely more than a centimeter in diameter. Mucous extravasation cysts inevitably require excision as they do not resolve spontaneously. Though much more common in adults, particularly the elderly, salivary calculi may occasionally develop in children and be the cause of duct obstruction. The submandibular gland is the most frequently affected gland and this is thought to be due to its relatively mucoid secretion and the higher calcium concentration of its saliva. The child may complain of intermittent pain that gets worse at mealtimes, making them reluctant to eat certain foods that provoke increased salivation. There may be a visible swelling in the submandibular triangle that slowly subsides. The gland will be tender to palpate and the calculus is often visible in the floor of the mouth close to the duct orifice. If the gland becomes infected, pus may exude from the duct, and a local lymphadenopathy may be apparent. Salivary gland calculi are normally radiopaque and can be imaged using standard plain film techniques. If it is possible to milk the calculus toward the duct orifice, relief of obstruction can be achieved by a simple ductotomy or dilatation. In the young, this might necessitate a general aaesthetic, but older children could be amenable to this procedure under local anesthetic. In adults, calculi deeper in the gland can be retrieved using endoscopic techniques. At present, endoscopic techniques are being used to manage juvenile recurrent parotitis and this minimally invasive method is increasingly applied to calculus retrieval. 4, 5, 6 A number of viruses may involve the salivary glands but the classical cause of viral sialadenitis is mumps caused by the mumps virus, a paramyxovirus, which can affect many glands other than the parotids and also the neural tissue. Infection is probably by droplet spread and is thus very contagious. The incubation period is typically 18 to 21 days. After a prodromal period of malaise and anorexia, there is characteristically swelling of one or both parotid glands and fever. The parotid swelling is tense and painful. Sometimes, the submandibular glands may also be affected. The diagnosis is not difficult and can be made on the basis of clinical signs only, especially when there has been a recent outbreak of the disease. With widespread immunity as a result of measles, mumps, and rubella (MMR) vaccination, mumps is nowadays sometimes not considered in the differential and it may take days to make the diagnosis on the basis of exclusion of other conditions. Simple blood tests will fail to show the characteristic neutrophil leukocytosis that is present with other forms of parotitis, and the precise diagnosis can be confirmed by serology using enzyme-linked immunosorbent assay (ELISA). Mumps is usually a mild, self-limiting disease. If contracted after puberty, approximately 20% of males with mumps develop orchitis, but sterility rarely results. Other possibilities include oophoritis or pancreatitis. Serious complications include aseptic meningitis, encephalitis, and permanent, usually unilateral, profound sensorineural hearing loss. There is no specific treatment, and vaccination against mumps is therefore important for children over 1 year of age to avoid the risk of deafness. In Britain, the recommended preparation is MMR triple vaccine, which is more than 95% effective. Cat-scratch disease is common in the United States and less commonly seen in Europe. The infection follows a scratch or bite from a cat and is caused by the gram-negative bacterium Bartonella henselae or Bartonella quintana. The event that introduced the infection is often not remembered but usually takes place 2 to 4 weeks previously. A small papule or pustule forms at the site and is associated with a regional lymphadenopathy in the neck. The child may develop parotid swelling and it has been known for a facial palsy to develop which can confuse the situation. There is normally mild pyrexia, malaise, muscular aches and pains, decreased appetite, and sometimes conjunctivitis. The infection is usually mild and self-limiting and does not require any specific treatment. Much more serious complications can very rarely include encephalopathy and endocarditis. The diagnosis is made on clinical signs and on the histological appearances of biopsied tissue. More recent tests using the polymerase chain reaction are extremely sensitive. Tuberculosis is a rare cause of sialadenitis that is seen from time to time. The parotid glands are the most commonly affected site. The infection may involve the intraglandular lymph nodes or the gland parenchyma. It is far less common than nontuberculous mycobacteria but needs to be considered, particularly in children at high risk. These include especially those with immune system compromise, and human immunodeficiency virus (HIV) and those from a background of extreme poverty and deprivation. Mycobacterium tuberculosis is the cause of tuberculous sialadenitis. The incidence of this condition is increasing greatly in Europe. The child will present with a tumor-like swelling of the neck, often in the region of the parotid or the submandibular gland. The parotid gland in children has a much higher content of lymphoid tissue, as distinct from salivary parenchymal tissue, than in the adult, hence the tendency for cervical lymphadenopathy in this area. Affected children are generally 18 months to 3 years old. The skin may develop a characteristic violet hue and may break down. Children are usually well with no fever and no pain. Diagnosis is largely clinical but can be confirmed histologically when the characteristic granulomas are seen. Aspiration cytology usually produces a thin green fluid that often shows no more than lymphocytes on cytological examination. Culture is often unhelpful. The condition is entirely benign and self-limiting with inevitable regression of the lesions over a period of a year or so.
26.2.1 Anatomical Anomalies
Aplasia and Dysplasia
Ranula
26.2.2 Congenital Tumors and Hamartomas
Parotid Gland Hemangioma
Lymphangioma
Presentation and Management
Prenatal Diagnosis
26.2.3 Pharyngeal (Branchial) Arch Anomalies
26.2.4 Parotitis in Cystic Fibrosis
26.3 Acquired Salivary Gland Disorders
26.3.1 Salivary Gland Trauma
Mucous Extravasation Cysts
Sialolithiasis (Salivary Gland Stones)
26.3.2 Inflammatory Disorders
Viral Sialadenitis (Mumps)
Cat-Scratch Disease
Granulomatous Disease
Tuberculous Parotitis
Nontuberculous Mycobacteria
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