Tongue-Tie (Ankyloglossia) This is a congenital condition in which the lingual frenulum is abnormally short, resulting in reduced mobility of the tip of the tongue ( ▶ Fig. 18.1). The prevalence has been reported to be up to 11%, depending upon the definition and whether the finding is actively sought. It is commoner in males. 1 Diagnosis can be difficult particularly in an infant. The short frenulum restricts passive attempts to elevate the tongue. The parents or carer may have noticed a notch in the tongue or a heart shape to the tongue when the infant attempts to protrude it. In an older cooperative child, there is obvious restriction of protrusion and elevation. There have been some attempts to quantify or grade the degree of tongue-tie but there are obvious difficulties with this in an infant. Fig. 18.1 Tongue-tie. The symptoms of a tongue-tie relate to decreased mobility of the tongue tip. There is much controversy surrounding how much of an impact this has upon functions such as feeding and speech. Tongue tip mobility is required to produce lingual sounds (e.g., t, d, n) and sibilants (e.g., s, z). Children with ankyloglossia usually develop compensatory mechanisms to overcome the reduced mobility that allows them to produce these sounds. The overall effect of tongue-tie on speech is uncertain, and speech and language therapists seem to be divided upon whether or not there is a significant impact upon speech or on whether surgery for tongue-tie improves articulation. 2, 3 There has certainly been an increased awareness of an effect upon breast-feeding in recent years, 4 with many reports of improved outcomes following release of the tight frenulum probably because the infant can then develop an improved “latch” to the breast during feeding. 5, 6 Tongue-tie can also have an impact upon bottle-feeding. Other symptoms are mechanical and social and include the child not being able to clean his/her teeth with his/her tongue, a gap between the lower incisors, which can cause aesthetic concern, inability to protrude the tongue, difficulty playing wind instruments, and inability to “French kiss.” Treatment is only indicated if there are troublesome symptoms (including social ones). Many older children with tongue-tie, especially if the frenulum is only slightly shortened, need no treatment. Presumptive treatment in the newborn to avoid future symptoms is controversial. It is the author’s practice to offer division in the outpatient setting to all infants with a tongue-tie below the age of 6 months, following a discussion with the parents concerning the current evidence base for treatment. The treatment is essentially division of the frenulum (frenotomy), which is more often than not a thin fibrous band. Provided the surgeon is adequately trained and experienced, frenotomy can be performed safely in neonates (and infants) without the need for any local or general anesthesia. In an older child, general anesthesia is required. There have been some case reports of severe complications such as profuse bleeding and even infection leading to Ludwig’s angina when the division was carried out by untrained personnel. 7 In some children, the frenulum can be very thick and a frenuloplasty (horizontal to vertical or Z-plasty) may be indicated. Macroglossia is defined as protrusion of the tongue beyond the incisors in the resting state. In true macroglossia ( ▶ Fig. 18.2) the tongue is enlarged, whereas in pseudomacroglossia, the tongue is of normal size but appears large as it protrudes beyond the incisors. True macroglossia can be: Primary, when there is hypertrophy or hyperplasia of the tongue musculature; or Secondary, where there is infiltration of normal tongue musculature with abnormal elements. Causes of primary macroglossia include Beckwith-Wiedemann’s syndrome and hypothyroidism, whereas secondary macroglossia is caused by lymphatic malformations, hemangioma, neurofibroma, metabolic disorders such as mucopolysaccharidosis, and lipid storage diseases. Pseudomacroglossia may be due to micrognathia or poor muscle control as can occur in children with hypotonia. This is seen in Down’s syndrome, some cases of cerebral palsy, and Pierre Robin sequence (see ▶ 27). It usually improves with age. Symptoms of macroglossia in children include drooling, speech and swallowing difficulties, anterior open bite, ulceration and necrosis of the exposed mucosa, and airway obstruction. Moreover, the appearance can lead to social isolation, teasing, and bullying. Fig. 18.2 Pseudomacroglossia in a newborn. Treacher Collins’ syndrome. Children who present with macroglossia may need investigations under the supervision of a pediatrician. The otolaryngologist may be asked to intervene if there is a significant functional issue such as airway obstruction or a disorder of speech articulation, or to address aesthetic concerns. Children with airway obstruction due to macroglossia or pseudomacroglossia may require airway intervention such as an oropharyngeal (Guedel) airway, endotracheal intubation, or very occasionally tracheostomy particularly in the event of an acute deterioration, for example, during the course of a severe upper respiratory tract infection. Surgical reduction of the tongue (partial glossectomy) under the care of an experienced team with appropriate support facilities is a safe procedure with relatively good results and low morbidity 8, 9 This usually involves removing a wedge of tongue musculature from the anterior part of the tongue ( ▶ Fig. 18.3), often combined with dorsoventral reduction to reduce the thickness. There is a requirement for adjuvant speech and language therapy and also ongoing psychological support. A variety of lesions can present as a swelling on the tongue and may need surgical excision (e.g., pyogenic granuloma; ▶ Fig. 18.4). Fig. 18.3 Wedge excision of the tongue, a rarely needed treatment for macroglossia. Fig. 18.4 Macroglossia in a child with Down’s syndrome. A pyogenic granuloma has developed on the exposed tongue mucosa. A ranula is an extravasation mucocele that arises from the sublingual salivary gland either from a ruptured main duct or from ruptured acini following obstruction ( ▶ Fig. 18.5). The sublingual gland produces a steady flow of mucous even without stimulation. As the mucous extravasates, an inflammatory reaction takes place that creates a fibrotic pseudocapsule. When the extravasation is limited to the floor of the mouth, it leads to a painless swelling in the floor of the mouth. In some cases, the extravasation extends through a hiatus in the mylohyoid muscle into the neck and forms a “plunging” ranula. This presents with a swelling in the neck. Fig. 18.5 Ranula. The diagnosis is clinical. Occasionally, a localized lymphatic malformation can cause confusion, and an ultrasound may help delineate the cyst and rule out rarer causes of intraoral swellings. Treatment is surgical. Incision and drainage alone has been shown to be successful in approximately 25% of neonates treated this way. It may be an acceptable treatment in this age group where more extensive surgery may result in complications associated with bleeding. Incision and drainage combined with marsupialization, that is, the incised edges of the cyst are sutured to adjacent tissue to minimize the risk of the cyst filling up again, gives a lower recurrence risk, but recurrence is least likely to occur when the ipsilateral sublingual gland is excised. 10, 11, 12 Success rates are approximately 95% if the sublingual gland is removed. Complications of this procedure are uncommon but include infection, bleeding, and rarely lingual nerve trauma leading to hypoesthesia of the anterior tongue. Adenoids and tonsils form part of the Waldeyer’s ring of lymphoid tissue in the oropharynx and nasopharynx. The function of these structures remains unclear but is thought to play a role in the development of B cells as part of the immune response. It is thought that this immune function peaks in early childhood and after that involution occurs associated with replacement of lymphoid tissue with fibrous tissue. There has been much discussion on the exact age at which the immune function declines without any real resolution. Biofilms are microbial communities that attach to surfaces and produce their own protective matrix. This enables the microbes to have increased resistance to environmental factors such as extremes of temperature, humidity, and light. This also confers increased resistance to antimicrobial therapy and to phagocytosis. 13 Biofilms have been demonstrated in up to 73% of enlarged tonsils removed from children for both recurrent tonsillitis and obstructive sleep apnea (OSA). 14 Biofilms may contribute to the development of tonsillar hyperplasia. Acute tonsillitis is a common infection of childhood and usually begins with a viral infection that may then lead to an acute bacterial infection. Acute infection causes obstruction or inflammation of the tonsillar crypts, leading to accumulation of debris and multiplication of bacterial flora leading to inflammation and pus exudates. Streptococcus pyogenes is the commonest bacterial organism associated with acute tonsillitis. However, up to 40% of children have positive cultures for this bacterium without evidence of active disease. Symptoms and signs of acute bacterial tonsillitis include swelling, erythema, and exudate on the tonsils ( ▶ Fig. 18.6) in association with pain on swallowing, fever, and cervical lymphadenopathy. Penicillin V is the treatment of choice. Viral tonsillitis can be caused by rhinovirus, adenovirus, enterovirus, and influenza virus. Infection caused by these organisms presents in much the same way as bacterial tonsillitis but usually is not associated with exudate. Infection with Epstein–Barr virus causes enlarged tonsils with a coalescent exudate in association with posterior (often dramatic) cervical lymphadenopathy, fever, fatigue, and hepatosplenomegaly, a condition known as infectious mononucleosis. In this condition, amoxicillin can cause a generalized skin rash and should be avoided. There is a risk of airway obstruction. Fig. 18.6 Acute tonsillitis. Adenotonsillectomy remains one of the commonest surgical procedures performed in children. An increasingly common indication for both adenoidectomy and tonsillectomy in children now is sleep-disordered breathing (see ▶ 19). This chapter will focus primarily upon the other indications for tonsillectomy in children, surgical techniques, perioperative management, complications of surgery, and outcomes. Assessment of a child for (adeno)tonsillectomy begins with a history of one or more of the indications listed in Box 18.1 and Box 18.2. Examination includes assessment of stertor, mouth breathing, and reduced nasal airflow, which are all indicators of nasal obstruction. Some parents will bring videos of their child asleep demonstrating snoring and possible episodes of OSA. The adenoids themselves can only be viewed by using a postnasal mirror, something that is very rarely used and is not well tolerated by children, or nasendoscopy with a rigid or flexible nasendoscope. Tonsils can be assessed by asking the child to open his/her mouth and say “aah.” This depresses the tongue and lifts the soft palate, allowing a view of the oropharynx without the need for a tongue depressor. Occasionally, a tongue depressor or wooden spatula is required to see the tonsils. Note the size and the nature of the uvula (a bifid uvula may be an indicator of a submucous cleft palate). A commonly used grading system for assessing the size of tonsils is the Brodsky Grading Scale. 15 The size of the tonsils are graded from 1 to 4 depending upon the percentage of the oropharyngeal airway that is occupied by the tonsils: Grade 1: ≤ 25%. Grade 2: 26 to 50%. Grade 3: 51 to 75%. Grade 4: > 75%. This scale has been demonstrated to have good inter- and intraobserver reliability. 16 Adenoid size has been a little more difficult to standardize given the difficulty in assessment in an outpatient setting. Attempts have been made using lateral skull X-rays, acoustic rhinometry, and assessment with flexible nasendoscopy intraoperatively. A recently proposed grading system by Parikh et al grades the adenoids as seen in outpatients with an endoscope and may be useful as a standard for reporting clinical outcome studies 17: Grade 1: adenoid tissue not in contact with surrounding structures. Grade 2: adenoid tissue in contact with Eustachian tube cushions. Grade 3: adenoid tissue in contact with vomer. Grade 4: adenoid tissue in contact with soft palate. Adenoidectomy and tonsillectomy are often undertaken together, but the indications for each may be different for each procedure. It is important to assess the need for each on its own merits rather than routinely combining the two, as each is associated with potential morbidity. Box 18.1 Indications for Adenoidectomy Sleep-disordered breathing/OSA (see ▶ 19). Nasal obstruction: enlarged adenoids lead to physical impairment of the nasal airway causing nasal obstruction, nasal discharge, mouth breathing, hyponasal speech, and sleep-disordered breathing. There is some evidence that adenoidal hypertrophy is more likely in children with allergic rhinitis and that treatment with intranasal steroids reduces the size of the adenoids in this group. 18, 19 There is also some evidence that intranasal steroids might benefit “adenoidal” children without allergic rhinitis, although the degree of improvement, the duration of required treatment, and persistence of benefit, and comparison with surgical removal have not yet been reported. 20, 21 Otitis media with effusion (OME): adenoidectomy may have an important role in the management of some children with OME (see ▶ 8). Chronic rhinosinusitis (CRS) as considered in ▶ 17. Box 18.2 Indications for Tonsillectomy Sleep-disordered breathing/OSA. Recurrent acute tonsillitis. Unilateral tonsillar enlargement. Peritonsillar abscess (quinsy). Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS). Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Halitosis secondary to tonsillar crypt debris/tonsilloliths. Until the relatively recent increase in the acceptance of sleep-disordered breathing as an indication for tonsillectomy in children, recurrent tonsillitis was the most common reason for tonsillectomy in this age group. There have been numerous attempts to rationalize criteria for tonsillectomy for this indication in an effort to prevent unnecessary surgery and to ensure the desired outcome of an improvement in quality of life. There are large differences in the adenotonsillectomy rates internationally with low rates in Canada and the highest rates in Northern Ireland. It is unclear why these differences exist and also what the indications for adenotonsillectomy were in different studies. 22 In the United Kingdom, there have been increasing rates of hospital admissions recently with acute throat infections, whereas the tonsillectomy rates have decreased. It is felt that this increase in admissions with sore throats is unrelated to the decreasing adenotonsillectomy rate and is probably due to changing management protocols within the primary care setting. 22 A recent Cochrane review concludes that tonsillectomy in children reduces the number of episodes of sore throat and the number of days with a sore throat. 23 This effect is most marked in severely affected children. 24 It has been shown that tonsillectomy is not an effective treatment for recurrent mild sore throat. 25 The potential benefit has to be weighed against the risks of surgery and against the fact that a significant number of patients with recurrent tonsillitis undergo spontaneous resolution of their symptoms. There are currently multicenter projects underway studying the effect of tonsillectomy upon quality of life in children using the 14-item Paediatric Throat Disorders Outcome Test, which is an appropriate, disease-specific, parent-reported outcome measure for children with throat disorders. 26 From a practical point of view, the following guidelines are used in the author’s practice to determine whether tonsillectomy is indicated for recurrent tonsillitis. These are based on the Scottish Intercollegiate Guidelines Network guidelines and the ENTUK position paper on indications for tonsillectomy. 27 Sore throats are due to acute tonsillitis. The episodes of sore throat are disabling and prevent normal functioning, for example, missing school. Seven or more well-documented, clinically significant, adequately treated sore throats in the preceding year; or Five or more such episodes in each of the preceding 2 years; or Three or more such episodes in each of the preceding 3 years.
18.2.1 Definition and Prevalence
18.2.2 Effects
18.2.3 Management
18.3 Macroglossia
18.3.1 Definition and Classification
18.3.2 Management
18.4 Ranula
18.4.1 Etiology and Presentation
18.4.2 Management
18.5 Adenoids and Tonsils
18.5.1 Applied Physiology
18.5.2 Acute Tonsillitis
18.5.3 Adenotonsillectomy
Indications for Adenotonsillectomy
Sleep-Disordered Breathing/Obstructive Sleep Apnea
Recurrent Acute Tonsillitis
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