Original article 115 Reduction glossectomy for macroglossia in children Essam E. Elhalaby, Hussam S. Hassan and Hisham A. Almetaher Background/aim Although several surgical techniques functions, especially taste sensation, were not affected have been described for reduction glossectomy in children, by resection. Speech articulation errors were corrected many general pediatric surgeons are still skeptical about in cases that stared speech. One complication occurred in the treatment of patients with macroglossia because of one patient who had undergone a key-hole glossectomy: potential surgical complications. The aim of this study was wound infection, followed by partial wound disruption. to describe our initial surgical experience with reduction Conclusion Partial glossectomy for macroglossia in glossectomy in a series of eight patients with children is both feasible and safe. It results in minimal macroglossia. complications. Many clinical problems caused by the Materials and methods A retrospective file review was pathology improve after surgery repair. The recommended carried out for all patients who underwent reduction surgical technique should be based on the extent of glossectomy during the period from October 2009 to involvement of the tongue. Ann Pediatr Surg 11:115–119 December 2014. Charts were designed to collect the c 2015 Annals of Pediatric Surgery. following data from the patients’ files: age; etiology of Annals of Pediatric Surgery 2015, 11:115–119 macroglossia; and full clinical examination including the functional respiration, deglutition, phonation deficit, and Keywords: Beckwith–Wiedemann syndrome, macroglossia, reduction glossectomy dental occlusion alterations. Pediatric Surgery Department, Tanta University Hospital, Faculty of Medicine, Results The records of eight patients were retrieved. Five Tanta University, Tanta, Egypt patients, in whom macroglossia affected the tongue width Correspondence to Essam E. Elhalaby, MD, Pediatric Surgery Department, and length, underwent peripheral glossectomy, whereas Tanta University Hospital, Faculty of Medicine, Tanta University, Tanta 31111, Egypt two patients, in whom macroglossia affected the tongue in Tel: + 20 10 545 1157, + 20 40 341 2127; all directions, underwent key-hole glossectomy. In one e-mails: [email protected], [email protected] patient in whom only half the tongue was affected, a central Received 9 March 2014 accepted 10 March 2015 longitudinal strip was excised. Motor and sensory Introduction The multiple techniques advocated for tongue reduction Macroglossia is a generalized term used to describe the show that an ideal procedure is yet to emerge. This is tongue that protrudes beyond the teeth during a natural understandable as the condition is relatively rare, with a resting posture [1]. The Myer classification, on the basis of variation in the degree of macroglossia. Also, there the extent of involvement, subdivides macroglossia into remains no consensus on the timing of tongue reduction. generalized or localized [2]. Vogel classification, on the basis The collection of follow-up objective data is also of etiology, divides macroglossia into true or relative [3]. difficult [17]. When a primary disorder of tongue tissue leads to macroglossia, it is termed true and when affected seconda- Several surgical procedures were described for glossect- rily, such as by amyloidosis, it is referred to as relative omy. The surgical techniques may be subdivided into two macroglossia. True macroglossia originates from several groups: glossectomy along the median line and peripheral causes: idiopathic muscular hypertrophy as occurs with glossectomy. Both techniques include the resection of a Beckwith–Wiedemann syndrome [4,5], vascular malforma- tissue portion and the subsequent suture of the tions such as angiomas and lymphangiomas [6], tumors such margins [15]. as dermoid cysts, teratomas, myoblastomas, rhabdomyomas, With the glossectomy procedures, mobility of the tongue sarcomas, fibromas, and plasmocytomas [7], inflammatory will not be significantly decreased [18]. The lateral, disease such as glossitis and Ludwig’s angina [8], allergy- downward, and protrusive movements will usually remain related edemas [9], pharmacologic alterations [10], unchanged, although movement of the tongue cephalad endocrine disorders such as gigantism, acromegaly, myxedema may be somewhat restricted. The more muscle removed [11,12], infective pathoses such as tuberculoma of the from the anterior tongue, the less upward mobility the tongue [13], and dermatosis (pemphigus) [14]. Relative tongue will retain. Taste sensation appears to be macroglossia may occur with Down syndrome [12]. unaltered after glossectomy [18]. Even though the In terms of frequency, the most frequent causes are primary taste buds for sweetness are located in the idiopathic muscular hypertrophy, Down’s syndrome, anterior tongue, the other taste buds (sourness, bitter- lymphangiomas, angiomas, and fibromas [15]. ness, saltiness) seem to be stimulated sufficiently, by sweets, to provide the appropriate sensation [19]. Macroglossia can affect all oral functions such as breathing, chewing, swallowing, and speech [3] and can cause esthetic The aim of this study was to describe our initial surgical problems such as perception of mental retardation, widened experience with reduction glossectomy in a series of eight interdental spaces, and mandibular prognathism [3,16]. patients with macroglossia. 1687-4137 c 2015 Annals of Pediatric Surgery DOI: 10.1097/01.XPS.0000462929.45595.1a Copyright © Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited. 116 Annals of Pediatric Surgery 2015, Vol 11 No 2 Fig. 1 Peripheral reduction glossectomy. (a) Preoperative photo of a 6-month-old female patient with Beckwith–Wiedemann syndrome, (b) marking the peripheral incisions and vertical mattress suture, (c) excision of peripheral tissue, (d and e) peripheral reduction glossectomy is completed, (f) outcome 5 weeks after surgery. Materials and methods technique was made on the basis of the type of The study was designed as a retrospective file review of all macroglossia. In patients with macroglossia affecting patients who underwent reduction glossectomy in Tanta mainly the width of the tongue, peripheral glossectomy University Hospitals and affiliated hospitals during the was performed (Fig. 1). However, in patients with period from October 2009 to December 2014. The study macroglossia affecting all dimensions of the tongue, a was approved by IRB. Charts were designed to collect the key-hole glossectomy was performed (Fig. 2). In one following data from the patients’ files: age; etiology of patient with only half the tongue affected, a central macroglossia; and full clinical examination including the longitudinal strip was excised (Fig. 3). functional respiration, deglutition, phonation deficit (eval- uated by a speech therapist for all children older than 2 Peripheral glossectomy years), and dental occlusion alterations (evaluated by an A marker pen was used to highlight the amount of tissue on oral and maxillofacial surgeon). the tongue periphery on either side to be removed. We In the presence of a musculoskeletal deformity with a have modified the Dingman and Grabb [20] technique by malocclusion, the sequence of surgical intervention was starting with the insertion of a vertical mattress sutures either reduction glossectomy, followed by orthognathic along the incision line to minimize blood loss. The incision surgery, orthognathic surgery, followed by reduction glos- was made just peripheral to the vertical mattress sutures by sectomy, or both in one surgical stage. The first option was a scalpel and then electrocautery was used to complete the used when extensive orthodontics were necessary before excision, taking more tissue from the ventral aspect than orthognathic surgery, and the size of the tongue impeded the dorsal aspect. Wounds were closed using interrupted the required orthodontic movements. The second sequen- polyglactin sutures (Vicryl; Ethicon, New Jersey, USA) cing option was indicated if occlusion instability developed (Fig. 1). after orthodontics and orthognathic surgery. Key-hole glossectomy Surgical technique We used the technique described by Morgan et al. [21]. After the parents had signed an informed consent, the The tongue resection consists of an anterior wedge surgery was planned. The choice of the surgical combined with a posterior circular incision, which gives Copyright © Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited. Reduction glossectomy for macroglossia Elhalaby et al.117 Fig. 2 Central key-hole glossectomy. (a and b) Preoperative photos of a 12-month-old female patient with Beckwith–Wiedemann syndrome, (c) marking the key-hole incision, (d) excision of central tissue, (e) reduction glossectomy is completed, (f) outcome 12 weeks after surgery showing healing after partial dehiscence at the tip of the tongue. the appearance of a key-hole. The incision line was improvement or resolution of respiratory disorders; marked either by a marker pen or by diathermy, ensuring improvement in swallowing and phonation; preservation that the resected area was symmetrical around the of taste, heat and pain sensitivity; and improvement in midline of the tongue to ensure a good lingual contour tongue mobility. and cosmetic result (Fig. 2). The initial resection should be conservative as it is very Results difficult to replace