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Treatment of macroglossia in a child with Beckwith-Wiedemann syndrome
J Oral Maxillofac Surg 58:1058-1061, 2000
Treatment of Macroglossia in a Child With Beckwith-Wiedemann Syndrome Pedro Diz Dios, MD, DDS,* Jacobo Limeres Posse, DDS,† Jacinto Ferna ´ ndez Sanroma ´ n, MD,‡ and Emma Va ´ zquez Garcı´a, DDS§ In 1963, Beckwith1 presented a report entitled “Extreme Cytomegaly of the Adrenal Fetal Cortex, * Special Needs Unit, School of Medicine and Dentistry, Santiago de Compostela University, Spain. † Special Needs Unit, School of Medicine and Dentistry, Santiago de Compostela University, Spain. ‡ Oral and Maxillofacial Surgery Department, Policlı´nico Vigo S.A. (POVISA), Vigo, Spain. § Special Needs Unit, School of Medicine and Dentistry, Santiago de Compostela University, Spain. Address correspondence and reprint requests to Dr Dios: C/Panama´ 2; 2° Drcha, 36203 Vigo (Spain); e-mail: [email protected] © 2000 American Association of Oral and Maxillofacial Surgeons
0278-2391/00/5809-0020$3.00/0 doi:10.1053/joms.2000.8753
Hyperplasia of the Kidneys and Pancreas, and Leydig-Cell Hyperplasia: A New Syndrome?” Later, Wiedemann2 completed the description of the Beckwith-Wiedemann syndrome (BWS) by adding umbilical hernia and macroglossia. The prevalence of BWS is estimated to be about 1 in every 17,000 births.3 Most of these cases are isolated, although there have been some detected familial examples, which suggests an autosomic dominant, polygenic, or late mutational pattern.4,5 Usually there are no karyotypic changes, but several cases of BWS have been described in which abnormalities in the chromosome 11 short arm were detected.6 Diagnosis is carried out by use of the clinical signs: omphalocele, macroglossia, and somatic gigantism (OMG),7,8 as well as congenital asymmetry (hemihy-
1059
DIOS ET AL
pertrophy), visceromegaly (mainly liver, kidney, and pancreas), and hypoglycemia at birth. Although visceromegaly does not cause either morbidity or mortality in these patients, it has been linked with the appearance of malignant tumors, such as neuroblastoma, hepatoblastoma, renal medullar dysplasia, Wilms tumor, and, less frequently, lymphoma or rhabdomyosarcoma.9 At the craniofacial level, microcephaly, capillary hemangioma on the forehead, earlobe anomalies, exophthalmos, and flattened nasal dorsum may be present.10 Some authors8,9 have described other less common manifestations such as cleft palate and bifid uvulae, although the most relevant characteristic is macroglossia because of its high frequency (82% to 95%) and its potential aesthetic and functional implications, as well as the fact that it constitutes a major finding for establishing the diagnosis of BWS.3,11 In this report, a typical case of BWS with severe macroglossia is described, and aspects related to its treatment combining physiotherapy and surgery are discussed.
Report of Case A 9-month-old child was admitted to the School of Dentistry at the University of Santiago de Compostela (Spain) to be evaluated for macroglossia. The patient was a male born in the 37th week of gestation from a 25-year-old primiparous mother without any noteworthy personal or family history. Macrostomia, muscular hypotony, omphalocele, bilateral cryptorchid, alterations of both earlobes, and macroglossia were observed during the physical examination at birth. The most significant findings in the complementary tests were hypoglycemia, which remitted in 24 hours; symmetric myocardial hypertrophy; and a normal karyotype. A diagnosis of BWS was made. On oral examination, hypotony of the perioral muscles, a prominent tongue that hung over the lower lip, and severe drooling that required changing the bib every 5 to 10 minutes were observed (Fig 1). Despite the size of the tongue, there was no severe difficulty in sucking and swallowing. It was therefore decided to start oral physiotherapy by using the Castillo-Morales technique.12,13 This method uses a combination of massage exercises and an oral stimulation plate in the orofacial area. The special physiotherapeutic exercise program has 2 components: the first is called a basic muscle activation exercise, and the second consists of stimulating 7 anatomic facial points by pressure, traction, and vibration as described earlier.12 The plate, which is made of thermopolymerizable acrylic, has a hollow cylinder at the junction between the posterior one third and anterior two thirds of the external surface and a thicker part in the anterior vestibular area, together with light grooves that stimulate the orbicularis muscles of the lips and the perioral muscles (Fig 2). Once the plate is placed on the palate, there is an immediate reaction because of the introduction of a foreign body in the mouth (Weiffenbach reflex) (Fig 3). The tongue, when pressing on the palatal cylinder, generates a backward and upward force that counteracts the forward and downward position caused by the muscu-
FIGURE 1. Patient at age 9 months, showing the prominent tongue and severe drooling.
lar hypotony and keeps it in the oral cavity. The plate is placed twice per day, first for 5 minutes, and then the time is progressively increased until it reaches 15 minutes per session. After 1 month of treatment, a highly significant reduction in drooling was observed. Four months later, the resting position and function of the tongue had improved, although the patient still had some difficulty in swallowing. The plate was used for a period of 9 months, after which instability attributable to eruption of the teeth made it impossible to use it further. The plate had previously been replaced twice for the same reason. During this period, and up to 3 years of age, the patient underwent daily orofacial physiotherapy exercises that were performed by the mother who had been trained to use the Castillo-Morales method.13 At 3.5 years, because of development of a progressive anterior open bite secondary to tongue prolapse, it was decided to perform a partial glossectomy. At that time, preoperative oral function (swallowing and speech intelligibility) was evaluated as previously described.14 Resection of the tongue was performed with general anesthesia, by use of the Egyedi and Obwegeser technique10 (Fig 4). No complications were observed during or after surgery. An evaluation of oral function performed 4 months later showed that the surgical reduction of tongue volume had improved the swallowing and also made speech more intelligible.
Discussion The diagnosis of macroglossia is based on subjective clinical criteria such as the morphology and amount of protrusion of the tongue, difficulty in articulating sounds, breathing, and drooling. Some authors15 have suggested that the tongue size may be analyzed radiographically with a cephalogram, although they admit that this technique is not without its inconveniences. Other, more sophisticated imaging techniques have proved useful for analyzing tongue morphology and structure,16 although up until now epidemiologic studies have not been published
1060
FIGURE 2. Castillo-Morales stimulation plate. 1. Palatal hollow cylinder to provoke Weiffenbach reflex. 2. Thicker part in the anterior vestibular area with light grooves to stimulate the orbicularis muscles.
on the size of the tongue in relation to the oral cavity. As a consequence, the treatment of macroglossia has led to considerable controversy, because there are no objective diagnostic criteria. Furthermore, there are few long-term longitudinal studies in which the effects of untreated macroglossia have been discussed. However, 3 fundamental types of complications resulting from macroglossia have been described, which represent selection criteria for surgical treatment: dentoskeletal problems such as mandibular prognathism, increase in the gonion angle and anterior facial height, development of an anterior open bite and excessive proclination of the lower incisors; functional deficits such as difficulty in swallowing, drooling, alterations in speech,4,17-19 obstruction of the upper airway,10 and psychologic consequences derived from the patient’s physical appearance, where the prominent tongue, bad pronunciation, and
FIGURE 3. Tongue repositioning once the plate has been fitted on the palate (Weiffenbach reflex).
MACROGLOSSIA IN BECKWITH-WIEDEMANN SYNDROME
drooling may give a false impression of mental deficiency.20,21 The most common techniques for partial glossectomy are wedge resection (horizontal or vertical), marginal resection, amputation of the tip of the tongue, or a combination of the three.21 In some patients the macroglossia spontaneously corrects itself during development10 because of different causes, such as the catch-up of the orofacial growth or repositioning of the base of the tongue.11,15 This raises the question of whether surgery should be performed in the first few years of life in cases of mild macroglossia. Rimell and Shapiro10 found that most obstructions of the airway that are seen in early infancy are attributable to lengthening of the base of the tongue and, in later childhood, are most likely the result of tonsil and adenoid hypertrophy. Airway obstruction in these cases would not be made any better by anterior surgical tongue reduction. All of the authors who favor surgical management of macroglossia suggest that this should be performed early.18,20,21 The main disadvantages are derived from
FIGURE 4. Technique of partial glossectomy. A, Tongue resection design (Egydi and Obwegeser technique); B, Reapproximation of tongue flaps.
1061
DIOS ET AL
the general anesthesia, which in some cases requires a tracheotomy,22 and the need to maintain a nasal tube for several days after the surgical procedure.18 Oral regulation therapy as described by CastilloMorales has been used in Down syndrome and cerebral palsy children, with encouraging results.23,24 Secondary effects such as drooling and increasing tongue protrusion are infrequent and can be rectified by discontinuing treatment. To our knowledge, this technique has not been applied previously in patients with primary macroglossia syndromes such as BWS. Although severe morphologic or functional macroglossia represents an urgent indication for surgery, in less severe cases one should consider delaying surgical treatment, which also would reduce the inherent risks of general anesthesia. In those patients with slight macroglossia, orofacial therapy, as previously described, in combination with changing the consistency of the diet and using special feeding nipples,10,15 could help motor control of the tongue, reduce drooling, and improve swallowing activity.
7.
8. 9.
10.
11. 12.
13. 14.
15.
16.
17.
References 1. Beckwith JB: Extreme cytomegaly of the adrenal fetal cortex, hyperplasia of the kidneys and pancreas, and Leydig-cell hyperplasia: another syndrome? Personal comunication. Annual Meeting of Western Society for Pediatric Research, Los Angeles, CA, November 1963 2. Wiedemann HR: Complexe malformatif familiar avec hernie umbilicale et macroglosie un “syndrome noveau”? J Hum Genet 13:223, 1964 3. Engstro ¨ m W, Lindham S, Schofield P: Wiedemann-Beckwith syndrome. Eur J Pediatr 147:450, 1988 4. Lubinsky M, Hermann J, Kosseff A, et al: Autosomal dominant sex dependent transmission of the Beckwith-Wiedemann syndrome. Lancet 1:932, 1984 5. Niikawa N, Ishikariyama S, Takahashi S: The Beckwith-Wiedemann studies on five families with evidence for autosomal dominant inheritance with variable expressity. Am J Med Genet 24:41, 1986 6. Waziri M, Patil SR, Hanson JW, et al: Abnormality of cromo-
18. 19.
20.
21.
22. 23.
24.
some 11 in patients with features of Beckwith-Wiedemann syndrome. J Pediatr 102:873, 1983 McManamny DS, Barnett JS: Macroglossia as a presentation of the Beckwith-Wiedemann syndrome. Plast Reconstr Surg 75: 170, 1985 Takato T, Kamei M: Cleft palate in the Beckwith-Wiedemann syndrome. Ann Plast Surg 22:347, 1989 Sotelo-Avila C, Gonzalez-Crussi F, Fowler JW: Complete and incomplete forms of the Beckwith-Wiedemann syndrome: Their oncogenic potencial. J Pediatr 96:47, 1980 Rimell FL, Shapiro AM: Head and neck manifestations of the Beckwith-Wiedemann syndrome. Otolaryngol Head Neck Surg 113:262, 1995 Filippi G, McKusick VA: The Beckwith-Wiedemann syndrome. Medicine 49:279, 1970 Limbrock GJ, Castillo-Morales R, Hoyer H, et al: The CastilloMorales approach to orofacial pathology in Down syndrome. Int J Orofac Myol 19:30, 1993 Castillo-Morales R: Die Orofaciale Regulationstherapie. Mu ¨ nchen, Germany, Pflaum Verlag, 1991. Diz Dios P, Castro Ferreiro M, Alvarez Alvarez FJ, et al: Functional consequences of partial glossectomy. J Oral Maxillofac Surg 52:12, 1994 Friede H, Figueroa A: The Beckwith-Wiedemann syndrome: A longitudinal study of the macroglossia and dentofacial complex. J Craniofac Genet Dev Biol 1:179, 1985 McKenna KM, Jabour BA, Lufkin RB, et al: Magnetic resonance imaging of the tongue and oropharynx. Top Magn Reson Imagin 2:49, 1990 Patterson GT, Ramasastry SS, Davis JU: Macroglossia and ankiloglossia in Beckwith-Wiedemann syndrome. Oral Surg Oral Med Oral Pathol 65:29, 1988 Kveim M, Fisher JC, Jones KL, et al: Early tongue resection for Beckwith-Wiedemann macroglossia. Ann Plast Surg 14:142, 1985 Klaiman P, Witzel A, Margar-Bacal F, et al: Changes in aesthetic appearance intelligibility of speech after partial glossectomy in patient with Down syndrome. Plast Recontr Surg 82:403, 1988 Menard RM, Delarie J, Schendel SA: Treatment of the craniofacial complications of Beckwith-Wiedemann syndrome. Plast Reconstr Surg 96:27, 1995 Siddiqui A, Pensler JM: The efficacy of tongue resection in treatment of syntomatic macroglossia in the child. Ann Plast Surg 25:14, 1990 Murthy P, Laing MR: Macroglossia. BMJ 309:1386, 1994 Hohoff A, Ehmer U: Short term and long term results after treatment with the Castillo Morales stimulating plate: A longitudinal study. J Orofac Orthop 60:2, 1999 Fischer-Brandies H, Avalle C, Limbrock GJ: Therapy of orofacial dysfunctions in cerebral palsy according to Castillo-Morales: First results of a new treatment concept. Eur J Orthod 9:139, 1987
Treatment of Macroglossia in a Child With Beckwith-Wiedemann Syndrome Pedro Diz Dios, MD, DDS,* Jacobo Limeres Posse, DDS,† Jacinto Ferna ´ ndez Sanroma ´ n, MD,‡ and Emma Va ´ zquez Garcı´a, DDS§ In 1963, Beckwith1 presented a report entitled “Extreme Cytomegaly of the Adrenal Fetal Cortex, * Special Needs Unit, School of Medicine and Dentistry, Santiago de Compostela University, Spain. † Special Needs Unit, School of Medicine and Dentistry, Santiago de Compostela University, Spain. ‡ Oral and Maxillofacial Surgery Department, Policlı´nico Vigo S.A. (POVISA), Vigo, Spain. § Special Needs Unit, School of Medicine and Dentistry, Santiago de Compostela University, Spain. Address correspondence and reprint requests to Dr Dios: C/Panama´ 2; 2° Drcha, 36203 Vigo (Spain); e-mail: [email protected] © 2000 American Association of Oral and Maxillofacial Surgeons
0278-2391/00/5809-0020$3.00/0 doi:10.1053/joms.2000.8753
Hyperplasia of the Kidneys and Pancreas, and Leydig-Cell Hyperplasia: A New Syndrome?” Later, Wiedemann2 completed the description of the Beckwith-Wiedemann syndrome (BWS) by adding umbilical hernia and macroglossia. The prevalence of BWS is estimated to be about 1 in every 17,000 births.3 Most of these cases are isolated, although there have been some detected familial examples, which suggests an autosomic dominant, polygenic, or late mutational pattern.4,5 Usually there are no karyotypic changes, but several cases of BWS have been described in which abnormalities in the chromosome 11 short arm were detected.6 Diagnosis is carried out by use of the clinical signs: omphalocele, macroglossia, and somatic gigantism (OMG),7,8 as well as congenital asymmetry (hemihy-
1059
DIOS ET AL
pertrophy), visceromegaly (mainly liver, kidney, and pancreas), and hypoglycemia at birth. Although visceromegaly does not cause either morbidity or mortality in these patients, it has been linked with the appearance of malignant tumors, such as neuroblastoma, hepatoblastoma, renal medullar dysplasia, Wilms tumor, and, less frequently, lymphoma or rhabdomyosarcoma.9 At the craniofacial level, microcephaly, capillary hemangioma on the forehead, earlobe anomalies, exophthalmos, and flattened nasal dorsum may be present.10 Some authors8,9 have described other less common manifestations such as cleft palate and bifid uvulae, although the most relevant characteristic is macroglossia because of its high frequency (82% to 95%) and its potential aesthetic and functional implications, as well as the fact that it constitutes a major finding for establishing the diagnosis of BWS.3,11 In this report, a typical case of BWS with severe macroglossia is described, and aspects related to its treatment combining physiotherapy and surgery are discussed.
Report of Case A 9-month-old child was admitted to the School of Dentistry at the University of Santiago de Compostela (Spain) to be evaluated for macroglossia. The patient was a male born in the 37th week of gestation from a 25-year-old primiparous mother without any noteworthy personal or family history. Macrostomia, muscular hypotony, omphalocele, bilateral cryptorchid, alterations of both earlobes, and macroglossia were observed during the physical examination at birth. The most significant findings in the complementary tests were hypoglycemia, which remitted in 24 hours; symmetric myocardial hypertrophy; and a normal karyotype. A diagnosis of BWS was made. On oral examination, hypotony of the perioral muscles, a prominent tongue that hung over the lower lip, and severe drooling that required changing the bib every 5 to 10 minutes were observed (Fig 1). Despite the size of the tongue, there was no severe difficulty in sucking and swallowing. It was therefore decided to start oral physiotherapy by using the Castillo-Morales technique.12,13 This method uses a combination of massage exercises and an oral stimulation plate in the orofacial area. The special physiotherapeutic exercise program has 2 components: the first is called a basic muscle activation exercise, and the second consists of stimulating 7 anatomic facial points by pressure, traction, and vibration as described earlier.12 The plate, which is made of thermopolymerizable acrylic, has a hollow cylinder at the junction between the posterior one third and anterior two thirds of the external surface and a thicker part in the anterior vestibular area, together with light grooves that stimulate the orbicularis muscles of the lips and the perioral muscles (Fig 2). Once the plate is placed on the palate, there is an immediate reaction because of the introduction of a foreign body in the mouth (Weiffenbach reflex) (Fig 3). The tongue, when pressing on the palatal cylinder, generates a backward and upward force that counteracts the forward and downward position caused by the muscu-
FIGURE 1. Patient at age 9 months, showing the prominent tongue and severe drooling.
lar hypotony and keeps it in the oral cavity. The plate is placed twice per day, first for 5 minutes, and then the time is progressively increased until it reaches 15 minutes per session. After 1 month of treatment, a highly significant reduction in drooling was observed. Four months later, the resting position and function of the tongue had improved, although the patient still had some difficulty in swallowing. The plate was used for a period of 9 months, after which instability attributable to eruption of the teeth made it impossible to use it further. The plate had previously been replaced twice for the same reason. During this period, and up to 3 years of age, the patient underwent daily orofacial physiotherapy exercises that were performed by the mother who had been trained to use the Castillo-Morales method.13 At 3.5 years, because of development of a progressive anterior open bite secondary to tongue prolapse, it was decided to perform a partial glossectomy. At that time, preoperative oral function (swallowing and speech intelligibility) was evaluated as previously described.14 Resection of the tongue was performed with general anesthesia, by use of the Egyedi and Obwegeser technique10 (Fig 4). No complications were observed during or after surgery. An evaluation of oral function performed 4 months later showed that the surgical reduction of tongue volume had improved the swallowing and also made speech more intelligible.
Discussion The diagnosis of macroglossia is based on subjective clinical criteria such as the morphology and amount of protrusion of the tongue, difficulty in articulating sounds, breathing, and drooling. Some authors15 have suggested that the tongue size may be analyzed radiographically with a cephalogram, although they admit that this technique is not without its inconveniences. Other, more sophisticated imaging techniques have proved useful for analyzing tongue morphology and structure,16 although up until now epidemiologic studies have not been published
1060
FIGURE 2. Castillo-Morales stimulation plate. 1. Palatal hollow cylinder to provoke Weiffenbach reflex. 2. Thicker part in the anterior vestibular area with light grooves to stimulate the orbicularis muscles.
on the size of the tongue in relation to the oral cavity. As a consequence, the treatment of macroglossia has led to considerable controversy, because there are no objective diagnostic criteria. Furthermore, there are few long-term longitudinal studies in which the effects of untreated macroglossia have been discussed. However, 3 fundamental types of complications resulting from macroglossia have been described, which represent selection criteria for surgical treatment: dentoskeletal problems such as mandibular prognathism, increase in the gonion angle and anterior facial height, development of an anterior open bite and excessive proclination of the lower incisors; functional deficits such as difficulty in swallowing, drooling, alterations in speech,4,17-19 obstruction of the upper airway,10 and psychologic consequences derived from the patient’s physical appearance, where the prominent tongue, bad pronunciation, and
FIGURE 3. Tongue repositioning once the plate has been fitted on the palate (Weiffenbach reflex).
MACROGLOSSIA IN BECKWITH-WIEDEMANN SYNDROME
drooling may give a false impression of mental deficiency.20,21 The most common techniques for partial glossectomy are wedge resection (horizontal or vertical), marginal resection, amputation of the tip of the tongue, or a combination of the three.21 In some patients the macroglossia spontaneously corrects itself during development10 because of different causes, such as the catch-up of the orofacial growth or repositioning of the base of the tongue.11,15 This raises the question of whether surgery should be performed in the first few years of life in cases of mild macroglossia. Rimell and Shapiro10 found that most obstructions of the airway that are seen in early infancy are attributable to lengthening of the base of the tongue and, in later childhood, are most likely the result of tonsil and adenoid hypertrophy. Airway obstruction in these cases would not be made any better by anterior surgical tongue reduction. All of the authors who favor surgical management of macroglossia suggest that this should be performed early.18,20,21 The main disadvantages are derived from
FIGURE 4. Technique of partial glossectomy. A, Tongue resection design (Egydi and Obwegeser technique); B, Reapproximation of tongue flaps.
1061
DIOS ET AL
the general anesthesia, which in some cases requires a tracheotomy,22 and the need to maintain a nasal tube for several days after the surgical procedure.18 Oral regulation therapy as described by CastilloMorales has been used in Down syndrome and cerebral palsy children, with encouraging results.23,24 Secondary effects such as drooling and increasing tongue protrusion are infrequent and can be rectified by discontinuing treatment. To our knowledge, this technique has not been applied previously in patients with primary macroglossia syndromes such as BWS. Although severe morphologic or functional macroglossia represents an urgent indication for surgery, in less severe cases one should consider delaying surgical treatment, which also would reduce the inherent risks of general anesthesia. In those patients with slight macroglossia, orofacial therapy, as previously described, in combination with changing the consistency of the diet and using special feeding nipples,10,15 could help motor control of the tongue, reduce drooling, and improve swallowing activity.
7.
8. 9.
10.
11. 12.
13. 14.
15.
16.
17.
References 1. Beckwith JB: Extreme cytomegaly of the adrenal fetal cortex, hyperplasia of the kidneys and pancreas, and Leydig-cell hyperplasia: another syndrome? Personal comunication. Annual Meeting of Western Society for Pediatric Research, Los Angeles, CA, November 1963 2. Wiedemann HR: Complexe malformatif familiar avec hernie umbilicale et macroglosie un “syndrome noveau”? J Hum Genet 13:223, 1964 3. Engstro ¨ m W, Lindham S, Schofield P: Wiedemann-Beckwith syndrome. Eur J Pediatr 147:450, 1988 4. Lubinsky M, Hermann J, Kosseff A, et al: Autosomal dominant sex dependent transmission of the Beckwith-Wiedemann syndrome. Lancet 1:932, 1984 5. Niikawa N, Ishikariyama S, Takahashi S: The Beckwith-Wiedemann studies on five families with evidence for autosomal dominant inheritance with variable expressity. Am J Med Genet 24:41, 1986 6. Waziri M, Patil SR, Hanson JW, et al: Abnormality of cromo-
18. 19.
20.
21.
22. 23.
24.
some 11 in patients with features of Beckwith-Wiedemann syndrome. J Pediatr 102:873, 1983 McManamny DS, Barnett JS: Macroglossia as a presentation of the Beckwith-Wiedemann syndrome. Plast Reconstr Surg 75: 170, 1985 Takato T, Kamei M: Cleft palate in the Beckwith-Wiedemann syndrome. Ann Plast Surg 22:347, 1989 Sotelo-Avila C, Gonzalez-Crussi F, Fowler JW: Complete and incomplete forms of the Beckwith-Wiedemann syndrome: Their oncogenic potencial. J Pediatr 96:47, 1980 Rimell FL, Shapiro AM: Head and neck manifestations of the Beckwith-Wiedemann syndrome. Otolaryngol Head Neck Surg 113:262, 1995 Filippi G, McKusick VA: The Beckwith-Wiedemann syndrome. Medicine 49:279, 1970 Limbrock GJ, Castillo-Morales R, Hoyer H, et al: The CastilloMorales approach to orofacial pathology in Down syndrome. Int J Orofac Myol 19:30, 1993 Castillo-Morales R: Die Orofaciale Regulationstherapie. Mu ¨ nchen, Germany, Pflaum Verlag, 1991. Diz Dios P, Castro Ferreiro M, Alvarez Alvarez FJ, et al: Functional consequences of partial glossectomy. J Oral Maxillofac Surg 52:12, 1994 Friede H, Figueroa A: The Beckwith-Wiedemann syndrome: A longitudinal study of the macroglossia and dentofacial complex. J Craniofac Genet Dev Biol 1:179, 1985 McKenna KM, Jabour BA, Lufkin RB, et al: Magnetic resonance imaging of the tongue and oropharynx. Top Magn Reson Imagin 2:49, 1990 Patterson GT, Ramasastry SS, Davis JU: Macroglossia and ankiloglossia in Beckwith-Wiedemann syndrome. Oral Surg Oral Med Oral Pathol 65:29, 1988 Kveim M, Fisher JC, Jones KL, et al: Early tongue resection for Beckwith-Wiedemann macroglossia. Ann Plast Surg 14:142, 1985 Klaiman P, Witzel A, Margar-Bacal F, et al: Changes in aesthetic appearance intelligibility of speech after partial glossectomy in patient with Down syndrome. Plast Recontr Surg 82:403, 1988 Menard RM, Delarie J, Schendel SA: Treatment of the craniofacial complications of Beckwith-Wiedemann syndrome. Plast Reconstr Surg 96:27, 1995 Siddiqui A, Pensler JM: The efficacy of tongue resection in treatment of syntomatic macroglossia in the child. Ann Plast Surg 25:14, 1990 Murthy P, Laing MR: Macroglossia. BMJ 309:1386, 1994 Hohoff A, Ehmer U: Short term and long term results after treatment with the Castillo Morales stimulating plate: A longitudinal study. J Orofac Orthop 60:2, 1999 Fischer-Brandies H, Avalle C, Limbrock GJ: Therapy of orofacial dysfunctions in cerebral palsy according to Castillo-Morales: First results of a new treatment concept. Eur J Orthod 9:139, 1987