The oral manifestations of Maroteaux-Lamy syndrome (mucopolysaccharidosis VI): A case report

Alpöz, Ali Rıza; Çöker, Mahmut; Çelen, Elif; Ersın, Nazan; Gökçen, Damla; Diggelenc, Otto P. van; Huijmansc, Jan G.M.

doi:10.1016/j.tripleo.2005.06.023

Cited by 42 publications

(33 citation statements)

References 8 publications

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“…This limitation may be due to the presence of condylar defects. 7,9,14,[19][20][21][22]24,25,27,28,30 Our study found that 75% patients with MPS had a higher incidence of bleeding gums in over 30% of the tooth surfaces, corroborating the marginal gingivitis previously described in MPS patients associated with a poor oral hygiene and limited manual dexterity. [7][8][9]13,19,[21][22][23][24] These findings are possibly related with tissue changes that occur in mucopolysaccharidosis, but also with other factors including difficulty in maintaining oral hygiene, lack of brushing techniques, poor attendance to dentist and lack of collaboration.…”

Section: ■ Discussionsupporting

confidence: 86%

“…8,9,19,22,24,25 Delayed tooth eruption can be explained by the presence of hyperplastic dental follicles due to excessive deposition of collagen and glycosaminoglycans around unerupted teeth 17,18,28,32 and also due to dentigerous cysts with smooth and clearly defined margins, as described by Alpôz et al 28 Through the evaluation of dental occlusion, we showed that MPS patients presented anterior open bite, right and left posterior cross-bites, limitation of mouth opening as well as maxillary compression in comparison to controls. Other studies corroborate these findings such as the study of Turra et al 14 that followed seventy-eight patients with MPS: 84.8% had anterior open bite, 37.9% had crossbite.…”

Section: ■ Discussionsupporting

confidence: 57%

“…8,21,22 Our results showed a greater prevalence of macroglosia and lingual interposition in patients with mucopolysaccharidosis, which is corroborated in the literature by several authors. 7,9,[13][14][15][20][21][22][23][24][26][27][28][29] The macroglossia is due to the deposition of glycosaminoglycans (GAG) in the tongue structure that usually results in an anterior open bite. 7,9,13,15,[19][20][21][22][23][24][25][26][27][28]30,31 The higher prevalence of lingual interposition in patients with MPS may be due to the presence of macroglossia, anterior open bite and strong maxillary compression.…”

Section: ■ Discussionmentioning

confidence: 99%

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Oral manifestations in children with mucopolysaccharidosis

Ponciano¹,

Areias²

2017

Medical Express

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Section: ■ Discussionsupporting

confidence: 86%

Section: ■ Discussionsupporting

confidence: 57%

Section: ■ Discussionmentioning

confidence: 99%

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Oral manifestations in children with mucopolysaccharidosis

Ponciano¹,

Areias²

2017

Medical Express

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“…Progressive upper airway obstruction can be compounded by deformities of the skull or spine, such as a flattened nasal bridge, short neck, high anterior larynx, mandibular abnormalities or abnormal cervical vertebrae (Alpöz et al 2006; Leighton et al 2001; Myer 1991; Simmons et al 2005). Multilevel airway obstruction may occur if upper airway obstruction is accompanied by tracheobronchomalacia or accumulation of GAG in the tracheal mucosa (Ingelmo et al 2011; Leighton et al 2001; Nagano et al 2007; Pelley et al 2007; Shih et al 2002; Sims and Kempiners 2007).…”

Section: Identifying Anaesthetic Risk Factors In Mpsmentioning

confidence: 99%

Anaesthesia and airway management in mucopolysaccharidosis

Walker

Belani

Braunlin

et al. 2012

J of Inher Metab Disea

117

136

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This paper provides a detailed overview and discussion of anaesthesia in patients with mucopolysaccharidosis (MPS), the evaluation of risk factors in these patients and their anaesthetic management, including emergency airway issues. MPS represents a group of rare lysosomal storage disorders associated with an array of clinical manifestations. The high prevalence of airway obstruction and restrictive pulmonary disease in combination with cardiovascular manifestations poses a high anaesthetic risk to these patients. Typical anaesthetic problems include airway obstruction after induction or extubation, intubation difficulties or failure [can’t intubate, can’t ventilate (CICV)], possible emergency tracheostomy and cardiovascular and cervical spine issues. Because of the high anaesthetic risk, the benefits of a procedure in patients with MPS should always be balanced against the associated risks. Therefore, careful evaluation of anaesthetic risk factors should be made before the procedure, involving evaluation of airways and cardiorespiratory and cervical spine problems. In addition, information on the specific type of MPS, prior history of anaesthesia, presence of cervical instability and range of motion of the temporomandibular joint are important and may be pivotal to prevent complications during anaesthesia. Knowledge of these risk factors allows the anaesthetist to anticipate potential problems that may arise during or after the procedure. Anaesthesia in MPS patients should be preferably done by an experienced (paediatric) anaesthetist, supported by a multidisciplinary team (ear, nose, throat surgeon and intensive care team), with access to all necessary equipment and support.Electronic supplementary materialThe online version of this article (doi:10.1007/s10545-012-9563-1) contains supplementary material, which is available to authorized users.

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“…True or relative macroglossia may be distinguished into two main sub‐types: congenital and acquired: Congenital macroglossia might be caused by idiopathic muscular hypertrophy 20 , adenoid hyperplasia 21 , haemangiomas 22 , lymphangiomas 23 , Down syndrome 24 , Beckwith Wiedemann syndrome 25 , Hurler syndrome 26 , Maroteux‐Lamy syndrome 27 , Crouzon syndrome 28 , hypertelorism 29 , Βecker and Duchenne dystrophies 30 , Pompe’s Disease 31 and other causes.…”

Section: Classification: Aetiologymentioning

confidence: 99%

Macroglossia

Topouzelis

Iliopoulos

Kolokitha

2011

International Dental Journal

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Macroglossia is defined as an enlarged tongue and it is usually clinically diagnosed. Pseudomacryglossia concerns a tongue that is of normal size but gives a false impression of being too large in relation to adjacent anatomical structures. The causes of macroglossia are numerous and this is why various classifications have been proposed for this condition. The consequences of macroglossia usually include a possible malfunction of the stomatognathic system, breathing and speech problems, increased mandible size, tooth spacing, diastema and other orthodontic abnormalities. The treatment of macroglossia depends on its aetiology and generally includes correcting the systemic disease underlying the increase in lingual mass, surgical treatment, radiotherapy and treatment of orthodontic abnormalities that might have been caused by the condition.

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The oral manifestations of Maroteaux-Lamy syndrome (mucopolysaccharidosis VI): A case report

Cited by 42 publications

References 8 publications

Oral manifestations in children with mucopolysaccharidosis

Oral manifestations in children with mucopolysaccharidosis

Anaesthesia and airway management in mucopolysaccharidosis

Macroglossia

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