Srpski arhiv za celokupno lekarstvo 2011 Volume 139, Issue 5-6, Pages: 366-369
https://doi.org/10.2298/SARH1106366K
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Macroglossia and Beckwith-Wiedemann syndrome
Krasić Dragan (Klinika za stomatologiju, Niš)
Radović Predrag (Klinika za stomatologiju, Niš)
Burić Nikola (Klinika za stomatologiju, Niš)
Krasić Dragana (Klinika za mentalno zdravlje, Klinički centar, Niš)
Pešić Zoran (Klinika za stomatologiju, Niš)
Videnović Goran (Klinika za stomatologiju, Medicinski fakultet, Priština)
Introduction. In 1963 Beckwith presented a report on the first patient with
extreme cytomegaly of adrenal cortex, hyperplasia of kidneys and pancreas and
Leydig cell hyperplasia. Wiedemann completed description of the new syndrome
by adding umbilical hernia and macroglossia. The diagnosis is made based on
the clinical signs of omphalocele or some other umbilical deformity,
macroglossia, congenital asymmetry, visceromegaly (liver, pancreas, and
kidneys). Case Outline. A 16-month-old male child was admitted for
examination because of macroglossia. He underwent examination on several
occasions by an endocrinologist due to recurrent hypoglycaemic crisis. The
patient was observed by a paediatric neurophysicatrist for disorders of
mental development. Hypoglycaemia, muscular hypotonia of the anterior
abdominal wall with umbilical hernia and macroglossia were observed by
clinical examination. Inratraoral examination revealed macroglossia with
microstomia, suckling and swallowing difficulties, hypotonia of the
perioral muscles with increased salivation. It was therefore decided to
perform surgical reduction of the prominent tongue and develop good condition
for nutrition, speech function and the development of orofacial system.
Conclusion. 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 hypersalivation. Some
authors have suggested that the tongue size may be analyzed radiographically
with a cephalogram. Treatment of macroglossia is controversial because of the
absence of objective clinical criteria.
Keywords: tongue, reduction, diagnosis, prognosis
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