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Operative Management of OSAS in a Complex Case of Proteus Syndrome.

Cantone E, Cavaliere M, Castagna G, Marino A, Del Vecchio L, Iengo M - Case Rep Otolaryngol (2015)

Bottom Line: Proteus Syndrome (PS) is a rare hamartoneoplastic disorder associated with disproportionate and asymmetric overgrowth of body parts and hypertrophy or malformation of lymphatic tissues, such as palatine tonsils.To avoid the risk of a general anesthesia and remove only the obstructive portion of the palatine tonsil bipolar radiofrequency-induced thermotherapy (RFITT) under local anesthesia was performed.Recovery of the obstructive respiratory disease was obtained.

View Article: PubMed Central - PubMed

Affiliation: Department of Neuroscience, Reproductive and Odontostomatologic Science, Section of ENT, "Federico II" University, 80131 Naples, Italy.

ABSTRACT
Obstructive sleep apnea syndrome (OSAS) is a common disorder in childhood with high prevalence in syndromic subjects with craniofacial malformations. Proteus Syndrome (PS) is a rare hamartoneoplastic disorder associated with disproportionate and asymmetric overgrowth of body parts and hypertrophy or malformation of lymphatic tissues, such as palatine tonsils. We report a case of a 12-year-old boy diagnosed with Proteus Syndrome (PS) and suffering from OSAS due to asymmetric palatine tonsillar hypertrophy, treated with partial resection of left tonsil. To avoid the risk of a general anesthesia and remove only the obstructive portion of the palatine tonsil bipolar radiofrequency-induced thermotherapy (RFITT) under local anesthesia was performed. Recovery of the obstructive respiratory disease was obtained. To our knowledge, this is the first case reported in the literature of partial tonsillar resection performed in a patient with PS suffering from OSAS under local anesthesia.

No MeSH data available.


Related in: MedlinePlus

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fig2: MRI.

Mentions: A 12-year-old boy diagnosed with PS presented to our ENT Unit with a history of snoring and impaired swallowing. He had all of the general mandatory criteria for PS, and one feature of subgroup B (linear epidermal nevus) and three features of subgroup C (lipomas, vascular malformations, and facial phenotype) of specific criteria. In particular, he had macrocephaly, dolichocephaly, frontal bossing, and high stature for age. Some years before the child underwent surgical excision of multiple nape lipomas, whereas some angiomas in different areas were not removed (Figure 1), the ENT evaluation revealed a grade II adenoidal hypertrophy [6] and asymmetric enlargement of the palatine tonsils with a prevalence of the left one, corresponding to a Friedman grade IV with a severe narrowing of the upper airway. Laboratory tests revealed normal renal and liver functions, as well as red and white cells count. To exclude the presence of malignancy of the left tonsil, a magnetic resonance (MR) contrast image of head and neck was performed. It confirmed the presence of benign tonsillar enlargement (Figure 2) as well as vascular brain malformations, Arnold-Chiari type I, and fluid collection in prevertebral region. For this reason, the patient underwent chest computed tomography (CT) scan that showed a chylous collection in paramediastinal area, close to the lung pleura and encompassing the left subclavian and common artery.


Operative Management of OSAS in a Complex Case of Proteus Syndrome.

Cantone E, Cavaliere M, Castagna G, Marino A, Del Vecchio L, Iengo M - Case Rep Otolaryngol (2015)

MRI.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4493299&req=5

fig2: MRI.
Mentions: A 12-year-old boy diagnosed with PS presented to our ENT Unit with a history of snoring and impaired swallowing. He had all of the general mandatory criteria for PS, and one feature of subgroup B (linear epidermal nevus) and three features of subgroup C (lipomas, vascular malformations, and facial phenotype) of specific criteria. In particular, he had macrocephaly, dolichocephaly, frontal bossing, and high stature for age. Some years before the child underwent surgical excision of multiple nape lipomas, whereas some angiomas in different areas were not removed (Figure 1), the ENT evaluation revealed a grade II adenoidal hypertrophy [6] and asymmetric enlargement of the palatine tonsils with a prevalence of the left one, corresponding to a Friedman grade IV with a severe narrowing of the upper airway. Laboratory tests revealed normal renal and liver functions, as well as red and white cells count. To exclude the presence of malignancy of the left tonsil, a magnetic resonance (MR) contrast image of head and neck was performed. It confirmed the presence of benign tonsillar enlargement (Figure 2) as well as vascular brain malformations, Arnold-Chiari type I, and fluid collection in prevertebral region. For this reason, the patient underwent chest computed tomography (CT) scan that showed a chylous collection in paramediastinal area, close to the lung pleura and encompassing the left subclavian and common artery.

Bottom Line: Proteus Syndrome (PS) is a rare hamartoneoplastic disorder associated with disproportionate and asymmetric overgrowth of body parts and hypertrophy or malformation of lymphatic tissues, such as palatine tonsils.To avoid the risk of a general anesthesia and remove only the obstructive portion of the palatine tonsil bipolar radiofrequency-induced thermotherapy (RFITT) under local anesthesia was performed.Recovery of the obstructive respiratory disease was obtained.

View Article: PubMed Central - PubMed

Affiliation: Department of Neuroscience, Reproductive and Odontostomatologic Science, Section of ENT, "Federico II" University, 80131 Naples, Italy.

ABSTRACT
Obstructive sleep apnea syndrome (OSAS) is a common disorder in childhood with high prevalence in syndromic subjects with craniofacial malformations. Proteus Syndrome (PS) is a rare hamartoneoplastic disorder associated with disproportionate and asymmetric overgrowth of body parts and hypertrophy or malformation of lymphatic tissues, such as palatine tonsils. We report a case of a 12-year-old boy diagnosed with Proteus Syndrome (PS) and suffering from OSAS due to asymmetric palatine tonsillar hypertrophy, treated with partial resection of left tonsil. To avoid the risk of a general anesthesia and remove only the obstructive portion of the palatine tonsil bipolar radiofrequency-induced thermotherapy (RFITT) under local anesthesia was performed. Recovery of the obstructive respiratory disease was obtained. To our knowledge, this is the first case reported in the literature of partial tonsillar resection performed in a patient with PS suffering from OSAS under local anesthesia.

No MeSH data available.


Related in: MedlinePlus