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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

ENT evaluation after RFITT.
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fig4: ENT evaluation after RFITT.

Mentions: Histopathology confirmed the benign follicular hyperplasia (Figure 3). In addition, we performed a peripheral blood and tissue cytofluorometric exam that did not show quantitative anomalies in T, B, and NK lymphocytes. Postoperative course was regular without any complication and the patient was discharged on the same day. The postoperative follow up (Figure 4) showed improvement in symptoms, in questionnaire, and, what is most important, in the polysomnography performed 3 months after surgery that revealed an AHI of 2,4.


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)

ENT evaluation after RFITT.
© Copyright Policy
Related In: Results  -  Collection

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

fig4: ENT evaluation after RFITT.
Mentions: Histopathology confirmed the benign follicular hyperplasia (Figure 3). In addition, we performed a peripheral blood and tissue cytofluorometric exam that did not show quantitative anomalies in T, B, and NK lymphocytes. Postoperative course was regular without any complication and the patient was discharged on the same day. The postoperative follow up (Figure 4) showed improvement in symptoms, in questionnaire, and, what is most important, in the polysomnography performed 3 months after surgery that revealed an AHI of 2,4.

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