SavedAdd your journal

Journals / ENTcase / 2020 / Cilt: 6 - Sayı: 4

AN UNUSUAL CASE REPORT: UNEVENTFUL ADENOTONSILLECTOMY IN A WILLIAMS SYNDROME PATIENT WITH ADENOTONSILLAR HYPERTROPHY

WILLIAMS SENDROMLU ADENOTONSİLLER HİPERTROFİLİ HASTADA SORUNSUZ ADENOTONSİLLEKTOMİ

Başak BALLICA
Fuat BULUT
Ayşe Sezim ŞİMŞEK
Journal
ENTcase
Pages
126–130
Publication Date
2022-10-12
DOI
—
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Williams-Beuren Syndrome (WS;OMIM194050), also called Williams Syndrome ischaracterized by dysmorphic facial features,neurological findings and endocrinologicalabnormalities resulting from various lengths ofdeletions in the q11.23 region of the 7th chromosome.We present unproblematic adenotonsillectomy undergeneral anesthesia in a child with WS andadenotonsillar hypertrophy.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Adenotonsillar disease is one of the most commondiseases of childhood.
📊
Key Findings & Data:Children suffer from upperairway obstruction due to adenotonsillar hypertrophyas well as attacks of infection.
💡
Conclusion & Impact:Williams-Beuren Syndrome (WS;OMIM194050), also called Williams Syndrome ischaracterized by dysmorphic facial features,neurological findings and endocrinologicalabnormalities resulting from various lengths ofdeletions in the q11.23 region of the 7th chromosome.We present unproblematic adenotonsillectomy undergeneral anesthesia in a child with WS andadenotonsillar hypertrophy.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Adenotonsillar disease is one of the most commondiseases of childhood. Children suffer from upperairway obstruction due to adenotonsillar hypertrophyas well as attacks of infection. Its clinical presentationranges from mild recurrent infection to congestiveheart failure. Williams-Beuren Syndrome (WS;OMIM194050), also called Williams Syndrome ischaracterized by dysmorphic facial features,neurological findings and endocrinologicalabnormalities resulting from various lengths ofdeletions in the q11.23 region of the 7th chromosome.We present unproblematic adenotonsillectomy undergeneral anesthesia in a child with WS andadenotonsillar hypertrophy.

Özet

Adenotonsiller hastalık, çocukluk çağının en sık görülen hastalıklarından biridir. Çocuklar, adenotonsiller hipertrofiye bağlı üst solunum yolu tıkanıklığı ve enfeksiyon ataklarından müzdariptir. Klinik görünümü hafif tekrarlayan enfeksiyondan konjestif kalp yetmezliğine kadar değişir. Williams-Beuren Sendromu (WS;OMIM 194050) aynı zamanada Williams Syndrome olarak adlandırılan 7. kromozomun q11.23 bölgesindeki çeşitli uzunluktaki delesyonlardan kaynaklanan dismorfik yüz özellikleri, nörolojik bulgular ve endokrinolojik anormalliklerle karakterize bir sendromdur. Adenotonsiller hipertrofisi olan WS'li bir çocukta genel anestezi altında sorunsuz adenotonsillektomi sunuyoruz.

🔖

Cite This Article

BALLICA, B., BULUT, F., ŞİMŞEK, A. S. (2022). AN UNUSUAL CASE REPORT: UNEVENTFUL ADENOTONSILLECTOMY IN A WILLIAMS SYNDROME PATIENT WITH ADENOTONSILLAR HYPERTROPHY. ENTcase, Cilt: 6 - Sayı: 4, 126–130. https://ajindex.com/articles/501297

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (9)

  1. P. E. Horowitz, S. Akhtar, J. A. Wulff, F. Al Fadley, and Z. Al Halees, “Coronary artery disease and anesthesia-related death in children with Williams syndrome.,” Journal of cardiothoracic and vascular anesthesia, vol. 16, no. 6, pp. 739–741, Dec. 2002
  2. K. Broder, E. Reinhardt, J. Ahern, R. Lifton, W. Tamborlane, and B. Pober, “Elevated ambulatory blood pressure in 20 subjects with Williams syndrome.,” American journal of medical genetics, vol. 83, no. 5, pp. 356–360, Apr. 1999
  3. O. H. Wilder-Smith, P. A. Ravussin, L. A. Decosterd, P. A. Despland, and B. Bissonnette, “Midazolam premedication reduces propofol dose requirements for multiple anesthetic endpoints.,” Canadian journal of anaesthesia = Journal canadien d’anesthesie, vol. 48, no. 5, pp. 439–445, May 2001
  4. T. M. Burch, F. X. J. McGowan, B. D. Kussman, A. J. Powell, and J. A. DiNardo, “Congenital supravalvular aortic stenosis and sudden death associated with anesthesia: what’s the mystery?,” Anesthesia and analgesia, vol. 107, no. 6, pp. 1848–1854, Dec. 2008
  5. G. J. Latham, F. J. Ross, M. J. Eisses, M. J. Richards, J. M. Geiduschek, and D. C. Joffe, “Perioperative morbidity in children with elastin arteriopathy.,” Paediatric anaesthesia, vol. 26, no. 9, pp. 926–935, Sep. 2016
  6. M. D. Twite, S. Stenquist, and R. J. Ing, “Williams syndrome.,” Paediatric anaesthesia, vol. 29, no. 5, pp. 483–490, May 2019
  7. R. T. 2nd Collins, P. F. Aziz, M. M. Gleason, P. B. Kaplan, and M. J. Shah, “Abnormalities of cardiac repolarization in Williams syndrome.,” The American journal of cardiology, vol. 106, no. 7, pp. 1029–1033, Oct. 2010
  8. G. E. Staudt and S. S. Eagle, “Anesthetic Considerations for Patients With Williams Syndrome.,” Journal of cardiothoracic and vascular anesthesia, Jan. 2020
  9. J. Medley, P. Russo, and J. D. Tobias, “Perioperative care of the patient with Williams syndrome.,” Paediatric anaesthesia, vol. 15, no. 3, pp. 243–247, Mar. 2005.