Postoperative prolonged apnea in weill marchesani syndrome

Weill Marchesani Sendromu (WMS), nadir, görülen bir sistemik konnektif doku hastalığıdır. Sendrom karakteristik olarak mikroseferofaki, ektopik lens, glokom, brakiodaktili ve brakiomorfı içermektedir. Bu olgu sunumunda, glokom cerrahisi geçiren, belirgin rezidüel blok ve postoperatif apne gelişen 17 ve 18 yaşlarında iki kız kardeş bildirilmektedir. Her iki hastamnda bir sonraki anestezi uygulamaları farklı anestezîk ajanlar kullanılarak sorunsuz tamamlanmıştır. Bu yazıda, uygulanan ilaçlar arasındaki muhtemel reaksiyonlar, konjenital sendrom ve uzamış apne nedenleri araştırılarak, tartışılmıştır.

Weill marchesani sendromunda uzamış postoperatif apne

Weill Marchesani Syndrome (WMS) is an unusual systemic connective tissue disorder. This case report describes two sisters, 17 and 18 years old, with WMS. Both patients underwent glaucoma surgery and had prolonged postoperative recovery times because of apnea and an apparent residual neuromuscular block. Additional anesthetic exposures with different anesthetic drugs in both patients were uneventful. In this report, the possible interactions among the applied drugs, the hereditary syndrome and prolonged apnea were discussed.

PDF

___

1. Weill G. Ectopie des cristallins et malformations generales. Ann Ocul (French, Paris) 1932; 169: 21-24.
2. Wirtz MK, Samples JR, Kramer PZ, Rust K. Weill- Marchesani syndrome possible linkage of the autosomal dominant form to 15q 21. Am J Med Genet 1996; 65: 68- 75.
3. Evereklioglu C, Hepsen IF, Er H. Weill-Marchesani syndrome in three generations. Eye 1999;13: 773-77.
4. Denlinger JK, Gravenstein N, Kirby RR. Complications in Anesthesiology. Philadelphia: Lippincott-Raven, 1996; 441-480.
5. Pantuck EJ. Plasma cholinesterase: gene and variations. Anesth Analg 1993; 77: 380-386.
6. Brown SC. Moyamoya disease : a review of clinical experience and anesthetic management. Can J Anesth 1990; 37: 210-218.
7. Argov Z, Mastaglia FL. Disorders of neuromuscular transmission caused by drugs. N Engl J Med 1979; 30: 409-413.
8. Berthoud MC, Reilly CS. Adverse effects of general anaesthetics. Drug Saf 1992; 7: 434-459.
9. Scott IU, Greenfield DS, Schiffman J. Outcomes of primary trabeculotomy with the use of adjunctive Mitomycin. Arch Ophthalmol 1998; 116: 286-291.
10. Riedl CR, Knoll M, Plas E, Pfluger H. Intravesical electromotive drug administration technique: preliminary results and side effects. J Urol 1998; 159: 1851-56.
11. Viby-Mogensen J. Succinylcholine neuromuscular blockade in subjects homozygous for atypical plasma cholinesterase. Anesthesiology 1988; 55: 429-434.
12. Ostergaard D, Engbaek J, Viby-Mogensen J. Adverse reactions and interactions of the neuromuscular blocking drugs. Med Toxicol Adverse Drug Exp 1989; 4: 351-368.