Disfaji Olan Çocuk Hastalarda Plummer-Vinson Sendromunun Değerlendirilmesi

Year 2024, Volume: 13 Issue: 2, 47 - 53

Sevinç Garip

https://doi.org/10.47493/abantmedj.1355176

Abstract

Amaç: Disfaji çocukluk çağında sık görülmeye başlanmakta ve birçok nedene bağı olarak gelişmektedir. Çalışmamızda pediatrik çağda disfajinin diğer tüm nedenleri dışlandıktan sonra demir eksikliği anemisi disfaji olan Plummer-Vinson sendromu tanısı ile takip ve tedavi edilen hastalar sunulmaya çalışılmıştır. Amacımız sadece anemi tedavisi ile düzelebilecek disfaji olabileceğini ve çocuk sağlığı ve hastalıkları uzmanı tarafından tedavi ile düzelebileceğini vurgulamaktır.
Materyal ve Metod: Hastanemizde Mayıs 2019 ve Kasım 2020 arasında disfaji şikayeti ile başvuran, disfajinin diğer tüm nedenleri dışlandıktan sonra Plummer-Vinson sendromu tanısı alan hastalar retrospektif olarak değerlendirildi. Hastaların yaş, cinsiyet, disfaji şekli, özelliği ve süresi retrospektif olarak değerlendirilerek literatür eşliğinde sunmak amaçlanmıştır.
Bulgular: Disfaji şikayetiyle başvuran 200 çocuk hastanın 24’ünde PWS saptandı. Hastaların %37,5’u erkek olup, ortalama yaşları 97,5 aydı. Hastaların disfaji şikayet süresi ortalama 11,2 ay olup, 4 hastanın sadece sıvı gıda yutmada zorlanması varken 12 hastada katı, 8 hastada hem katı hem sıvıya karşı yutma güçlüğü mevcuttu. Tam kan sayımı yapılarak ve hemoglobin, hematokrit düzeyleri yaşa uygun 2 standart sapma değerinin altında olanlar anemi; vitamin B12 düzeyi 200 pg/ml değerinin altında olanlar kobalamin eksikliği olarak tanımlandı. Kontraslı özofagografi çekilen 4 hastada özofageal web saptandı. Web saptanan hastaların özofagogastroduodensokopide darlık endoskopik olarak tedavi edildi. Yirmi hastada ise altı aylık demir eksikliği anemi tedavisi sonrası yutma güçlüğünün devam etmediği görüldü.
Sonuç: PVS tanısı çocuklarda nadir görülse de, demir eksikliği anemisi ile ilişkili yutma güçlüğü çeken tüm çocuklarda akla gelmeli ve tedavisi verilmelidir. Uygun dozda ve sürede tedavi sonrası şikayeti devam eden hastalar çocuk gastroenteroloji değerlendirmesi için yönlendirilmelidir.

Keywords

disfaji, çocuk, Plummer-Vinson sendromu, özofageal web, demir eksikliği anemisi

Evaluation Of Plummer-Vinson Syndrome in Pediatric Patients with Dysphagia

Abstract

Aim: In our study, after excluding all other causes of dysphagia in the pediatric age, we tried to present the patients who were followed up and treated with the diagnosis of Plummer-Vinson syndrome(PWS) with iron deficiency anemia and dysphagia. Our aim is to emphasize that there may be dysphagia that can only be cured with anemia treatment and that it can be improved with treatment by a pediatrician.
Material method: Pediatric patients who applied to our hospital with the complaint of dysphagia between May 2019-November 2020 were included in the study. After excluding all other causes of dysphagia, patients diagnosed with PWS were evaluated retrospectively. It was aimed to evaluate the age, gender, type, characteristics and duration of dysphagia of the patients retrospectively and to present them in the light of the literature.
Results: PWS was detected in 24 of 200 pediatric patients who presented with dysphagia. The proportion of male patients was 37.5%, and their mean age was 97 months. The mean duration of dysphagia complaints was 11.2 months. Four patients had difficulty swallowing liquid food only, 12 patients had solid swallowing difficulties, and 8 patients had difficulty swallowing both solid and liquid food. Anemia was diagnosed by performing a complete blood count and having hemoglobin and hematocrit levels below 2 standard deviations appropriate for age; Those with vitamin B12 levels below 200 pg/ml were defined as cobalamin deficiency. Esophageal web was detected in 4 patients who underwent contrast esophagography. In esophagogastroduodensoscopy, stenosis was treated endoscopically in patients with web. In 20 patients, dysphagia did not persist after six months of iron deficiency anemia treatment.
Conclusion: Although the diagnosis of PVS is rare in children, it should be considered and treated in all children with dysphagia associated with iron deficiency anemia. Patients whose complaints continue after treatment at the appropriate dose and duration should be referred for pediatric gastroenterology evaluation.

Keywords

child, Plummer-Vinson syndrome, iron-deficiency anemia, esophageal web, dysafgia

Ethical Statement

Ethics Committee Approval: The study was approved by Adana city training and research hospital clinical research ethics committee (approval date: 17.08.2023, meeting number: 133, decision no: 2772).

Supporting Institution

Yok

Thanks

Acknowledgement: We would like to thank the Pediatric Health and Diseases Clinic staff who contributed to the diagnosis and treatment processes of the patients who participated in the relevant study.

References

• Crawfurd MD, Jacobs A, Murphy B, Peters DK. Paterson-Kelly syndrome in adolescence: a report of five cases. Br Med J. 1965;1: 693-695.
• Seitz ML, Sabatino D. Plummer-Vinson syndrome in an adolescent. J Adolesc Health 1991;12: 279-281.
• Wynder EL, Fryer JH. Etiologic considerations of Plummer-Vinson (Patterson-Kelly) syndrome. Ann Intern Med. 1958;49:1106–28.
• Kelly AB. Spasm at the entrance of the esophagus. J Laryngol Rhinol Otol. 1919;34:285-89.
• Plummer S. Diffuse dilatation of the esophagus without anatomic stenosis (cardiospasm).A report of ninety one cases. J Am Med Assoc. 1912;58: 2013-2015.
• Paterson DR. Clinical type of dysphagia. J Laryngol Rhinol Otol 1919; 24: 289-291.
• Godino J, Wong PW. A triad of troubling findings. Plummer-Vinson syndrome. Postgrad Med 2000;108:109-10.
• Hoffman RM, Jaffe PE. Plummer-Vinson syndrome. A case report and literature review. Arch Intern Med 1995;155:2008–11.
• Novacek G. Plummer-Vinson syndrome. Orphanet J Rare Dis 2006; 1:36.
• Daghfous MH, Slim R, Mouelhi M, L Hendeouı. Esophageal transit in the Plummer-Vinson syndrome. Ann Radiol (Paris). 1985;28:533–9.
• Dantas RO, Villanova MG. Esophageal motility impairment Plummer-Vinson syndrome. Correction by iron treatment. Dig Dis Sci. 1993;38:968-71.
• Anderson SR, Sinacori JT. Plummer-Vinson syndrome heralded by postcricoid carcinoma. Am J Otolaryngol. 2007; 28: 22-24.
• Dantas RO. Iron deficiency and dysphagia. Am J Gastroentero1. 1999; 94: 3072-3073.
• Miranda AL, Dantas RO. Esophageal contractions and oropharyngeal and esophageal transits in patients with iron deficiency anemia. Am J Gastroenterol. 2003; 98: 1000-1004.
• Makharia GK, Nandi B, Garg PK, Tandon RK. Plummer-Vinson syndrome: unusual features. Indian J Gastroenterol. 2002;21:74-5.
• Malhotra P, Kochhar R, Varma N, S Kumari, S Jain, S Varma. Paterson-Kelly syndrome and celiac disease-a rare combination. Indian J Gastroenteroly. 2000;19:191-2.
• Dickey W, McConnell B. Celiac disease presenting as the PatersonBrown Kelly (Plummer-Vinson) syndrome. Am J Gastroenterol 1999;94:527-9.
• Butori M, Mahmoudi S, Dugelay Ecochard E, et al. J Pediatr Gastroenterol Nutr 2015;61(5):547-52.
• Hoffman RM, Jaffe PE. Plummer-Vinson syndrome. A case report and literature review. Arch Intern Med. 1995;155:2008–11.
• Enomoto M, Kohmoto M, Arafa UA et al. Plummer-Vinson syndrome successfully treated by endoscopic dilatation. J Gastroenterol Hepatol. 2007;22:2348–51.
• Nosher JL, Campbell WL, Seaman WB. The clinical significance of cervical esophageal and hypopharyngeal webs. Radiology 1975;117(1):45-47.
• Chisholm M. The association between webs, iron and post-cricoid carcinoma. Postgrad Med J 1974;50(582):215-19.
• Beyler AR, Yurdaydin C, Bahar K, Gören A, Soykan I, Uzunalimoğlu O. Dilation therapy of upper esophageal webs in two cases of Plummer-Vinson syndrome. Endoscopy 1996;28:266-7.
• Demirci F, Savaş MC, Kepkep N et al. Plummer-Vinson syndrome and dilation therapy: a report of two cases. Turk J Gastroenterol 2005;16:224-7.
• Ribeiro U Jr, Posner MC, Safatle-Ribeiro AV, Reynolds JC. Risk factors for squamous cell carcinoma of the oesophagus. Br J Surg 1996;83:1174-85.
• Katsinelos P, Gkagkalis S, Chatzimavroudis G, Zavos C, Spyridakis J, Kountouras J. Recurrent esophageal web in Plummer-Vinson syndrome successfully treated with postdilation intralesional injection of mitomycin C. Gastrointest Endosc. 2012;75:1124.
• Goel A, Lakshmi CP, Bakshi SS, Soni N, Koshy S. Single-center prospective study of Plummer-Vinson syndrome. Dis Esophagus. 2016;29(7):837-41.
• Patil M, Malipatel R, Devarbhavi H. Plummer-Vinson syndrome: A decade’s experience of 132 cases from a single center. J Gastroenterol Hepatol. 2021;36(1):181-85.
• Lopez Rodriguez MJ, Robledo Andres P, Amarilla Jimenez A, Roncero Maillo M, Lopez Lafuente A, Arroyo Carrera I. Sideropenic dysphagia in an adolescent. J Pediatr Gastroenterol Nutr. 2002; 34: 87-90.