Incidence and Outcome of Patterson-Kelly Syndrome in Thi-Qar Governorate / Iraq (2013-2022)

Background Patterson-Kelly Syndrome is an uncommon collection of signs and symptoms in the form of difficulty of swallowing, sideropenic anemia leading sometimes to koilonychias (scooped out nails) and post-cricoid web. This Syndrome which is also called Plummer-Vinson syndrome (PVS) is more commonly seen in females of middle age group and is associated with an increased possibility of malignancy in the form of Squamous Cell Carcinoma of the regions of GIT (terminal part of pharynx and beginning of esophagus). The etiology of Plummer Vinson syndrome remains unknown. Barium swallow radiography is the investigation of choice and the simplest and most accurate option to diagnose this uncommon syndrome. Aim of study: The purpose of this study is to show the importance of an uncommon syndrome called Plummer-Vinson Syndrome and it's classic triad of difficulty of swallowing, sideropenic anemia leading sometimes to koilonychias (scooped out nails) and post-cricoid web and that simple esophageal dilatation of the affected patient can change his/her fate and compare our study with other studies in the world. Patients & Methods: Our study is a retrospective study of fifty patients for whom management was applied over a period of about nine years (2013 - 2022). Most of our patients were exposed to simple esophageal dilatation under general anesthesia with excellent results. A minority of patients (10%) had been managed without dilatation by only correction of iron deficiency anemia. On admission into the hospital, history was taken from patients including age, onset and nature of signs and symptoms which are related to the syndrome itself. Other information included possible chronic illnesses, presence of previous surgical procedures and also history of drug allergy. Results: Females (80%) were more commonly affected than males by Plummer-Vinson Syndrome. The ages affected ranged from 30 years to more than 50 years but most of the patients' age ranged from 40 to 50 years. Regarding the signs and symptoms of patients at time of presentation , 100% of the patients had dysphagia associated always with iron deficiency anemia and significant number of our patients ( 80% ) had obvious weight loss due to inability of having normal oral intake. No malignancy was found to be associated with Plummer-Vinson Syndrome at time of presentation but chronic illnesses like rheumatoid arthritis (10%), thyroid disease (20%) & rarely Crohn's disease (2%) were found as an associated disease. Malnutrition and vitamin B deficiency was a common associated condition and found in most patients. Routine blood investigations and barium swallow were done for all cases but Computerized Tomography was carried out for a certain percentage (20%) of patients. All patients received management with good long term results and dilatation was the procedure of choice for 90% of patients while 10% of patients could be treated without need for dilatation by giving iron and vitamins supplements to the patient. Perioperative complications included bleeding, delayed recovery from anesthesia, sinus tachycardia & atrial fibrillation. No cases of perforation were recorded in our study and mortality rate was nil. Conclusion & Recommendations: 1. Dysphagia associated with iron deficiency anemia may be part of a syndrome called Plummer-Vinson Syndrome (Patterson-Kelly Syndrome). 2. Simple barium swallow is diagnostic for Plummer-Vinson Syndrome. 3. Most patients responded to simple mechanical dilatation which is usually carried out under general anesthesia with minimal complication rate. 4. No significant morbidity rate was observed. 5. No mortality rate was observed. 6. Excellent results were observed after dilatation of stenosed segment of esophagus in Plummer-Vinson Syndrome. 7. Careful follow up after dilatation of stenosed segment of esophagus in Plummer-Vinson Syndrome is mandatory to detect and treat complications as early as possible and to exclude malignancy. 8. Bleeding after mechanical dilatation in Plummer-Vinson Syndrome patients was controllable and was expected to occur due to tear of the web during procedure of mechanical dilatation. 9. Searching for associated conditions and disorders with Plummer-Vinson Syndrome like rheumatoid arthritis, thyroid disease and rarely Crohn's disease is indicated. 10. Major intervention including resection of segment of esophagus containing the web followed by end to end anastomosis or bowel interposition in cases of Plummer-Vinson Syndrome is contraindicated because of the excellent results obtained by simple mechanical dilatation. 11.Iron and vitamins supplements should always be offered to Plummer-Vinson Syndrome patients. 12. Follow up ofPlummer-Vinson Syndrome patients is important because it is a premalignant condition.