The unexpected surgical emergency in a child with cystic fibrosis: An acute appendicitis with unusual presentation
Abstract
Cystic fibrosis is an autosomal recessive disease characterized by structural and metabolic genetic dysfunction of the exocrine glands. Abdominal pain is the most common symptom in patients with cystic fibrosis within gastrointestinal symptoms. It is more commonly related to distal ileal obstruction syndrome. In patients with cystic fibrosis, the appendix is frequently distended due to an intense eosinophilic secretion but ironically, the incidence of acute appendicitis has been reported under the general population. Acute appendicitis in patients with cystic fibrosis commonly present with atypical symptoms. We present the case of cystic fibrosis with an acute appendicitis which had atypical radiologic and laboratory findings that were not compatible with the diagnosis. The diagnosis was made on the basis of clinical examination. As a result, it is of great importance to know that acute appendicitis in patients with cystic fibrosis can present with unusual clinical and radiological findings in order to avoid delayed diagnosis and related serious complications.
References
- 1. Timuragaoglu L, Sen V, Gurkan MF. Evaluation of the Clinical and Laboratory Characteristics of Children with Cystic Fibrosis. Archives of Pediatrics. 2016;1(1):21-6.
- 2. Lavelle LP, McEvoy SH, Mhurchu EN, Gibney RG, McMahon CJ, Heffernan EJ, et al. Cystic Fibrosis below the Diaphragm: Abdominal Findings in Adult Patients. RadioGraphics. 2015;35:680-95.
- 3. Shields MD, Levison H, Reisman JJ, Durie PR, Canny GJ. Appendicitis in cystic fibrosis. Archives of Disease in Childhood. 1990;65:307-10.
- 4. Davis PB. Therapy for cystic fibrosis: the end of the beginning? N Engl J Med. 2011;365(18):1734-35.
- 5. Menten R, Lebecque P, Saint-Martin C, Clapuyt P. Outer diameter of the vermiform appendix: not a valid sonographic criterion for acute appendicitis in patients with cystic fibrosis. AJR Am J Roentgenol. 2005;184(6):1901-03.
- 6. McCarthy VP, Mischler EH, Hubbard VS, Chernick MS, Di Sant'Agnese PA. Appendiceal abscess in cystic fibrosis: a diagnostic challenge. Gastroenterology. 1984;86:564-8.
- 7. Kilinc MO, Ninis VN, Dağli E, Demirkol M, Ozkinay F, Arikan Z, et al. Highest heterogeneity for cystic fibrosis: 36 mutations account for 75% of all CF chromosomes in Turkish patients. Am J Med Genet. 2002;113(3):250-7
- 8. Coughlin JP, Gauderer MW, Stern RC, Doershuk CF, Izant RJ Jr, Zollinger RM Jr. The spectrum of appendiceal disease in cystic fibrosis. J Pediatr Surg. 1990;25(8):835–9.
- 9. Lardenoye SW, Puylaert JB, Smit MJ, Holscher HC. Appendix in children with cystic fibrosis: US features. Radiology. 2004;232(1):187-89.
Abstract
Kistik fibrozis ekzokrin glandların yapısal ve metabolik genetik disfonksiyonu ile karakterize otozomal resesif bir hastalıktır. Kistik fibrozisli hastalarda gastrointestinal semptomlar içerisinde ağrı en yaygın semptomdur ve genellikle distal ileal obstrüksiyon sendromuna bağlıdır. Kistik fibrozisli hastalarda apendiks yoğun bir eozinofilik sekresyondan dolayı sıklıkla distandüdür ancak ironik olarak akut apandisit insidansı genel popülasyondan daha düşüktür. Akut apandisit, kistik fibrozisli hastalarda sıklıkla atipik semptomlarla kendini gösterir. Bu yazıda atipik radyolojik bulguları olan kistik fibrozisli bir akut apandisit olgusu sunuldu ve laboratuvar bulguları, tanı ile uyumsuzdu. Hastada sadece defans ve rebound gibi klasik muayene bulguları mevcuttu ve bu temelde hasta tanı aldı. Sonuç olarak, geç tanıya bağlı ciddi komplikasyonları önlemek için, akut apandisitli kistik fibrozis hastalarının atipik klinik ve radyolojik bulgularla başvurabileceği akılda tutulmalıdır.
Keywords
References
- 1. Timuragaoglu L, Sen V, Gurkan MF. Evaluation of the Clinical and Laboratory Characteristics of Children with Cystic Fibrosis. Archives of Pediatrics. 2016;1(1):21-6.
- 2. Lavelle LP, McEvoy SH, Mhurchu EN, Gibney RG, McMahon CJ, Heffernan EJ, et al. Cystic Fibrosis below the Diaphragm: Abdominal Findings in Adult Patients. RadioGraphics. 2015;35:680-95.
- 3. Shields MD, Levison H, Reisman JJ, Durie PR, Canny GJ. Appendicitis in cystic fibrosis. Archives of Disease in Childhood. 1990;65:307-10.
- 4. Davis PB. Therapy for cystic fibrosis: the end of the beginning? N Engl J Med. 2011;365(18):1734-35.
- 5. Menten R, Lebecque P, Saint-Martin C, Clapuyt P. Outer diameter of the vermiform appendix: not a valid sonographic criterion for acute appendicitis in patients with cystic fibrosis. AJR Am J Roentgenol. 2005;184(6):1901-03.
- 6. McCarthy VP, Mischler EH, Hubbard VS, Chernick MS, Di Sant'Agnese PA. Appendiceal abscess in cystic fibrosis: a diagnostic challenge. Gastroenterology. 1984;86:564-8.
- 7. Kilinc MO, Ninis VN, Dağli E, Demirkol M, Ozkinay F, Arikan Z, et al. Highest heterogeneity for cystic fibrosis: 36 mutations account for 75% of all CF chromosomes in Turkish patients. Am J Med Genet. 2002;113(3):250-7
- 8. Coughlin JP, Gauderer MW, Stern RC, Doershuk CF, Izant RJ Jr, Zollinger RM Jr. The spectrum of appendiceal disease in cystic fibrosis. J Pediatr Surg. 1990;25(8):835–9.
- 9. Lardenoye SW, Puylaert JB, Smit MJ, Holscher HC. Appendix in children with cystic fibrosis: US features. Radiology. 2004;232(1):187-89.
Details
| Primary Language | English |
|---|---|
| Subjects | Clinical Sciences |
| Journal Section | Case report |
| Authors | |
| Publication Date | January 27, 2019 |
| Published in Issue | Year 2019 Volume: 3 Issue: 1 |
