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.

Keywords

• Cystic fibrosis,Appendicitis,Pediatric age group

Kistik fibrozlu bir çocukta beklenmedik cerrahi acil: Atipik sunumlu akut apandisit

Öz

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.

Anahtar Kelimeler

• Kistik fibrozis,Apandisit,Pediatrik yaş grubu

Kaynakça

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