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G446(P) An Unusual cause of Chronic Cough in a 15 year old girl
  1. T Moore,
  2. N Makwana,
  3. R Jayatunga
  1. General Paediatrics, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, UK

Abstract

Aims Coughing is one of the most common paediatric presentations both in primary and secondary care. We present an unusual cause of chronic cough in a teenager.

Method A 15 year old girl presented to general paediatric clinic with a one year history of chronic coughing, chest tightness and difficulty in swallowing with excessive production of saliva. She complained of episodic wheeze and occasional nocturnal breathlessness. She had weight loss of one stone in four months. Tonsillectomy earlier in the year and salbutamol and beclomethasone inhalers had not improved her symptoms. There was no recent foreign travel and no significant family history.

Physical examination was normal, as were her growth parameters. Provisional differential diagnoses were gastro-oesophageal reflux, tuberculosis or psychological cause. Chest x-ray showed mediastinal widening. Routine bloods were all normal. Sputum for Acid Fast Bacilli and Mantoux test were also negative. A barium swallow revealed narrowing of the lower oesophagus with “rat tail” appearance, proximal distension and fluid level characteristic of Achalasia.

Results Achalasia is rare in the paediatric population (0.18 per 100,000 children)1. It is characterised by the absence of oesophageal peristalsis with increased resting pressure and failure of relaxation of the lower oesophageal sphincter2. The aetiology is unknown although it is commoner in males3. Achalasia is associated with other syndromes such as Rozycki syndrome (achalasia with deafness, short stature, vitiligo, muscle wasting) and Allgrove’s syndrome (achalasia with glucocorticoid insufficiency and defective tear formation). The most common presenting symptom is dysphagia. Retrosternal pain, vomiting, regurgitation, retarded growth, and respiratory symptoms have been reported4. Cough occurs due to aspiration of food or airway compression by the dilated oesophagus5. Barium studies and oesophageal manometry are effective diagnostic investigations4. Surgery is the mainstay of treatment, with the most common operation being Heller’s Myotomy6 which has good results.

Conclusion Achalasia is an important but rare diagnosis to consider in chronic cough. Teenagers can present with a variety of symptoms which may have no physical cause. Barium swallow is diagnostic of achalasia and should be performed when other investigations are normal before dismissing symptoms as psychological.

References

  1. Marlais M, Fishman JR, Fell JM, et al. UK incidence of achalasia: an 11-year national epidemiological study. Arch Dis Child. 2011 Feb;96(2):192–4.

  2. Morris-Stiff et al. Long-term results of surgery for childhood achalasia. Ann R Coll Surg Engl. 1997 November; 79(6): 432–434.

  3. Pedro M. Fernandez; Esophageal achalasia of unknown etiology in children. J Pediatr (Rio J). 2004;80(6):523-6.

  4. Zhang Y; Diagnosis and management of esophageal achalasia in children: analysis of 13 cases. World J Pediatr. 2009 Feb;5(1):56-9.

  5. Nighat F Mehdi; Achalasia: unusual cause of chronic cough in children. Cough 2008, 4:6.

  6. Lee CW; Outcomes of treatment of childhood achalasia. J Pediatr Surg. 2010 Jun;45(6):1173-7.

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