Case Presentation

Case Presentation: The boy with Chest Pain : 

Case Presentation: The boy with Chest Pain Ward 12, LRI, Balmoral Building

Background : 

PC: Chest pain HPC: 2 days history of sudden onset, right-sided chest pain since 15/8/10 Pain was present all the time No particular trigger, was just sitting down when pain first came Pain was described as sharp, with gurgling noise. Rated pain as 4/10. Background

Slide 3: 

Aggr. Factor: Movement Relieving factor: None Associated with mild SOB. No coughing episodes, sputum production, wheezes, fever, prior infection Never had symptoms before. Went for holidays for 2 weeks, when returned on 1/9, went to see GP and had a chest X-Ray

Slide 4: 

PMH: Inguinal hernia (Operation-10 months old) caused him feeding problem Bell’s Palsy  Age 1 year old & recur at 14 years old Birth history: Normal pregnancy and delivery DH None. Allergic to erythromycin. (diarrhoea)

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FH Nothing of significance Immunisation hx Up-to-date with vaccinations. Developmental hx After operation at 10 months old, never been able to catch up with feeding(weight always lower than centile). Height always been normal. Normal milestones.

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Social history Lives with Mom, Dad, and elder sister in a detached house. All family members tall stature. No one in family smokes. Doing well at school, except PE (least enjoyed subject) Systems Review No problem with bowel, bladder, abdomen and others.

Examination Findings on Admission : 

General inspection: Tall, very thin stature. Alert Inspection: Fingers: No significant findings. RR: 20bpm Pulse: 80bpm Not cyanosed, no signs of anaemia JVP not raised High arched palate. No scars on chest. Pectus carinatum present. Examination Findings on Admission

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Palpation Central trachea. No lymphadenopathy. Apex beat is not displaced. Reduced chest expansion on the right side. Percussion Hyperresonant on right side. Auscultation Reduced air entry on right side. No crepitations/wheezes.

Differential Diagnosis : 

Spontaneous Pneumothorax associated with ?Marfan Syndrome Musculoskeletal pain Pneumonia Pulmonary Embolism Asthma Differential Diagnosis

Investigation : 

Chest X-Ray Arterial Blood Gas Investigation Management ABC Oxygen 100% high-flow Chest drain Analgesia

Plan of investigation for Marfan Syndrome : 

Urine homocysteinuria Genetic screen for Marfan syndrome Echocardiographymonitor aortic root width and heart valve Ophthalmology review Plan of investigation for Marfan Syndrome

Pneumothorax : 

Pneumothorax defined as air in the pleural space. Classifications:- Traumatic Spontaneous pneumothorax

Aetiology : 

Aetiology Primary – healthy no lung disease Secondary – lung disease Asthma COPD CTD (eg: Marfan’s syndrome, Ehlers-Danlos syndrome), Trauma Smoking causes an increased risk of a spontaneous pneumothorax: Thin, tall man

Clinical features : 

Clinical features sudden onset of pleuritic chest pain accompanied by pallor, tachycardia and sweating reduced chest expansion on the affected side hyper-resonant percussion note - if large pneumothorax reduced or absent breath sounds at the affected site sticky clicking sound, heard in time with the heart beat - in a left sided pneumothorax

Investigations : 

Investigations Chest radiograph: Inspiratory & expiratory PA view Size of pneumothorax In these new guidelines the size of a pneumothorax is divided into “small” or “large” depending on the presence of a visible rim of <2 cm or >2 cm between the lung margin and the chest wall. Arterial blood gases

Management : 

Management ABC High flow O2 (10l) Simple aspiration & re-xray to check for resolution - is recommended as first line treatment for all primary pneumothoraces requiring intervention. Chest drain – large pneumothorax (>2cm) Analgesia for pleuritic pain

Complications chest drain : 

Complications chest drain Bleeding Infection in the pleural space (empyema) Damage to adjacent structures (e.g long thoracic nerve – wing scapula, lympathic damage, chylothorax) Abdominal and thoracic injury lung, stomach, spleen, liver, heart and great vessels, and are potentially fatal Surgical emphysema

SURGERY : 

Surgery is required for Air leak persisting for more than 10 days Failure of lung re-expansion Recurrent spontaneous pneumothorax Surgical options include Partial pleurectomy Operative abrasion of pleural lining Resection of pulmonary bullae Poor-risk patients may benefit from chemical pleurodesis with tetracycline SURGERY

Prognosis : 

Prognosis Reabsorption of air and re-expansion of lung will occur over 3-12 weeks unless bronchopleural fistula or tension pneumothorax develop Respiratory failure if respiratory function is poor Recurrence rate of 10-15% within 1 year for spontaneous pneumothoraces

Marfan Syndrome : 

Marfan Syndrome An inherited connective tissue disorder with characteristic skeletal, dermatological, cardiac, aortic, ocular and dural malformations. Autosomal dominant. Symptoms: Can be asymptomatic. Ptts are disproportionately tall and thin with unusually long arms and legs compared to their trunk (dolichostenomelia) Long 'spidery' fingers and toes (arachnodactyly).

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Signs Skin – striae Heart and blood vessels - thoracic aortic dilatation/rupture/dissection, aortic regurgitation, mitral valve prolapse, mitral regurgitation, abdominal aortic aneurysm, cardiac dysrhtyhmia Eyes –lens dislocation, closed angle glaucoma, high myopia Joints – hypermobility, arthralgia, instability Skeleton – pectus excavatum or carinatum deformities, misshapen chest, kyphoscoliosis Arachnodactyly Facial characteristics - maxillary/mandibular retrognathia, long face and high, arched palate are important and relatively discriminating signs of the condition Pleural rupture causing pneumothorax

Reference : 

Reference M Henry. British Thoracic Society guidelines for the management of spontaneous pneumothorax; Thorax 2003;58:ii39-ii52 doi:10.1136/thorax.58.suppl_2.ii39 O’Hara VS. Spontaneous pneumothorax. Milit Med 1978;143:32–5.[III] 10 Jansveld CA, Dijkman JH. Primary spontaneous pneumothorax and smoking. BMJ 1975;4:559–60. [IIa] Baumann M H, Strange C. Treatment of spontaneous pneumothorax. Chest 1997; 112: 789-804. Patient.co.uk –Marfan’s syndrome