An unusual case of primary spontaneous tension pneumothorax in a jamaican female

Spontaneous pneumothorax is a well-recognized entity with a classical presentation of acute onset chest pain and shortness of breath. It may be complicated by the development of a tension pneumothorax or a haemopneumothorax. We report an interesting case of a spontaneous tension haemopneumothorax which presented atypically and was diagnosed on computed tomography (CT) scan of the chest. The clinical and pathophysiological characteristics and treatment of this unusual entity is discussed.

Jamaican female, spontaneous pneumothorax Un Caso Inusual de Neumotórax Espontáneo Primario por Tensión en una Mujer de Jamaica M Johnson1, S French1, D Cornwall2 RESUMEN El neumotórax espontáneo es una conocida entidad patológica cuya manifestación clínica clásica consiste en un dolor de pecho agudo inicial y dificultad para respirar. Se puede complicar con el desarrollo de un neumotórax por tensión o un hemoneumotórax. Reportamos como caso interesante un hemoneumotórax espontáneo por tensión, el cual se presentó de forma atípica, y fue diagnosticado mediante escaneo con tomografía computarizada (CT) del tórax. Se discuten las características clínicas y fisiopatológicas, así como el tratamiento de esta entidad patológica inusual. Palabras claves: Neumotórax espontáneo, mujer Jamaicana West Indian Med J 2014; 63 (3): 274 CASE REPORTS

A pneumothorax is defined as “air in the pleural cavity” (1). It can arise spontaneously or after blunt or penetrating chest trauma. A spontaneous pneumothorax is classified as pri- mary or secondary. A primary spontaneous pneumothorax occurs in a previously healthy patient with no lung disease while a secondary spontaneous pneumothorax is associated with parenchymal lung disease such as emphysema or pul- DOI: 10.7727/wimj.2013.301 275 and was found to have a spontaneous right tension pneu- mothorax diagnosed on radiological imaging. The clinical presentation,riskfactorsandpathophysiologyarediscussed. CASE REPORT A 21-year old female presented to the Emergency Depart- ment (ED) of the University Hospital of the West Indies (UHWI) with a one-day history of chest pain. She had no knownmedicalillnessesandwaswelluntiltheeveningprior topresentationwhenshedevelopedsuddenonsetright-sided chest pain with chest tightness post-coitus. This was des- cribedasstickinginnature,withworseningondeepinspira- tionandmovementandrelievedbyleaningforward. There wasanassociateddrycoughbutnohaemoptysis. Therewas noreportoftrauma,shortnessofbreathorpalpitations. She had no significant past medical or surgical history, but re- ported a two-year history of smoking one to two cigarettes per day. The patient reported heavy menses with no asso- ciated dysmenorrhoea or menstrual irregularity. There was nopersonalorfamilyhistoryofbleedingdiathesis. Thepa- tient had experienced these symptoms on two occasions in the past (once nine months previously and another episode one year before that). She visited a family doctor on those occasions. No abnormality was found and her symptoms resolved. Examinationrevealedayoungfemale,awakeandalert butinmoderatepainfuldistress. Herrespiratoryratewas24 breathsperminutewithanoxygensaturationof100%. Her pulsewas74/minute,bloodpressure118/72mmHgandtem- perature 35.8 °C. The examining physician noted ten- derness along the right posterolateral chest wall along the eighth and tenth ribs. Breath sounds and percussion note were documented as normal. An electrocardiogram (ECG) revealed sinus rhythm with a normal axis and an S1Q3T3 pattern. Pulmonary embolism was considered and a com- putedtomographypulmonaryangiogram(CTPA)requested. This revealed a right-sided pneumothorax with contralateral deviation of the mediastinum (Figure). The radiological impression was that of a right tension pneumothorax. The patientwasimmediatelyreturnedtotheED. Re-examination of the chest by a senior physician revealed markedly de- creased air entry on the right side with mild tracheal devia- tion. A right thoracotomy tube was immediately placed under sterile conditions. She received parenteral analgesia andwasreferredtothecardiothoracicservice. While in the ED, approximately 350 milliliters of bloodwasdrainedfromthethoracostomytube. Thepatient remained haemodynamically stable and was subsequently admitted to the surgical ward. In the ensuing 24 hours of admission, 1750 ml of blood was drainedvia the thora- costomy tube. Her haemoglobin count dropped from 10.6 grams per decilitre (g/dL) to 8.2 g/dL. A prolonged pro- thrombin time of 20 seconds (control 14 seconds) was also noted. Packed red cells and fresh frozen plasma were ordered for the patient. Vitamin K 10 mg and tranexamic acid one gram were administered parenterally. Emergency posterolateralthoracotomyandmechanicalpleurodesiswere performed. Five hundred millilitres of clotted blood was found in the pleural space. The right lung parenchyma, pleuraanddiaphragmwerenormalinappearance. Acoagul- opathywasentertainedbutsubsequentlyexcluded.Thoracos- tomytubedrainageremainedhighandachestX-raydoneon daythreeofadmission(daytwopostsurgery)showedalarge right haemothorax. Her haemoglobin dropped further from 6.5 g/dL to 4.4 g/dL, prompting exploration of the right haemothorax. Seven hundred milliliters of blood and clots and a collapsed right lung with few apical bullae was ob- served. Asmall (less than 0.5 cm) tan lesion noted on the dome of the right hemi-diaphragm was biopsied. Histo- pathologic examination subsequently revealed it to be an organizedclot.Noendometrioticdepositswereidentified. Subsequently,thepatient’schesttubedrainagebecame minimal. AchestX-raydoneondayeightofadmissionafter removal of the thoracostomy tube showed complete re- expansion of the right lung. Her haemoglobin rose to 13.3 g/dLposttransfusionofthreeunitsofpackedredbloodcells andherprothrombintimenormalized. Shehadreceivedfour unitoffreshfrozenplasma. Thepatientremainedstableand wasdischargedthreedayspostre-exploratorysurgery.

Primaryspontaneouspneumothorax(PSP)occursinpatients with no underlying lung disease. In 80−90%, subpleural bullae are found on computed tomography (CT) imaging or atsurgicalexploration(1,2). Anincreaseinpleuralporosity secondary to inflammation is another proposed mechanism bywhichPSPmayoccur(2). Factorswhichmayberelated totheoccurrenceofblebs,bullaeandpleuralporosityinclude distal airway inflammation, distal bronchial tree anomaly, Johnsonetal Figure: Computed tomography pulmonary angiogram showing a large right-sided pneumothorax with contralateral deviation of the mediastinum. There was no evidence of pulmonary embolism. (ImagecourtesyoftheRadiologyDepartment,UniversityHospital oftheWestIndies) 276 PrimarySpontaneousTensionPneumothorax connectivetissuedisorders,localischaemiaandmalnutrition (2). The disease is mainly seen in healthy, tall, thin, young males who smoke (1). The index case was a healthy, thin, young female who admitted to a two-year smoking history. She remained at home for several hours before seeking medical care. Forty-six per cent of patients with PSP wait more than two days before seeing a physician (4). Indeed 10% are asymptomatic (4). Recurrence rates range from 16−52% with 10 years of follow-up (5). Most recur within 6−12 months of first episode (5). The index case reported having similar symptoms on two previous occasions which resolved. Itisquitelikelythatshehadprevioussmallpneu- mothoraces which resolved spontaneously. Previous epi- sodes of PSP would have likely resulted in adhesions and placed her at risk for the haemothorax which developed duringthisadmission. Theadhesionsmayalsoexplainwhy, despite developing tension pneumothorax, the patient re- mained haemodynamically stable. It is quite likely that by tethering the lung, the adhesions limited the degree of tracheal deviation, preventing significant mediastinal shift andlargevesselkinking. Primary spontaneous tension pneumothorax is a rare complication of PSP. These patients typically present in extremis with obvious signs of respiratory distress and cardiovascular compromise due to impaired venous return and decreased cardiac outputas a resultof mediastinalshift (1). Examination may reveal decreased breath sounds, de- creased chest wall expansion, hyper-resonance, decreased tactilevocalfremitus,displacedapexbeatandtrachealdevi- ation(2). Inpatientswithpneumothoracesthatoccupyless than 15% to 20% of the hemithorax, these signs may be absent (2). A spontaneous tension pneumothorax may not presentwithclassicalsignssuchashaemodynamicinstability (4, 6), as was evident in this patient. One case report indicatedthata19-yearoldmalewhowasdiagnosedwitha first episode of primary spontaneous tension pneumothorax on chest X-ray was tachypnoeic and tachycardic but remained haemodynamically stable (7). Holloway and Harrisreportedonfourcasesofspontaneouspneumothorax. Inthreeofthefourpatients,thediagnosisoftensionpneumo- thorax was made radiologically. Two of these four patients were clinically stable with no tracheal deviation. In one of those four cases, the treating physician elicited the signs of spontaneouspneumothoraxandtrachealdeviationbutdidnot believehisassessmentbecausethepatientlookedsowell(8). Simpson and colleagues commented on nine patients with PSP who were diagnosed with tension pneumothorax on radiologicalimagingbuthadnoclassicclinicalfeaturesofa tensionpneumothorax(9). Spontaneoushaemopneumothoraxoccurswhengreater than 400 ml of blood accumulate in the pleural cavity in associationwithaspontaneouspneumothorax(10).Aberrant blood vessels which grow from the chest wall through adhesion bands into pleural lesions are thought to be torn whenlungcollapseoccurs(2). Bleedingbloodvesselsmay alsoarisefromthesurfaceofrupturedbullae(2). Inareviewof18cases,themostfrequentlateralizing signofatensionpneumothoraxwasdecreasedairentry(1). A more thorough clinical examination should be done to assess for the presence of mediastinal shift which may be evidenced by tracheal deviation, displaced apex beat and resonance over the sternum (8). Distended neck veins and reversible Horner’s syndrome may also be apparent in patientswithtensionpneumothorax(11). Theindexpatient had an S1Q3T3 pattern noted on her ECG. This was previouslyreportedinan18-yearoldmalewitharight-sided PSPand reversed with treatment (12). This case highlights the importance of history-taking and proper clinical examination and demonstrates that a tension pneumothorax doesnotalwayspresentinextremis. There are rare female-specific causes of spontaneous pneumothorax, namely catamenial pneumothorax and pneu- mothorax with pulmonary hamartoangiomyomatosis (13). Catamenial pneumothorax, defined as “a recurrent pneumo- thorax occurring within 72 hours from the onset of men- struation”, is the most common manifestation of thoracic endometriosis (13, 14). It is usually unilateral and right- sided(14,15).Patientswithcatamenialpneumothoraxoften have a history of pelvic endometriosis or infertility (14). Therehavebeenreportsofcasesoccurringduringorshortly aftersexualintercourse(14). InJamaica,twotosixpercent of cases of spontaneous pneumothorax in females in the reproductive age group are associated with pleural endometriosis (16). Hamartoangiomyomatosis is said to occur predominantly in young to middle-aged women and hasanoveralllowincidence(13). SurgicalstrategiesforthemanagementofPSPinclude openthoracotomyandpleurectomyorvideo-assistedthoras- copicsurgery(VATS)withpleurectomyandpleuralabrasion (1,2). Anopenapproachofferslowerrecurrencerateswhile a less invasive approach with VATS has the advantages of lower morbidity, less postoperative pain, improved pulmonaryfunctionanddecreasedlengthofhospitalstay(2). Openthoracotomyistheprocedureofchoiceinpatientswith active bleeding and haemodynamic instability (17) and was chosenintheindexcase.

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