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Diagnosis of Factor VII Deficiency in an Adolescent Following Spontaneous Hemopneumothorax
March 2014, Vol 145, No. 3_MeetingAbstracts Disorders of the Pleura | March 2014
Diagnosis of Factor VII Deficiency in an Adolescent Following Spontaneous Hemopneumothorax Raja Dhar, MD; Arjun Ravi, MBBS Fortis Hospital, Kolkata, India
Chest. 2014;145(3_MeetingAbstracts):264A. doi:10.1378/chest.1834404
Abstract SESSION TITLE: Pleural Case Report Posters SESSION TYPE: Case Report Poster PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM INTRODUCTION: We present the case of a 15 year old boy who presented with a spontaneous haemopneumothorax and was subsequently found to have Factor VII deficiency. CASE PRESENTATION: A 15 year old schoolboy presented as an emergency admission with a history of left sided pleuritic chest pain and dyspnoea after a fall while playing football. Examination of chest revealed findings consistent with a left sided pleural effusion. The Chest Xray showed a left sided Hydropneumothorax. He subsequently underwent CT scan of thorax. This revealed a moderately large left haemopneumothorax, with no active focus of intercostal bleeding or rib fracture. His blood picture showed a normal HB (14.7g/dl) and platelet counts (274), but prolonged PT (17.5). 28 French chest drain was inserted which drained blood stained fluid. The chest drain was removed, a total of 850ml of blood stained fluid having been drained.A clotting factor assay identified that the patient’s coagulopathy was attributable to a reduction in factor VII levels to 15-16% of normal, which was attributable to a congenital haemostatic defect. Two units of fresh frozen plasma were administered prior to removal of the intercostal drain. Six weeks after discharge, the patient underwent a further CT scan of thorax which did not show any bullae or potential source of bleeding.
DISCUSSION: SHP occurs in 1 to 12% of all cases of spontaneous pneumothoraces. It is defined as an accumulation of >400ml of blood in the pleural space associated with a spontaneous pneumothorax1. Three mechanisms have been postulated to explain the occurrence of SHP: 1) Torn adhesions between visceral & parietal pleura, 2) rupture of vascularized bullae & 3) torn congenital aberrant vessels1. The treatment modalities for SHP comprise of Tube Thoracostomy followed by either Video Assisted Thoracoscopic Surgery (VATS) or Open Thoracostomy2. It has been suggested that conservative management with tube thoracostomy alone may suffice in patients who are haemodynamically stable and who do not demonstrate any of the following indicators for surgery: 1) hypovolaemic shock, 2) continuous bleeding (>100ml/hour), 3) persistent air leak, 4) impaired lung expansion, 5) pachypleuritis & 6) recurrent pneumothorax1 Factor VII deficiency is an autosomal recessive condition affecting 1 in 500,000 individuals. CONCLUSIONS: We believe this to be the first recorded case of Factor VII deficiency presenting with SHP. Management should be as for SHP with specialist advice from the haematological team. Reference #1: Hsu N-Y, Shih C-S, Hsu C-P, Chen P-R. Spontaneous Haemopneumothorax revisited: Clinical Approach and systematic review of the literature. Annals of Thoracic Surgery. 2005;80:1859-1863. Reference #2: Ali H A, Lippmann M, Mundathaje U, Khaleeq G. Spontaneous Pneumothorax A Comprehensive Review. Chest. 2008;134:1056-1065 DISCLOSURE: The following authors have nothing to disclose: Raja Dhar, Arjun Ravi No Product/Research Disclosure Information
Diagnosis of Factor VII Deficiency in an Adolescent Following Spontaneous Hemopneumothorax Raja Dhar, MD; Arjun Ravi, MBBS Fortis Hospital, Kolkata, India
Chest. 2014;145(3_MeetingAbstracts):264A. doi:10.1378/chest.1834404
Abstract SESSION TITLE: Pleural Case Report Posters SESSION TYPE: Case Report Poster PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM INTRODUCTION: We present the case of a 15 year old boy who presented with a spontaneous haemopneumothorax and was subsequently found to have Factor VII deficiency. CASE PRESENTATION: A 15 year old schoolboy presented as an emergency admission with a history of left sided pleuritic chest pain and dyspnoea after a fall while playing football. Examination of chest revealed findings consistent with a left sided pleural effusion. The Chest Xray showed a left sided Hydropneumothorax. He subsequently underwent CT scan of thorax. This revealed a moderately large left haemopneumothorax, with no active focus of intercostal bleeding or rib fracture. His blood picture showed a normal HB (14.7g/dl) and platelet counts (274), but prolonged PT (17.5). 28 French chest drain was inserted which drained blood stained fluid. The chest drain was removed, a total of 850ml of blood stained fluid having been drained.A clotting factor assay identified that the patient’s coagulopathy was attributable to a reduction in factor VII levels to 15-16% of normal, which was attributable to a congenital haemostatic defect. Two units of fresh frozen plasma were administered prior to removal of the intercostal drain. Six weeks after discharge, the patient underwent a further CT scan of thorax which did not show any bullae or potential source of bleeding.
DISCUSSION: SHP occurs in 1 to 12% of all cases of spontaneous pneumothoraces. It is defined as an accumulation of >400ml of blood in the pleural space associated with a spontaneous pneumothorax1. Three mechanisms have been postulated to explain the occurrence of SHP: 1) Torn adhesions between visceral & parietal pleura, 2) rupture of vascularized bullae & 3) torn congenital aberrant vessels1. The treatment modalities for SHP comprise of Tube Thoracostomy followed by either Video Assisted Thoracoscopic Surgery (VATS) or Open Thoracostomy2. It has been suggested that conservative management with tube thoracostomy alone may suffice in patients who are haemodynamically stable and who do not demonstrate any of the following indicators for surgery: 1) hypovolaemic shock, 2) continuous bleeding (>100ml/hour), 3) persistent air leak, 4) impaired lung expansion, 5) pachypleuritis & 6) recurrent pneumothorax1 Factor VII deficiency is an autosomal recessive condition affecting 1 in 500,000 individuals. CONCLUSIONS: We believe this to be the first recorded case of Factor VII deficiency presenting with SHP. Management should be as for SHP with specialist advice from the haematological team. Reference #1: Hsu N-Y, Shih C-S, Hsu C-P, Chen P-R. Spontaneous Haemopneumothorax revisited: Clinical Approach and systematic review of the literature. Annals of Thoracic Surgery. 2005;80:1859-1863. Reference #2: Ali H A, Lippmann M, Mundathaje U, Khaleeq G. Spontaneous Pneumothorax A Comprehensive Review. Chest. 2008;134:1056-1065 DISCLOSURE: The following authors have nothing to disclose: Raja Dhar, Arjun Ravi No Product/Research Disclosure Information