Carotid blow out syndrome

discuss a patient presenting with impending carotid blow out with emphasize on management implications

1. Carotid blow out syndrome Mawaddah Azman

2. Summary of history 74/Chinese gentleman Radical Neck Dissection 8 th June 2011 Now presents with persistent bloody discharge from operative wound He underwent Total thyroidectomy and functional neck dissection in Feb 02 for Papillary Thyroid Carcinoma Subsequently presented with recurrent neck swelling in 2009, Selective Neck Dissection performed Oct 09 Post operative Radiotherapy given 18 cycles of 56Gy, completed Jan 2010

3. Examination Wound dehiscence over vertical limb of incision measuring 2x1.5cm with slow ooze from anterior aspect of the wound No exposed carotid artery Surrounding skin ecchymoses

4. Preoperative radiological findings Matted, necrotic nodes at right side, encasing the carotid artery and medially invading mucosa of the oropharynx Laterally the SCM is displaced and infiltrated Posteriorly prevertebral muscles are involved

5. Patient progress

6. Introduction An emergency, pose risk of Exsanguinating haemorrhage and potential bleeding diathesis Compression to airway Neurological complications Clinical spectrum Chaloupka JC, Putman CM, Citardi MJ, Ross DA, Sasaki CT. Endovascular therapy of the carotid blowout syndrome in head and neck surgical patients: diagnostic and managerial considerations. AJNR Am J Neuroradiol 1996;17:843–852

7. Incidence First recognized in 1962; with a high mortality rate – 40%; neurologic morbidity - 60% occur in 3-4% of all patients who have underwent Head and Neck Surgery 1,2 . In advanced disease this can account for 11.6% of head and neck cancer deaths 3,4 . Morrissey, D.D., Andersen, P.E. Nesbit, G.M. Barnwell, S.L. Events, E.C. Cohen, J.I. (1997) Endovascular management of haemorrhage in patients with head and neck cancer. Archives of otolaryngology, head and neck surgery; 123:15-19 Koch, W.M. (1993) Complications of surgery to the neck. In complications of head and neck surgery. Edited by Eisele D. St Louis: Mosby; 393-413 Shedd , D.P. Shedd , C. (1980) Problems of terminal head and neck cancer patients, Head and Neck Surgery, 2:476-482 Smith, A. M. (1992) Emergencies in Palliative Care, Annals Academy of Medicine, vol 23, no2, 186-190

8. Type 1: Threatened CBS Visibly exposed carotid artery segment that will inevitably rupture if not covered with a viable tissue or Evidence on diagnostic angiograms of neoplastic invasion of the carotid artery or nonhemorrhagic pseudoaneurysm Grading system: Grade 0: No evidence of vascular disruption as seen in imaging Grade 1: There is focal weakening / irregularity of the vascular wall Grade 2: There is pseudoaneurysm Grade 3: There is evidence of extravasation from the ruptured artery Citardi MJ, Chaloupka JC, Son YH, Sasaki CT. Management of carotid artery rupture by monitored endovascular therapeutic occlusion (1988–1994). Laryngoscope 1995;1086-1092 Chaloupka JC, Roth TC, Putman CM, et al. Recurrent carotid blowout syndrome: diagnostic and therapeutic challenges in a newly recognized subgroup of patients. AJNR Am J Neuroradiol 1999;20:1069–1077 From The Interventional Neuroradiology Service Yale University School of Medicine 1995

9. Type 2: Impending CBS Presents as sentinel bleeding from the neck which may precede ultimate blow out. Typically resolves spontaneously or with surgical packing The period is highly variable and can range from moments to months. Citardi MJ, Chaloupka JC, Son YH, Sasaki CT. Management of carotid artery rupture by monitored endovascular therapeutic occlusion (1988–1994). Laryngoscope 1995;1086-1092 Chaloupka JC, Roth TC, Putman CM, et al. Recurrent carotid blowout syndrome: diagnostic and therapeutic challenges in a newly recognized subgroup of patients. AJNR Am J Neuroradiol 1999;20:1069–1077 From The Interventional Neuroradiology Service Yale University School of Medicine 1995

10. Type 3: Acute CBS Acute, profuse hemorrhage that is not self-limiting and is not well-controlled with surgical packing, invariably owing to complete rupture of the affected artery. Torrential bleeding due to rupture of carotid artery. This type carries the maximum mortality since the death is nearly instantaneous. Citardi MJ, Chaloupka JC, Son YH, Sasaki CT. Management of carotid artery rupture by monitored endovascular therapeutic occlusion (1988–1994). Laryngoscope 1995;1086-1092 Chaloupka JC, Roth TC, Putman CM, et al. Recurrent carotid blowout syndrome: diagnostic and therapeutic challenges in a newly recognized subgroup of patients. AJNR Am J Neuroradiol 1999;20:1069–1077 From The Interventional Neuroradiology Service Yale University School of Medicine 1995

11. Risk factors Surgery to sites local to the carotid artery Radical Neck Dissection Generally 4% Mainly due to 3 Removal of soft tissues protecting the carotid Iatrogenic or machanical injury to adventitia of the carotids Decreased healing d2 removal of lymphatics and increased venous stasis Risk increases Salvage surgery Flap necrosis Wound infection 2 Previous irradiation 1 Recurrent tumour involving the carotid artery Cohen, J. Rad , I. (2004) Contemporary management of carotid blowout, Current opinion in otolaryngology & Head and Neck Surgery, 12: 110-115 Nieto, C.S. Solano , J.M.E, Martinez, C.B. Martin, E.F. Colunga , J.C.M. Garcia, A.A. (1980) The carotid artery in head and neck oncology, Clinical Otolaryngology, 5, 403-417 Rodriguez, F. Carmeci , C. Dalman , R.L. Lee, A. (2001) Spontaneous Late Carotid- Cutaneous Fistula following radical neck dissection- a case report. Vascular surgery, vol 35, (5)

12. Risk factors Radiotherapy Most common factor leading to CBS 2,3,4,5 Almost 100% of CBS occurs within an irradiated field Moreso if delivered within 2 months of surgery 2 Associated with a 7.6fold increase in the risk of CBS in patients with head and neck cancer 1 . Aetiology Reduced flow in the vaso vasorum blood flow to the carotid wall is reduced by 50 % after a course of 30 Gy radiotherapy course 6. Adventitial fibrosis Premature atherosclerosis Weakening of the arterial wall, sub endothelial vacuolization and oedema, and fragmentation of the tunica media elastic fibres 7 . Cohen, J. Rad , I. (2004) Contemporary management of carotid blowout, Current opinion in otolaryngology & Head and Neck Surgery, 12: 110-115 Nieto, C.S. Solano , J.M.E, Martinez, C.B. Martin, E.F. Colunga , J.C.M. Garcia, A.A. (1980) The carotid artery in head and neck oncology, Clinical Otolaryngology, 5, 403-417 Rodriguez, F. Carmeci , C. Dalman , R.L. Lee, A. (2001) Spontaneous Late Carotid- Cutaneous Fistula following radical neck dissection- a case report. Vascular surgery, vol 35, (5) Shumrick , D.A. (1973) ‘Carotid artery rupture,’ Laryngoscope, 83(7): 1051-61 Swain, R. E. et al (1974) An experimental Analysis of causative factors and protective methods in Carotid Artery Rupture. Arch Otolaryngology vol 99, April, 235-241 Smith, A. M. (1992) Emergencies in Palliative Care, Annals Academy of Medicine, vol 23, no2, 186-190 Huvos , A.G. Leaming , R.H. Moore, O.S. (1973) Clinicopathologic study of resected carotid artery: analysis of 64 cases. American journal of surgery, 126:570-574 Lesarge , C. (1986)

13. Risk factors Postoperative impaired healing due to previous radiotherapy, infection and excision of the lymphatic chains The carotid artery can be exposed, flap necrosis can occur, which allows the invasion of bacteria and further desiccation of the adventitia 1-5 . Improper incision: vertical limb or three point junction 6 Cohen, J. Rad , I. (2004) Contemporary management of carotid blowout, Current opinion in otolaryngology & Head and Neck Surgery, 12: 110-115 Lesarge , C. (1986) ‘Carotid artery rupture’. Prediction, prevention and preparation. Cancer Nursing, 9 (1) 1-7 Nieto, C.S. Solano , J.M.E, Martinez, C.B. Martin, E.F. Colunga , J.C.M. Garcia, A.A. (1980) The carotid artery in head and neck oncology, Clinical Otolaryngology, 5, 403-417 Shumrick , D.A. (1973) ‘Carotid artery rupture,’ Laryngoscope, 83(7): 1051-61 Swain, R. E. et al (1974) An experimental Analysis of causative factors and protective methods in Carotid Artery Rupture. Arch Otolaryngology vol 99, April, 235-241 Maran , A.G.D. Amin M.A. Wilson, J.A. (1989) Radical neck dissection: a 19 year experience. The Journal of Laryngology and Otology, August, vol.103 pp 760-764

14. Risk factors Pharyngocutaneous fistula important causative factor in CBS. adventitia being bathed in saliva, which is bacteria laden and damaging to the outer lining of the arterial wall 1,3 Fungating tumour invading the carotid artery Direct infiltration destructing the arterial wall Tumour necrosis increasing vulnerability of the arterial wall Nieto, C.S. Solano , J.M.E, Martinez, C.B. Martin, E.F. Colunga , J.C.M. Garcia, A.A. (1980) The carotid artery in head and neck oncology, Clinical Otolaryngology, 5, 403-417 Shumrick , D.A. (1973) ‘Carotid artery rupture,’ Laryngoscope, 83(7): 1051-61 Swain, R. E. et al (1974) An experimental Analysis of causative factors and protective methods in Carotid Artery Rupture. Arch Otolaryngology vol 99, April, 235-241

15. Risk factors General systemic Over 50 years of age 10-15% loss of body weight Diabetes mellitus and immune deficiencies Generalised atherosclerosis Malnourishment Nieto, C.S. Solano , J.M.E, Martinez, C.B. Martin, E.F. Colunga , J.C.M. Garcia, A.A. (1980) The carotid artery in head and neck oncology, Clinical Otolaryngology, 5, 403-417 Lesarge , C. (1986) ‘Carotid artery rupture’. Prediction, prevention and preparation. Cancer Nursing, 9 (1) 1-7 Schiech , L. (2000) Carotid artery rupture. Clinical Journal of Oncology Nursing, vol 4, pp93-94 Shumrick , D.A. (1973) ‘Carotid artery rupture,’ Laryngoscope, 83(7): 1051-61 Swain, R. E. et al (1974) An experimental Analysis of causative factors and protective methods in Carotid Artery Rupture. Arch Otolaryngology vol 99, April, 235-241

16. Clinical feature Attempt to predict patients most likely to be at risk as sometimes there may be no warning at all ‘Sentinel bleeds’ or ‘herald bleeds’ minor bleeding from wound, flap site, tracheostomy or mouth 2,3 process of erosion is gradual 4 this is caused by a small rupture of the intima at the site of the defect of the tunica which seals temporarily. 1 Nieto, C.S. Solano , J.M.E, Martinez, C.B. Martin, E.F. Colunga , J.C.M. Garcia, A.A. (1980) The carotid artery in head and neck oncology, Clinical Otolaryngology, 5, 403-417 Lovel , T. (2000) Palliative care and head and neck cancer. Editorial, British Journal of Oral and Maxillofaxillofacial Surgery, 38, 253-254 Schiech , L. (2000) Forbes, K. (1997) Palliative care in head and neck cancer. Clinical Otolaryngology 22:117-22 Macmillan, K. Stuthers , C. (1987) Algorithm for the Emergency Nursing Management of spontaneous Carotid Artery Rupture. Canadian Critical Care Nursing Journal- March/April, 20-21

17. Clinical feature ‘Pulsations’ from artery or tracheostomy or flapsite 1,2 . ‘Ballooning’ of an artery 2,4,5 . Haemorrhage externally from the neck internally from within the oropharynx , directly into the airway or tracheostomy Death due to: Hypovolaemic shock is often the cause of death. Asphyxiation of blood may also be a contributory factor. Cerebral hypoxia Kane, K. K. (1983) ‘Carotid artery rupture on Advanced Head and Neck Cancer Patients’, Oncology Nursing Forum Vol 10, No.1, 14-18 Casey, D. (1988) ‘Carotid “Blow-out”’. Nursing Standard 2 (47): 30 Parsons, R. (1995) Practice Guidelines, carotid Artery rupture, Fall, vol 13, no.4, 30-31 Luo , C.B. Chang, F.C. Mu- Huo Teng , M. Chi-Chang Chen, C. Feng Lirng , J. Cheng, Y. (2003) Endovascular treatment of the carotid artery rupture with massive haemorrhage, Journal of Chinese medical Association, 66, 140-147 Schiech , L. (2000) Carotid artery rupture. Clinical Journal of Oncology Nursing, vol 4, pp93-94

18. Patophysiology Adventitial layer protects the artery, nourished by vasovasorum . Interrupted blood supply due to various reasons causes destruction of arterial wall occuring over 6-10 days 1,2 Damage and loss of adventitia forming eschar and slough Exposure of tunica media Sloughing of tunica media Exposure of tunica intima with subsequent thinning Eruption of blood Lesarge , C. (1986) ‘Carotid artery rupture’. Prediction, prevention and preparation. Cancer Nursing, 9 (1) 1 Kane, K. K. (1983) ‘Carotid artery rupture on Advanced Head and Neck Cancer Patients’, Oncology Nursing Forum Vol 10, No.1, 14-18

19. First case in late 18 th century Dr John Abernathy Traumatic laceration of the ICA after being gored in the neck by bull’s horn Treated with ligation of the vessel, well tolerated by the patient

20. Management What has changed? Historically CBS was associated with 60% neurologic morbidity and 40 % mortality Open surgical ligation Outcomes substantially improved with the advent of various endovascular surgical techniques Permanent balloon occlusion (15-20% neurologic morbidity) Endovascular Reconstruction of Carotid Artery (8% neurologic morbidity) Debate over indication and selection of patients Chaloupka JC, Roth TC, Putman CM, et al. Recurrent carotid blowout syndrome: diagnostic and therapeutic challenges in a newly recognized subgroup of patients. AJNR Am J Neuroradiol 1999;20:1069–1077 Chaloupka JC, Putman CM, Citardi MJ, Ross DA, Sasaki CT. Endovascular therapy of the carotid blowout syndrome in head and neck surgical patients: diagnostic and managerial considerations. AJNR Am J Neuroradiol 1996;17:843–852 Citardi MJ, Chaloupka JC, Son YH, Ariyan S, Sasaki CT. Management of carotid artery rupture by monitored endovascular therapeutic occlusion (1988 –1994). Laryngoscope 1995;105:1086–1092

21. Yale 1995: simple clinical classification scheme with interdisciplinary treatment algorithm

22. Imaging modalities CT/MR Gold standard DSA Selective catheterization of each common carotid, external carotid and or internal carotid artery Active extravasation Pseudoaneurysm Tumour bleeding (nodal or primary) Assess intracranial circulation prior to intervention (surgery or endovascular) Selective carotid and vertebral injection Incomplete circle of Willis

23. Management MDT meeting

24. Management of non terminal bleeding Resuscitation Airway Breathing Circulation Large bore branullas Volume replacement, preferably with blood Measurement of volume status Specific measures Compression Packing Hemostatic material Endovascular techniques Operative ligation

25. Endovascular treatment of CBS Evolved since 1980’s Divided into Deconstructive techniques: permanently occluding Reconstructive techniques: preserving flow Percutaneous Balloon Occlusion Using a detachable balloon (latex or silicone) Rapid occlusion of a large vessel can be achieved, hence more suitable for emergent conditions Multiple balloons can be used in the same setting Achieve success rate of 95% in Type 2 &3 CBS Embolization with coils (platinum based), polyvinyl alcohol or cyanoacrylate .

26. Reconstructive techniques Using overlapping or covered stents to diminish “porosity” between the stent struts. Promote sluggish flow and subsequent thrombosis around the stent Allows blood flow through stent and strengthen integrity of vessel Confirmed by second look angiography Technically more demanding and time consuming Indicated in patients at high risk for carotid occlusion: Angiographic documentation of incomplete circle of willis Contralateral carotid artery occlusion Adjunct embolization with coil or glue if persistent filling of the pseudoaneurysm occurs Chaloupka , J.C. Lesley W.S.Weigele J.B. (2003) Endovascular reconstruction for the management of carotid blow-out syndrome. American journal of neuroradiology ; 24: 975-981

27. Long term outcomes Although deployment of stent-grafts can achieve immediate and initial hemostasis in patients with head-and-neck cancer and CBS, the long-term safety, stent patency, and permanency of hemostasis appear unfavorable . Complications: Rebleeding : periprocedure patients need to be on antiplatelet therapy Thrombosis Persistent infection : reported brain abscess

28. Emergent endovascular techniques Direct carotid puncture Technically feasible for rapid arrest of haemorrhage in unstable patients

29. Open technique Principles: Often done in emergency setting hence less time for planning Ligate more proximally Ligation is preferable if there is multi level rupture or multiple pseudoaneurysm Site of ligature must always be covered with a thick viable muscle flap and is not infected Preferable in clinically unstable patients Provides rapid securing of bleeding Technically less demanding compared to endovascular technique

30. Ligation of Common Carotid Artery Carries significant neurologic morbidity and mortality due to variable intracranial cross circulation Ideally preceded with balloon occlusion test or angiography of collateral circulation Above the omohyoid Transverse incision middle portion of SCM Fascia at anterior border of SCM longitudinally incised SCM retracted posteriorly Omohyoid tendon retracted downwards Carotid sheath opened IJV retracted laterally Mobilize the CCA, free from the vagus and ligate CCA

31. Below the omohyoid Transverse incision at lower portion of SCM Anterior jugular vein ligated Fascia at anterior border of SCM longitudinally incised, omohyoid transected Inferiorly, carotid sheath is covered by omoclavicular fascia Omoclavicular fascia exposed Carotid sheath opened IJV retracted laterally Mobilize the CCA, free from the vagus and ligate CCA

32. The branches of the external carotid anastomose across the median line . S uperior thyroid Facial T he internal carotids communicate by means of the circle of Willis. From the subclavian the vertebral artery communicates by means of the basilar with the circle of Willis. The thyroid axis by its inferior thyroid branch communicates with the thyroid arteries of the opposite side. Finally the superior intercostal , which, like the vertebral and thyroid axis, is a branch of the first portion of the subclavian , through its profunda cervicis branch anastomoses with a deep descending branch of the princeps cervicis

33. Options: Ligation End-to-end anastomosis if the rupture is small and one level Interpositional grafts for reconstruction: Autologous graft: Saphenous vein Synthetic grafts: PTFE ( Polytetrafluoroethylene ) Dacron Theoretical as usually ligation is life saving in emergency situations and rarely anastomosis or graft reconstruction is attempted

34. Muscular flaps Levator Scapulae flap; is an option during radical neck dissection Inferior border of muscle divided, taking care not to damage the brachial plexus Posterior border of the muscle flipped anteriorly to cover the carotid artery and stitched to the sternohyoid muscle

35. Preparation for the event The Consultant in charge of the patient, accompanied will break the news of the likely occurrence of a CBS and its implications. The information should ensure that patients/relatives have a clear plan of care and are aware that NO resuscitation will take place; this must then be documented. In cases where herald bleeding occurs, patients and their families will undoubtedly have been extremely frightened and distressed by this experience. This may have been an event which had been unpredicted and which they were not prepared for, in which case it may be possible to explain how the experience may be helped in the future with better preparation. They may be comforted by the knowledge that sedation will be available and that they will be supported both at home and in a hospital/hospice setting.

36. Open and honest approach 1,2,4 . Contemplating the truth, knowing what to expect, what to do, and how distress can be relieved can be helpful to the patient and family 4 . It may also help the patient and family to know that, in the event of a massive carotid rupture there should be little pain and that death is usually very quick 3,5 . When? Feber , T. (2000) Head and Neck Oncology Nursing. Whurr Publishers Ltd, London Chapter 2.8, 245 – 252 Forbes, K. (1997) Palliative care in head and neck cancer. Clinical Otolaryngology 22:117-22 Cohen, J. Rad , I. (2004) Contemporary management of carotid blowout, Current opinion in otolaryngology & Head and Neck Surgery, 12: 110-115 Kane, K. K. (1983) ‘Carotid artery rupture on Advanced Head and Neck Cancer Patients’, Oncology Nursing Forum Vol 10, No.1, 14-18 Smith, A. M. (1992) Emergencies in Palliative Care, Annals Academy of Medicine, vol 23, no2, 186-190

37. The Event Equipments to be made available: Call bell Suction Syringes (10ml) for cuff inflation on a tracheostomy tube (if appropriate) Bowl Gloves, Plastic apron, eye protector/face shield Dark coloured towels Bedside locker with attached individual drug cabinet Midazolam ampoules, syringes, needles and alcohol wipes Patent IV access Patients should be nursed in a side ward to avoid shock and distress to other patients and relatives where possible

38. The Event Stay with the patient, hold their hand and call for assistance calmly 1 . Be aware of family presence and needs. Decide beforehand with the family if they wish to stay with the patient. Draw curtains and maintain the privacy of the patient as much as possible 1 . Apply towels around the bleeding site to absorb the blood loss. If a cuffed tracheostomy tube is insitu , inflate the cuff 2 . Apply gentle suctioning to mouth and tracheostomy site as necessary 2 . Administer Midazolam intravenously 2 . In the event of a massive, terminal bleed the patient may be unconscious within minutes and may die very quickly, even before the sedation has had a chance to work. Thus it is important to remember that whilst sedation is important, never leave the patient alone, and stay with them at all times. Kane, K. K. (1983) ‘Carotid artery rupture on Advanced Head and Neck Cancer Patients’, Oncology Nursing Forum Vol 10, No.1, 14-18 Smith, A. M. (1992) Emergencies in Palliative Care, Annals Academy of Medicine, vol 23, no2, 186-190

39. Use of Benzodiazepines Administration: Rapid bolus intravenous injection For anxiolysis and sedation where a catastrophic bleed occurs, give 5mg as a fast bolus. (If no IV access is available give 5 – 10 mg as a Subcutaneous or Intramuscular injection. Further doses may be given until the patient is fully sedated. Morphine is not indicated unless: Patient complains of pain and or breathlessness Patient already on regular opiates

40. Caring for the patient at home Many patients may wish to go home and may not wish to stay in hospital, ‘waiting to bleed’. The approach of death can evoke feelings of loss in a dying patient. Loss of control may be the most overwhelming and distressing feeling, which is often further intensified by hospitalization. Being at home may give the patient and family privacy, control over their surroundings, and may help the patient to retain their own identity. The team should discuss a management plan with the patient and family. A palliative team should be available to visit the patient and cater to any needs. Bourne, V. Frogge , M.H. (1999) Grief, in Yarbro CH, Frogge MH, Goodman ( eds ) Cancer symptom management ( ed 2). Sudbury, MA Jones and Bartlett, 618-626

41. Thank you Questions?

42. Sometimes, it may be necessary to block off a blood vessel as the primary means of treating a problem. If that blood vessel supplies the brain, doing so might result in a stroke. However, at the base of the brain, there can be connecting vessels which can take over the blood supply. Because we are not certain, in some patients, that these connections are sufficient, we do a test beforehand. This testing involves temporarily blocking off the blood flow in the vessel we are interested in permanently blocking. We do this with a small, soft balloon placed in the artery. With the balloon in place and inflated, we can then perform a neurological examination on the patient to make sure there is no problem. If the blood flow is insufficient, the patient will start to develop neurological impairment, such as weakness, loss of sensation, speech problems, etc. When this happens, the balloon is deflated to restore the normal blood flow. Depending upon these results, we can then decide on the most appropriate course of therapy. EEG during the procedure Angiography of collateral vessels