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Traumatic disruption of the subclavian artery and brachial plexus in a patient with Ehlers-Danlos syndrome
CASE REPORT syndrome, Ehlers-Danlos, complications
Traumatic Disruption of the Subclavian Artery and Brachial Plexus in a Patient With Ehlers-Danlos Syndrome A 17-year-old gift with Ehlers-Danlos syndrome underwent simple reduction of a dislocated shoulder that was complicated by disruption of the subclavian artery and brachial plexus. The shoulder dislocation was a recurrent condition that had been treated successfully on several previous occasions without complication. During the relocation process, only minor upper extremity manipulation caused injury to the brachial plexus and subclavian artery. Management of an iatrogenic vascular injury was made difficult by the fragile consistency of the anteriai wall that would not hold sutures. Amputation of the patient's arm ultimately was required. [Curley SA, Osler T, Demarest GB: Traumatic disruption of the subclavian artery and brachial plexus in a patient with Ehlers-Danlos syndrome. Ann Emerg Med August 1988;17:850-852.]
INTRODUCTION Ehlers-Danlos syndrome (EDS) is a rare hereditary connective tissue disorder. Eight distinct variants of EDS have been described. 1 The autosomal recessive Type IV, also known as the vascular form of EDS, is the most medically hazardous because of the risk of vascular or intestinal rupture. 1 We present the case of a patient with subclavian artery and brachial plexus disruption after reduction of an anterior shoulder dislocation.
Steve A Curley, MD Turner Osier, MD Gerald B Demarest, MD Albuquerque, New Mexico From the University of New Mexico, School of Medicine, Albuquerque, New Mexico. Received for publication December 21, 1987. Revision received April 18, 1988. Accepted for publication April 25, 1988. Address for reprints: Gerald B Demarest, MD, University of New Mexico Hospital, Department of Surgery, 2211 Lomas NE, Albuquerque, New Mexico 87131.
CASE REPORT A 17-year-old, severely scoliotic girl with Type IV EDS presented to a community hospital with a left anterior shoulder dislocation that occurred when she rolled over in bed. The patient had had two previous left shoulder dislocations from a similar mechanism. The patient's brother died at age 16 from a spontaneous rupture and dissection of the aorta. The dislocation was reduced in the following manner. A bed sheet was wrapped around the patient's body at the level of the axilla. This was held by an assistant as a means of countertraction. The left upper extremity was pulled inferiorly and then abducted. During the reduction, there was a give noted in the shoulder joint that was thought to imply reduction. Suddenly, the patient complained of acute loss of sensation in the left arm. Examination revealed markedly increasing swelling of the left anterior pectoral region and complete loss of motor and sensory function of the arm as well as absence of brachial and radial pulses. She was transported by helicopter to the University of New Mexico Hospital Regional Trauma Center. On admission, the patient had a blood pressure of 70/0 m m Hg and a heart rate of 130. A rapidly expanding infraclavicular left chest mass was noted. IV fluid and blood resuscitation, which included 2,000 mL Ringer's lactate and four units of packed red blood cells for 90 minutes, was instituted. An emergency arch aortagram was obtained. Complete disruption of the left subclavian artery was demonstrated with active bleeding (Figure 1). The patient was taken immediately to the operating room, and a left anterolateral thoracotomy was performed. Exposure of the subclavian artery revealed complete avulsion of the vessel just distal the takeoff of the vertebral artery with active bleeding. Proximal and distal control of the artery was difficult because of extensive tearing of the vessel with application of vascular clamps or ligatures. Metal hemoclips were required to control hemorrhage. The brachial plexus was completely disrupted and shredded (Figure 2). 17:8 August 1988
Annals of Emergency Medicine
850/127
SUBCLAVIAN ARTERY DISRUPTION Curley, Osier, & Demarest
FIGURE 1. Transected left subclavian artery with extravasation of contrast material. FIGURE 2. Thoracotomy incision
with disrupted brachial plexus. Because of the extreme fragility of the artery and loss of innervation of the arm, direct vascular reconstruction or repair by vein graft interposition was not attempte d . Three days after the initial operation, the patient underwent left m i d h u m e r a l a m p u t a t i o n . The t h o r a c o t o m y and a m p u t a t i o n wounds healed without complication, but the patient required mechanical ventilatory support for almost eight months because of her severe sco!iosis. Recovery was c o m p l i c a t e d by post-surgery adult respiratory distress s y n d r o m e and r e c u r r e n t b o u t s of p n e u m o n i a . U l t i m a t e l y , she succumbed to these complications. DISCUSSION EDS occurs in individuals with a genetic defect in the pathway of collagen biosynthesis. Type IV EDS is caused by a reduced rate of production of Type III collagen, 1 which is essential for normal tensile strength of skin, aorta, and large intestine arteries. ~ As a result of the deficiency of Type III collagen, patients with Type IV EDS have skin that is thin, translucent, and easily torn. Blood vessels are prominent beneath the fragile skin. The skin hyperextensibility seen in other types of EDS is absent in Type W. While skin injury and subcutaneous h e m a t o m a s occur frequently in patients with Type IV EDS, complications or death related to vascular or i n t e s t i n a l r u p t u r e are m o r e problematic. Spontaneous perforation of intestine, most commonly the colon, presents with typical findings of generalized peritonitis.3, 4 Standard surgical management with resection of the involved segment of bowel and colostomy or ileostomy is appropriate. 4 Abdominal pain in patients with EDS may also result from vascular catastrophe. Ruptured aneurysms of the aorta, iliac arteries, and visceral arteries have been described with rare survivals.g, s Awareness of this possibility requires careful hemodynamic monitoring and preparation before operation. Spontaneous rupture of the subclavian artery in patients with EDS has 128/851
been reported in two cases.67 The combination of subcla~/ian artery and brachial plexus disruption has not been described previously. Even trivial trauma can lead to tissue and vessel injury in these patients. In this case, the simple reduction of a dislocated shoulder performed without unusual force led to devastating tissue damage. The extremely friable subclavian artery made hemorrhage challenging to Annals of Emergency Medicine
control. As in this case, vascular reconstruction usually is neither possible nor prudent.
SUMMARY EDS is a constellation of enzymatic disorders of collagen biosynthesis. Type IV EDS is the most hazardous because of deficiency of Type III collagen in skin, intestine, and large arteries causing skin fragility, spon17:8 August 1988
taneous bowel perforation, and arterial aneurysms with spontaneous disruption. An awareness of these problems and m a n a g e m e n t issues in patients with Type IV EDS is exemplified by this first kmown case of traumatic disruption of the subclavian artery and brachial plexus in a patient with EDS.
Patients with Ehlers-Danlos syndrome type IV lack type III collagen. Proc Natl Acad Sci USA 1975;72:1314-1316.
REFERENCES
4. Sykes EM: Colon perforation in EhlersDanlos syndrome. A m J Surg 1984;147:410-413.
1. Pope FM, Martin GR, Lichtenstein JR, et al:
17:8 August 1988
2. Prockop DJ, Kivirikko KI, Tunderman L, et al: The biosynthesis of collagen and its disorders. N Engl J Med 1979;301:77-85. 3. Silva R, Cogbill TH, Hansbrough JF, et ah Intestinal perforation and vascular rupture in E h l e r s - D a n l o s s y n d r o m e . Int Surg 1986;71: 48-50.
Annals of Emergency Medicine
5. G e r t s c h P, Loup PW, L o c h m a n A, et ah C h a n g i n g p a t t e r n s in the vascular form of Ehlers-Danlos syndrome. Arch Surg 1986;I21: 1061-1064. 6. Wesley JR, Mahout GH, Woolley MM: Multiple surgical problems in two patients with E h l e r s - D a n l o s s y n d r o m e . Surgery 1980;87: 319-324. 7. McFarland W, Fuller DE: Mortality in EhlersDanlos syndrome due to spontaneous rupture of large arteries. N Engl J Med 1964;271:1309-1310.
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Traumatic Disruption of the Subclavian Artery and Brachial Plexus in a Patient With Ehlers-Danlos Syndrome A 17-year-old gift with Ehlers-Danlos syndrome underwent simple reduction of a dislocated shoulder that was complicated by disruption of the subclavian artery and brachial plexus. The shoulder dislocation was a recurrent condition that had been treated successfully on several previous occasions without complication. During the relocation process, only minor upper extremity manipulation caused injury to the brachial plexus and subclavian artery. Management of an iatrogenic vascular injury was made difficult by the fragile consistency of the anteriai wall that would not hold sutures. Amputation of the patient's arm ultimately was required. [Curley SA, Osler T, Demarest GB: Traumatic disruption of the subclavian artery and brachial plexus in a patient with Ehlers-Danlos syndrome. Ann Emerg Med August 1988;17:850-852.]
INTRODUCTION Ehlers-Danlos syndrome (EDS) is a rare hereditary connective tissue disorder. Eight distinct variants of EDS have been described. 1 The autosomal recessive Type IV, also known as the vascular form of EDS, is the most medically hazardous because of the risk of vascular or intestinal rupture. 1 We present the case of a patient with subclavian artery and brachial plexus disruption after reduction of an anterior shoulder dislocation.
Steve A Curley, MD Turner Osier, MD Gerald B Demarest, MD Albuquerque, New Mexico From the University of New Mexico, School of Medicine, Albuquerque, New Mexico. Received for publication December 21, 1987. Revision received April 18, 1988. Accepted for publication April 25, 1988. Address for reprints: Gerald B Demarest, MD, University of New Mexico Hospital, Department of Surgery, 2211 Lomas NE, Albuquerque, New Mexico 87131.
CASE REPORT A 17-year-old, severely scoliotic girl with Type IV EDS presented to a community hospital with a left anterior shoulder dislocation that occurred when she rolled over in bed. The patient had had two previous left shoulder dislocations from a similar mechanism. The patient's brother died at age 16 from a spontaneous rupture and dissection of the aorta. The dislocation was reduced in the following manner. A bed sheet was wrapped around the patient's body at the level of the axilla. This was held by an assistant as a means of countertraction. The left upper extremity was pulled inferiorly and then abducted. During the reduction, there was a give noted in the shoulder joint that was thought to imply reduction. Suddenly, the patient complained of acute loss of sensation in the left arm. Examination revealed markedly increasing swelling of the left anterior pectoral region and complete loss of motor and sensory function of the arm as well as absence of brachial and radial pulses. She was transported by helicopter to the University of New Mexico Hospital Regional Trauma Center. On admission, the patient had a blood pressure of 70/0 m m Hg and a heart rate of 130. A rapidly expanding infraclavicular left chest mass was noted. IV fluid and blood resuscitation, which included 2,000 mL Ringer's lactate and four units of packed red blood cells for 90 minutes, was instituted. An emergency arch aortagram was obtained. Complete disruption of the left subclavian artery was demonstrated with active bleeding (Figure 1). The patient was taken immediately to the operating room, and a left anterolateral thoracotomy was performed. Exposure of the subclavian artery revealed complete avulsion of the vessel just distal the takeoff of the vertebral artery with active bleeding. Proximal and distal control of the artery was difficult because of extensive tearing of the vessel with application of vascular clamps or ligatures. Metal hemoclips were required to control hemorrhage. The brachial plexus was completely disrupted and shredded (Figure 2). 17:8 August 1988
Annals of Emergency Medicine
850/127
SUBCLAVIAN ARTERY DISRUPTION Curley, Osier, & Demarest
FIGURE 1. Transected left subclavian artery with extravasation of contrast material. FIGURE 2. Thoracotomy incision
with disrupted brachial plexus. Because of the extreme fragility of the artery and loss of innervation of the arm, direct vascular reconstruction or repair by vein graft interposition was not attempte d . Three days after the initial operation, the patient underwent left m i d h u m e r a l a m p u t a t i o n . The t h o r a c o t o m y and a m p u t a t i o n wounds healed without complication, but the patient required mechanical ventilatory support for almost eight months because of her severe sco!iosis. Recovery was c o m p l i c a t e d by post-surgery adult respiratory distress s y n d r o m e and r e c u r r e n t b o u t s of p n e u m o n i a . U l t i m a t e l y , she succumbed to these complications. DISCUSSION EDS occurs in individuals with a genetic defect in the pathway of collagen biosynthesis. Type IV EDS is caused by a reduced rate of production of Type III collagen, 1 which is essential for normal tensile strength of skin, aorta, and large intestine arteries. ~ As a result of the deficiency of Type III collagen, patients with Type IV EDS have skin that is thin, translucent, and easily torn. Blood vessels are prominent beneath the fragile skin. The skin hyperextensibility seen in other types of EDS is absent in Type W. While skin injury and subcutaneous h e m a t o m a s occur frequently in patients with Type IV EDS, complications or death related to vascular or i n t e s t i n a l r u p t u r e are m o r e problematic. Spontaneous perforation of intestine, most commonly the colon, presents with typical findings of generalized peritonitis.3, 4 Standard surgical management with resection of the involved segment of bowel and colostomy or ileostomy is appropriate. 4 Abdominal pain in patients with EDS may also result from vascular catastrophe. Ruptured aneurysms of the aorta, iliac arteries, and visceral arteries have been described with rare survivals.g, s Awareness of this possibility requires careful hemodynamic monitoring and preparation before operation. Spontaneous rupture of the subclavian artery in patients with EDS has 128/851
been reported in two cases.67 The combination of subcla~/ian artery and brachial plexus disruption has not been described previously. Even trivial trauma can lead to tissue and vessel injury in these patients. In this case, the simple reduction of a dislocated shoulder performed without unusual force led to devastating tissue damage. The extremely friable subclavian artery made hemorrhage challenging to Annals of Emergency Medicine
control. As in this case, vascular reconstruction usually is neither possible nor prudent.
SUMMARY EDS is a constellation of enzymatic disorders of collagen biosynthesis. Type IV EDS is the most hazardous because of deficiency of Type III collagen in skin, intestine, and large arteries causing skin fragility, spon17:8 August 1988
taneous bowel perforation, and arterial aneurysms with spontaneous disruption. An awareness of these problems and m a n a g e m e n t issues in patients with Type IV EDS is exemplified by this first kmown case of traumatic disruption of the subclavian artery and brachial plexus in a patient with EDS.
Patients with Ehlers-Danlos syndrome type IV lack type III collagen. Proc Natl Acad Sci USA 1975;72:1314-1316.
REFERENCES
4. Sykes EM: Colon perforation in EhlersDanlos syndrome. A m J Surg 1984;147:410-413.
1. Pope FM, Martin GR, Lichtenstein JR, et al:
17:8 August 1988
2. Prockop DJ, Kivirikko KI, Tunderman L, et al: The biosynthesis of collagen and its disorders. N Engl J Med 1979;301:77-85. 3. Silva R, Cogbill TH, Hansbrough JF, et ah Intestinal perforation and vascular rupture in E h l e r s - D a n l o s s y n d r o m e . Int Surg 1986;71: 48-50.
Annals of Emergency Medicine
5. G e r t s c h P, Loup PW, L o c h m a n A, et ah C h a n g i n g p a t t e r n s in the vascular form of Ehlers-Danlos syndrome. Arch Surg 1986;I21: 1061-1064. 6. Wesley JR, Mahout GH, Woolley MM: Multiple surgical problems in two patients with E h l e r s - D a n l o s s y n d r o m e . Surgery 1980;87: 319-324. 7. McFarland W, Fuller DE: Mortality in EhlersDanlos syndrome due to spontaneous rupture of large arteries. N Engl J Med 1964;271:1309-1310.
852/129