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Tracheo-arterial fistula in tracheostomy patients with Duchenne muscular dystrophy
Brain & Development 28 (2006) 223–227 www.elsevier.com/locate/braindev
Original article
Tracheo-arterial fistula in tracheostomy patients with Duchenne muscular dystrophy Toshio Saitoa,*, Noriyoshi Sawabatab, Tsuyoshi Matsumuraa, Sonoko Nozakia, Harutoshi Fujimuraa, Susumu Shinnoa a
Division of Neurology, National Hospital Organization Toneyama National Hospital, 5-1-1 Toneyama, Toyonaka, Osaka 560-8552, Japan b Division of Surgery, National Hospital Organization Toneyama National Hospital, 5-1-1 Toneyama, Toyonaka, Osaka 560-8552, Japan Received 22 June 2004; received in revised form 5 July 2005; accepted 2 August 2005
Abstract A tracheo-arterial fistula is a serious and life threatening potential complication of a tracheostomy. Since 1984, we experienced nine fatal cases of tracheo-arterial fistula among 60 Duchenne muscular dystrophy (DMD) patients who underwent a tracheostomy. Representative cases included a patient with lordosis (Case 8), in whom the fistula was located in the brachiocephalic artery close to the trachea, and another with severe scoliosis (Case 9), which caused the aorta to compress the trachea. Such anatomical changes can be the cause of a fistula between the trachea and brachiocephalic artery. The anatomical locations between the trachea and brachiocephalic artery are modified by thoracic deformities in DMD patients, and should be confirmed using computed tomography (CT) prior to a tracheostomy procedure. Further, during such a procedure, the tracheal stoma must be placed in a location clearly away from the arteries, and should be followed by regular postoperative examinations using CT and careful management to avoid a tracheo-arterial fistula. q 2005 Elsevier B.V. All rights reserved. Keywords: Duchenne muscular dystrophy (DMD); Tracheostomy; Brachiocephalic artery; Tracheo-arterial fistula
1. Introduction Presently, non-invasive ventilation (NIV) is primarily indicated for treatment of patients with Duchenne muscular dystrophy (DMD) in the early stage of chronic respiratory failure [1]. In cases where such therapy is not effective, a shift to tracheal intermittent ventilation, the primary respiratory management used for DMD patients prior to the introduction of NIV, should be considered. Although a tracheostomy is an important procedure for management of a respiratory-insufficient DMD patient [2], the procedure is often accompanied by complications such as infection, bleeding, and pain, as well as others, while a tracheo-arterial fistula is a serious and life threatening complication [3–11]. In the present study, we evaluated the incidence of tracheoarterial fistula in DMD patients who underwent a tracheostomy at our hospital. We also report two fatal * Corresponding author. Tel.: C81 6 6853 2001; fax: C81 6 6850 1750. E-mail address: [email protected] (T. Saito).
0387-7604/$ - see front matter q 2005 Elsevier B.V. All rights reserved. doi:10.1016/j.braindev.2005.08.002
cases of tracheo-arterial fistula in patients with DMD and discuss the etiology in those cases. 2. Subjects and methods Sixty DMD patients who received a tracheostomy at Toneyama National Hospital from 1984 to 2004 were studied. Ages at the time of the procedure ranged from 15 to 27 years old (average 20 years). Following the introduction of NIV at our hospital in 1986, a tracheostomy procedure was indicated for 26 of the 60 DMD patients for whom NIV was not effective. None of the patients were ambulatory, and all demonstrated lordosis or scoliosis on spine roentgenograph or chest computed tomography (CT) images [2,12, 13]. We evaluated the incidence of tracheal bleeding due to a tracheo-arterial fistula, as well as prognosis and outcome.
3. Results Tracheal bleeding due to a tracheo-arterial fistula occurred in nine patients, for an overall incidence of 15%,
224
T. Saito et al. / Brain & Development 28 (2006) 223–227
Table 1 Summary of tracheo-arterial fistula patients who underwent a tracheostomy Case
Age at tracheostomy (in years)
Year of procedure
Duration until death
1 2 3 4 5 6 7 8 9 Mean
18 22 23 15 17 16 17 27 20 19.4
1984 1984 1984 1987 1989 1989 1990 1999 2003
10 days 58 months 62 months 39 months 22 days 16 days 28 months 20 days 6 months 22 months
all of whom died. In the 31 cases of tracheostomy up to 1990, a tracheo-arterial fistula occurred in seven, thus the incidence for the period from 1984 to 1990 was 23%. The age and year at tracheostomy, along with duration until death (mean, 22 months), are summarized in Table 1. All patients, except for case 8, who was confirmed by autopsy results, were diagnosed by clinical signs and symptoms, such as active uncontrolled bleeding at the tracheostomy site and shock status, as a bronchoscopy was not impossible for confirmation in active bleeding status and autopsies were not performed [6]. Following, we present the details of two representative cases.
4. Case 8 A 27-year-old man, diagnosed previously with DMD based on clinical signs, symptoms, and gene analysis using a multiplex PCR assay, which showed the deletion of exon 49,
50 in the dystrophin gene [14], was admitted due to an upper respiratory infection. Respiratory failure had been managed well with NIV for 5 years. Chest X-ray results upon admission showed no new lesions and only a previous consolidation in the right upper lobe of the lung, as well as mild scoliosis (Fig. 1A) and lordosis [2,12]. Chest CT findings revealed the thorax to have a compressed deformity (Fig. 1B and C), however, no cardiac complications were apparent. Following admission, the upper respiratory infection was controlled well by antibiotics. On the seventh day of hospitalization, the patient experienced a sudden and massive hemorrhage from the organized lesion in the right upper lobe of the lung, after which he was intubated with a 7.5-mm endotracheal tube in order to keep the respiratory tract open. Bleeding was difficult to control and a tracheostomy was performed on the next day. A 7.5-mm pliable tube made of silicon with a cuff was inserted through the second and third tracheal rings, a conventional site, using a transverse incision, and the surgeon confirmed that the brachiocephalic artery was not close to the stoma. After completion of the tracheostomy, bleeding from the old lesion stopped and his respiratory condition was well controlled. On day 19 after the procedure, minimal bleeding from the tracheostomy site was noticed, and the next day a sudden and massive hemorrhage occurred from the stoma. The cuff was immediately inflated, however, the bleeding did not stop, therefore, the artery in the neck was compressed with an index finger and the hemorrhaging was transiently controlled [9,10]. For surgical exploration, the patient was brought to the operation room, however, the massive bleeding hindered a therapeutic approach. Endotracheal intubation was not
Fig. 1. Chest X-ray and computed tomography images from Case 8. (A) Chest X-ray image taken upon admission showing mild scoliosis. (B, C) Chest computed tomography images revealing a compressed thorax deformity. The brachiocephalic artery can be seen overriding the trachea in a higher position than normal (arrow). Consolidation in the right upper lobe from a previous procedure is also shown.
T. Saito et al. / Brain & Development 28 (2006) 223–227
225
Fig. 2. Post-mortem appearance of the tracheostomy site of Case 8. (A) The brachiocephalic artery can be seen running across the tracheostomy site (arrow). (B) Pressure necrosis caused by the tracheostomy tube was observed in the mucosa of the trachea. The dotted line indicates the brachiocephalic artery.
successful and the operation was abandoned. Despite all cardiopulmonary resuscitation attempts, the patient died. An autopsy revealed a defect measuring approximately 3!5 mm in size in the posterior wall of the brachiocephalic artery, just adjacent to a defect in the tracheal wall (Fig. 2A and B). Tissue necrosis around the stoma was also observed.
5. Case 9 A 20-year-old man with DMD, whose respiratory failure was uncontrolled by NIV, underwent a
tracheostomy to provide mechanical ventilation as therapy for chronic respiratory failure. A 7.5-mm pliable tube made of silicon with a cuff was inserted through the second and third tracheal rings, and the physician confirmed that the brachiocephalic artery was not close to the stoma. The patient had been bedridden because of muscle atrophy and weakness in the limbs and trunk, and, since losing ambulation at 8 years old, scoliosis had progressively advanced [2,12]. Chest X-ray and CT findings revealed the thorax to have a compressed deformity (Fig. 3). A diagnosis of DMD was made, based on clinical signs and symptoms, as well as muscle
Fig. 3. Chest X-ray and computed tomography images from Case 9. (A) Chest X-ray image showing severe scoliosis. (B, C) Chest computed tomography images revealing a compressed thorax deformity.
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biopsy findings using a Dystrophin-immunohistochemistry examination. Six months after the tracheostomy, the patient experienced a sudden and massive hemorrhage from the tracheostomy stoma, while prior to this, minimal bleeding was observed from the tracheostomy site. Neither an inflated cuff nor compression with an index finger to the tracheostomy site was effective, as the bleeding could not be stopped. Despite all cardiopulmonary resuscitation attempts, the patient died. An autopsy was not performed.
6. Discussion The most frequent cause of a tracheo-arterial fistula is reported to be the contribution of direct pressure from the elbow, cuff, and/or tip of an inserted tracheal tube against the brachiocephalic artery, particularly with a stoma set low [9,10]. The brachiocephalic artery normally covers the trachea at the ninth ring, with a range from the sixth to thirteenth rings [7,9], thus a tracheo-arterial fistula can usually be avoided by correct placement of the tracheostomy stoma through or above the second and third tracheal rings [8,9]. However, such a fistula occurred in the representative cases, in spite of confirmation of the absence of pulsation by the brachiocephalic artery at the operation site, and correct placement of the tracheostomy stoma through the second and third tracheal rings. In DMD patients, generalized muscle weakness and contracture of multiple joints force a compensated posture with lordosis [2,12]. Following the loss of ambulation, an advancement of spine deformity, known as scoliosis, occurs in some patients. Such deformity induces a compressed thorax, resulting in unusual anatomical locations of the trachea and brachiocephalic artery. At the present time, spinal fusion with internal fixation is not a conventional treatment option in Japan [13,15]. Among the present patients, Case 8 demonstrated lordosis, which was unremarkable on chest X-ray images, while CT images revealed a short antero-posterior distance of the thorax, as well as the brachiocephalic artery overriding the trachea in a higher position than normal. Because of the anatomical locations in this case, the location of the stoma, which was placed in a conventional manner, might have been near the brachiocephalic artery, though the surgeon confirmed the absence of pulsation by the brachiocephalic artery at the operation site. In addition, localized chronic stimulation by the elbow gradually enlarged the stoma, though the actual stoma site did not become infected. As a result, pressure and necrosis deterioration caused by the elbow might have contributed to creating the fistula between the trachea and brachiocephalic artery. Case 9 demonstrated severe scoliosis, which might have caused the tip of the cannula to press the wall of the trachea chronically, inducing necrosis at the site in
the wall of the brachiocephalic artery. Although there was no autopsy and we could not confirm the anatomical location between the tracheal stoma and artery, the ineffectiveness of an inflated cuff and failure of compression with an index finger to the tracheostomy site were likely due to the point at which the cannula and artery were attached. Approximately 10% of post-tracheostomy hemorrhages are due to a tracheo-arterial fistula, while the overall incidence of tracheo-arterial fistula ranges from 0 to 1%, with an average of 0.3% [9,10]. Our nine fatal cases among 60 tracheostomy patients over the past 20 years is a higher rate than usual, while the incidence of 23% from 1984 to 1990 is further elevated. The annual reports of the Japanese research grant for nervous and mental disorders on muscular dystrophy reported from 1991 to 1993 stated that incidence of tracheo-arterial fistula in muscular dystrophy patients seems to be high, though the overall incidence in Japan has not been estimated. Those reports also noted that tracheoarterial fistula in patients with muscular dystrophy may be associated with their spine deformity. All of our DMD patients had lordosis or scoliosis. Although we did not examine all to determine the anatomical locations of the trachea and brachiocephalic artery, it was considered that the abnormal position induced by spine deformity is one of the causes of the high incidence of tracheo-artery fistula in our series of cases. In addition, in the early years until 1990 we performed tracheostomies in our hospital, our awareness of tracheo-arterial fistula was not enough and evaluations prior to the tracheostomy procedure were incomplete. Although detailed information regarding the tracheostomy site in fatal cases between 1984 and 1990 was not preserved, we presume that an inappropriate position such as close to an artery was often chosen as the tracheostomy site. In addition, sharply bent and rigid tubes used during that period might have caused adverse effects [3,10]. In general, the best treatment for a tracheo-arterial fistula is prevention [8–10]. If a tracheo-arterial fistula occurs, successful management relies on early diagnosis and prompt management [4,5,9,11]. The patient will likely not survive without surgical intervention. Approximately 25% of the patients brought to the operation room survive [9,10]. A simple ligation or resection of the brachiocephalic artery is advocated because of the high rate of rebleeding after vascular reconstruction [5,8,9]. However, among member institutions of the National Hospital Organization in Japan with muscular dystrophy wards, those with emergency systems prepared for cases of tracheo-arterial fistula are limited. Actually there is no effective treatment strategy in place at the majority of those with muscular dystrophy wards in Japan. For treatment of respiratory failure in a patient with DMD, a tracheostomy is inevitable. Therefore, to prevent a tracheo-arterial fistula in those patients, appropriate placement of the stoma and careful respiratory management are extremely important. For appropriate placement of the stoma, an experienced surgeon should count the tracheal rings from the cricoid to
T. Saito et al. / Brain & Development 28 (2006) 223–227
place the stoma through the second and third tracheal rings with confirmation of the absence of pulsation by the brachiocephalic artery at the tracheostomy site. In addition, the surgeon must also confirm the anatomical locations of the trachea and brachiocephalic artery using CT imaging, as the tracheal stoma should be placed fully separate from the brachiocephalic artery in patients with DMD. For proper management, pliable tubes with a cuff pressure ordinarily below 20 mmHg are preferable [5,8,9], while ventilatorinduced tracheostomy movement should be minimized [5,8,9] and infection around the stoma controlled [8]. Even after the tracheostomy, spine deformity in DMD continues to slowly progress, thus regular confirmation of the position of the tube and brachiocephalic artery using CT imaging is needed. Seventy-two percent of tracheo-arterial fistula cases occur during the first 3 weeks after a tracheostomy procedure [5], while the earliest was reported to occur at 2 days and late occurrence can appear several months after the operation [9,10]. The present cases occurred between 10 days and 62 months following surgery, with a mean of 22 months. Therefore, long-term careful management must be continued. In conclusion, a tracheostomy for respiratory failure in patients with DMD should be performed after pre-operative evaluations including CT findings to confirm the anatomical locations of the trachea and brachiocephalic artery, and followed by post-operative careful management to avoid the causes of tracheo-arterial fistula.
Acknowledgements This study was supported by a Japanese Research Grant for Child Health and Development.
227
References [1] Rideau Y, Delaubier A, Guillou C, Renardel-Irani A. Treatment of respiratory insufficiency in Duchenne’s muscular dystrophy: nasal ventilation in the initial stages. Monaldi Arch Chest Dis 1995;50: 235–8. [2] Sussman M. Duchenne muscular dystrophy. J Am Acad Orthop Surg 2002;10:138–51. [3] Silen W, Spieker D. Fatal hemorrhage from the innominate artery after tracheostomy. Ann Surg 1965;162:1005–12. [4] Deslauriers J, Ginsberg RJ, Nelems JM, Pearson FG. Innominate artery rupture: a major complication of tracheal surgery. Ann Thorac Surg 1975;20:671–7. [5] Jones JW, Reynolds M, Hewitt RL, Drapanas T. Tracheoinnominate artery erosion: successful surgical management of a devastating complication. Ann Surg 1976;184:194–204. [6] Yang FY, Criado E, Schwartz JA, Keagy BA, Wilcox BR. Tracheainnomonate artery fistula: retrospective comparison of treatment methods. South Med J 1988;81:701–6. [7] Oshinsky AE, Rubin JS, Gwozdz E. The anatomical basis for posttracheostomy innominate artery rupture. Laryngoscope 1988;98: 1061–4. [8] Gelman JJ, Aro M, Weiss SM. Tracheo-innominate artery fistula. J Am Coll Surg 1994;179:626–34. [9] Wright CD. Management of tracheoinnominate artery fistula. Trachea 1996;6:865–73. [10] Allan JS, Wright CD. Tracheoinnominate fistula: diagnosis and management. Chest Surg Clin N Am 2003;13:331–41. [11] Gasparri MG, Nicolosi AC, Almassi GH. A novel approach to the management of tracheoinnominate artery fistula. Ann Thorac Surg 2004;77:1424–6. [12] Dobowitz V. Muscle disorders in childhood. 2nd ed. London: W.B. Saunders; 1995. [13] Duport G, Gayet E, Pries P, Thirault C, Renardel-Irani A, Fons N, et al. Spinal deformities and wheelchair seating in Duchenne muscular dystrophy: 20 years of research and clinical experience. Semin Neurol 1995;15:29–37. [14] Hoffman EP, Wang J. Duchenne-Becker muscular dystrophy and the nondystrophic myotonias: paradigms for loss of function and change of function of gene products. Arch Neurol 1993;50:1227–37. [15] Matsumura T, Kang J, Nozaki S, Takahashi PM. The effects of spinal fusion on respiratory function and quality of life in Duchenne muscular dystrophy. Clin Neurol 1997;37:87–92.
Original article
Tracheo-arterial fistula in tracheostomy patients with Duchenne muscular dystrophy Toshio Saitoa,*, Noriyoshi Sawabatab, Tsuyoshi Matsumuraa, Sonoko Nozakia, Harutoshi Fujimuraa, Susumu Shinnoa a
Division of Neurology, National Hospital Organization Toneyama National Hospital, 5-1-1 Toneyama, Toyonaka, Osaka 560-8552, Japan b Division of Surgery, National Hospital Organization Toneyama National Hospital, 5-1-1 Toneyama, Toyonaka, Osaka 560-8552, Japan Received 22 June 2004; received in revised form 5 July 2005; accepted 2 August 2005
Abstract A tracheo-arterial fistula is a serious and life threatening potential complication of a tracheostomy. Since 1984, we experienced nine fatal cases of tracheo-arterial fistula among 60 Duchenne muscular dystrophy (DMD) patients who underwent a tracheostomy. Representative cases included a patient with lordosis (Case 8), in whom the fistula was located in the brachiocephalic artery close to the trachea, and another with severe scoliosis (Case 9), which caused the aorta to compress the trachea. Such anatomical changes can be the cause of a fistula between the trachea and brachiocephalic artery. The anatomical locations between the trachea and brachiocephalic artery are modified by thoracic deformities in DMD patients, and should be confirmed using computed tomography (CT) prior to a tracheostomy procedure. Further, during such a procedure, the tracheal stoma must be placed in a location clearly away from the arteries, and should be followed by regular postoperative examinations using CT and careful management to avoid a tracheo-arterial fistula. q 2005 Elsevier B.V. All rights reserved. Keywords: Duchenne muscular dystrophy (DMD); Tracheostomy; Brachiocephalic artery; Tracheo-arterial fistula
1. Introduction Presently, non-invasive ventilation (NIV) is primarily indicated for treatment of patients with Duchenne muscular dystrophy (DMD) in the early stage of chronic respiratory failure [1]. In cases where such therapy is not effective, a shift to tracheal intermittent ventilation, the primary respiratory management used for DMD patients prior to the introduction of NIV, should be considered. Although a tracheostomy is an important procedure for management of a respiratory-insufficient DMD patient [2], the procedure is often accompanied by complications such as infection, bleeding, and pain, as well as others, while a tracheo-arterial fistula is a serious and life threatening complication [3–11]. In the present study, we evaluated the incidence of tracheoarterial fistula in DMD patients who underwent a tracheostomy at our hospital. We also report two fatal * Corresponding author. Tel.: C81 6 6853 2001; fax: C81 6 6850 1750. E-mail address: [email protected] (T. Saito).
0387-7604/$ - see front matter q 2005 Elsevier B.V. All rights reserved. doi:10.1016/j.braindev.2005.08.002
cases of tracheo-arterial fistula in patients with DMD and discuss the etiology in those cases. 2. Subjects and methods Sixty DMD patients who received a tracheostomy at Toneyama National Hospital from 1984 to 2004 were studied. Ages at the time of the procedure ranged from 15 to 27 years old (average 20 years). Following the introduction of NIV at our hospital in 1986, a tracheostomy procedure was indicated for 26 of the 60 DMD patients for whom NIV was not effective. None of the patients were ambulatory, and all demonstrated lordosis or scoliosis on spine roentgenograph or chest computed tomography (CT) images [2,12, 13]. We evaluated the incidence of tracheal bleeding due to a tracheo-arterial fistula, as well as prognosis and outcome.
3. Results Tracheal bleeding due to a tracheo-arterial fistula occurred in nine patients, for an overall incidence of 15%,
224
T. Saito et al. / Brain & Development 28 (2006) 223–227
Table 1 Summary of tracheo-arterial fistula patients who underwent a tracheostomy Case
Age at tracheostomy (in years)
Year of procedure
Duration until death
1 2 3 4 5 6 7 8 9 Mean
18 22 23 15 17 16 17 27 20 19.4
1984 1984 1984 1987 1989 1989 1990 1999 2003
10 days 58 months 62 months 39 months 22 days 16 days 28 months 20 days 6 months 22 months
all of whom died. In the 31 cases of tracheostomy up to 1990, a tracheo-arterial fistula occurred in seven, thus the incidence for the period from 1984 to 1990 was 23%. The age and year at tracheostomy, along with duration until death (mean, 22 months), are summarized in Table 1. All patients, except for case 8, who was confirmed by autopsy results, were diagnosed by clinical signs and symptoms, such as active uncontrolled bleeding at the tracheostomy site and shock status, as a bronchoscopy was not impossible for confirmation in active bleeding status and autopsies were not performed [6]. Following, we present the details of two representative cases.
4. Case 8 A 27-year-old man, diagnosed previously with DMD based on clinical signs, symptoms, and gene analysis using a multiplex PCR assay, which showed the deletion of exon 49,
50 in the dystrophin gene [14], was admitted due to an upper respiratory infection. Respiratory failure had been managed well with NIV for 5 years. Chest X-ray results upon admission showed no new lesions and only a previous consolidation in the right upper lobe of the lung, as well as mild scoliosis (Fig. 1A) and lordosis [2,12]. Chest CT findings revealed the thorax to have a compressed deformity (Fig. 1B and C), however, no cardiac complications were apparent. Following admission, the upper respiratory infection was controlled well by antibiotics. On the seventh day of hospitalization, the patient experienced a sudden and massive hemorrhage from the organized lesion in the right upper lobe of the lung, after which he was intubated with a 7.5-mm endotracheal tube in order to keep the respiratory tract open. Bleeding was difficult to control and a tracheostomy was performed on the next day. A 7.5-mm pliable tube made of silicon with a cuff was inserted through the second and third tracheal rings, a conventional site, using a transverse incision, and the surgeon confirmed that the brachiocephalic artery was not close to the stoma. After completion of the tracheostomy, bleeding from the old lesion stopped and his respiratory condition was well controlled. On day 19 after the procedure, minimal bleeding from the tracheostomy site was noticed, and the next day a sudden and massive hemorrhage occurred from the stoma. The cuff was immediately inflated, however, the bleeding did not stop, therefore, the artery in the neck was compressed with an index finger and the hemorrhaging was transiently controlled [9,10]. For surgical exploration, the patient was brought to the operation room, however, the massive bleeding hindered a therapeutic approach. Endotracheal intubation was not
Fig. 1. Chest X-ray and computed tomography images from Case 8. (A) Chest X-ray image taken upon admission showing mild scoliosis. (B, C) Chest computed tomography images revealing a compressed thorax deformity. The brachiocephalic artery can be seen overriding the trachea in a higher position than normal (arrow). Consolidation in the right upper lobe from a previous procedure is also shown.
T. Saito et al. / Brain & Development 28 (2006) 223–227
225
Fig. 2. Post-mortem appearance of the tracheostomy site of Case 8. (A) The brachiocephalic artery can be seen running across the tracheostomy site (arrow). (B) Pressure necrosis caused by the tracheostomy tube was observed in the mucosa of the trachea. The dotted line indicates the brachiocephalic artery.
successful and the operation was abandoned. Despite all cardiopulmonary resuscitation attempts, the patient died. An autopsy revealed a defect measuring approximately 3!5 mm in size in the posterior wall of the brachiocephalic artery, just adjacent to a defect in the tracheal wall (Fig. 2A and B). Tissue necrosis around the stoma was also observed.
5. Case 9 A 20-year-old man with DMD, whose respiratory failure was uncontrolled by NIV, underwent a
tracheostomy to provide mechanical ventilation as therapy for chronic respiratory failure. A 7.5-mm pliable tube made of silicon with a cuff was inserted through the second and third tracheal rings, and the physician confirmed that the brachiocephalic artery was not close to the stoma. The patient had been bedridden because of muscle atrophy and weakness in the limbs and trunk, and, since losing ambulation at 8 years old, scoliosis had progressively advanced [2,12]. Chest X-ray and CT findings revealed the thorax to have a compressed deformity (Fig. 3). A diagnosis of DMD was made, based on clinical signs and symptoms, as well as muscle
Fig. 3. Chest X-ray and computed tomography images from Case 9. (A) Chest X-ray image showing severe scoliosis. (B, C) Chest computed tomography images revealing a compressed thorax deformity.
226
T. Saito et al. / Brain & Development 28 (2006) 223–227
biopsy findings using a Dystrophin-immunohistochemistry examination. Six months after the tracheostomy, the patient experienced a sudden and massive hemorrhage from the tracheostomy stoma, while prior to this, minimal bleeding was observed from the tracheostomy site. Neither an inflated cuff nor compression with an index finger to the tracheostomy site was effective, as the bleeding could not be stopped. Despite all cardiopulmonary resuscitation attempts, the patient died. An autopsy was not performed.
6. Discussion The most frequent cause of a tracheo-arterial fistula is reported to be the contribution of direct pressure from the elbow, cuff, and/or tip of an inserted tracheal tube against the brachiocephalic artery, particularly with a stoma set low [9,10]. The brachiocephalic artery normally covers the trachea at the ninth ring, with a range from the sixth to thirteenth rings [7,9], thus a tracheo-arterial fistula can usually be avoided by correct placement of the tracheostomy stoma through or above the second and third tracheal rings [8,9]. However, such a fistula occurred in the representative cases, in spite of confirmation of the absence of pulsation by the brachiocephalic artery at the operation site, and correct placement of the tracheostomy stoma through the second and third tracheal rings. In DMD patients, generalized muscle weakness and contracture of multiple joints force a compensated posture with lordosis [2,12]. Following the loss of ambulation, an advancement of spine deformity, known as scoliosis, occurs in some patients. Such deformity induces a compressed thorax, resulting in unusual anatomical locations of the trachea and brachiocephalic artery. At the present time, spinal fusion with internal fixation is not a conventional treatment option in Japan [13,15]. Among the present patients, Case 8 demonstrated lordosis, which was unremarkable on chest X-ray images, while CT images revealed a short antero-posterior distance of the thorax, as well as the brachiocephalic artery overriding the trachea in a higher position than normal. Because of the anatomical locations in this case, the location of the stoma, which was placed in a conventional manner, might have been near the brachiocephalic artery, though the surgeon confirmed the absence of pulsation by the brachiocephalic artery at the operation site. In addition, localized chronic stimulation by the elbow gradually enlarged the stoma, though the actual stoma site did not become infected. As a result, pressure and necrosis deterioration caused by the elbow might have contributed to creating the fistula between the trachea and brachiocephalic artery. Case 9 demonstrated severe scoliosis, which might have caused the tip of the cannula to press the wall of the trachea chronically, inducing necrosis at the site in
the wall of the brachiocephalic artery. Although there was no autopsy and we could not confirm the anatomical location between the tracheal stoma and artery, the ineffectiveness of an inflated cuff and failure of compression with an index finger to the tracheostomy site were likely due to the point at which the cannula and artery were attached. Approximately 10% of post-tracheostomy hemorrhages are due to a tracheo-arterial fistula, while the overall incidence of tracheo-arterial fistula ranges from 0 to 1%, with an average of 0.3% [9,10]. Our nine fatal cases among 60 tracheostomy patients over the past 20 years is a higher rate than usual, while the incidence of 23% from 1984 to 1990 is further elevated. The annual reports of the Japanese research grant for nervous and mental disorders on muscular dystrophy reported from 1991 to 1993 stated that incidence of tracheo-arterial fistula in muscular dystrophy patients seems to be high, though the overall incidence in Japan has not been estimated. Those reports also noted that tracheoarterial fistula in patients with muscular dystrophy may be associated with their spine deformity. All of our DMD patients had lordosis or scoliosis. Although we did not examine all to determine the anatomical locations of the trachea and brachiocephalic artery, it was considered that the abnormal position induced by spine deformity is one of the causes of the high incidence of tracheo-artery fistula in our series of cases. In addition, in the early years until 1990 we performed tracheostomies in our hospital, our awareness of tracheo-arterial fistula was not enough and evaluations prior to the tracheostomy procedure were incomplete. Although detailed information regarding the tracheostomy site in fatal cases between 1984 and 1990 was not preserved, we presume that an inappropriate position such as close to an artery was often chosen as the tracheostomy site. In addition, sharply bent and rigid tubes used during that period might have caused adverse effects [3,10]. In general, the best treatment for a tracheo-arterial fistula is prevention [8–10]. If a tracheo-arterial fistula occurs, successful management relies on early diagnosis and prompt management [4,5,9,11]. The patient will likely not survive without surgical intervention. Approximately 25% of the patients brought to the operation room survive [9,10]. A simple ligation or resection of the brachiocephalic artery is advocated because of the high rate of rebleeding after vascular reconstruction [5,8,9]. However, among member institutions of the National Hospital Organization in Japan with muscular dystrophy wards, those with emergency systems prepared for cases of tracheo-arterial fistula are limited. Actually there is no effective treatment strategy in place at the majority of those with muscular dystrophy wards in Japan. For treatment of respiratory failure in a patient with DMD, a tracheostomy is inevitable. Therefore, to prevent a tracheo-arterial fistula in those patients, appropriate placement of the stoma and careful respiratory management are extremely important. For appropriate placement of the stoma, an experienced surgeon should count the tracheal rings from the cricoid to
T. Saito et al. / Brain & Development 28 (2006) 223–227
place the stoma through the second and third tracheal rings with confirmation of the absence of pulsation by the brachiocephalic artery at the tracheostomy site. In addition, the surgeon must also confirm the anatomical locations of the trachea and brachiocephalic artery using CT imaging, as the tracheal stoma should be placed fully separate from the brachiocephalic artery in patients with DMD. For proper management, pliable tubes with a cuff pressure ordinarily below 20 mmHg are preferable [5,8,9], while ventilatorinduced tracheostomy movement should be minimized [5,8,9] and infection around the stoma controlled [8]. Even after the tracheostomy, spine deformity in DMD continues to slowly progress, thus regular confirmation of the position of the tube and brachiocephalic artery using CT imaging is needed. Seventy-two percent of tracheo-arterial fistula cases occur during the first 3 weeks after a tracheostomy procedure [5], while the earliest was reported to occur at 2 days and late occurrence can appear several months after the operation [9,10]. The present cases occurred between 10 days and 62 months following surgery, with a mean of 22 months. Therefore, long-term careful management must be continued. In conclusion, a tracheostomy for respiratory failure in patients with DMD should be performed after pre-operative evaluations including CT findings to confirm the anatomical locations of the trachea and brachiocephalic artery, and followed by post-operative careful management to avoid the causes of tracheo-arterial fistula.
Acknowledgements This study was supported by a Japanese Research Grant for Child Health and Development.
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