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Posterior spinal fusion for scoliosis after Fontan procedure: A case series
Journal of Orthopaedic Science xxx (2017) 1e5
Contents lists available at ScienceDirect
Journal of Orthopaedic Science journal homepage: http://www.elsevier.com/locate/jos
Original Article
Posterior spinal fusion for scoliosis after Fontan procedure: A case series Masashi Uehara a, 1, Jun Takahashi a, *, Yohei Akazawa b, 2, Shugo Kuraishi a, 1, Shota Ikegami a, 1, Toshimasa Futatsugi a, 1, Hiroki Oba a, 1, Hiroyuki Kato a, 1 a b
Department of Orthopaedic Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan Department of Pediatrics, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan
a r t i c l e i n f o
a b s t r a c t
Article history: Received 11 August 2017 Received in revised form 17 October 2017 Accepted 2 November 2017 Available online xxx
Background: As the surgical treatment of scoliosis after a Fontan procedure is very challenging due to the risk of various perioperative complications, case reports are scarce. We herein describe three patients who were successfully treated for scoliosis following a Fontan procedure and discuss their clinical and radiological outcomes. Methods: We retrospectively reviewed three cases of scoliosis treated by posterior spinal fusion after a Fontan procedure. Results: Mean preoperative major curve Cobb angle was 83.7, mean surgical time was 233.0 min, and mean blood loss was 1167 g. The mean correction rate of the major curve was 48.0%. Surgical outcome as evaluated by Scoliosis Research Society-22 patient questionnaires revealed acceptable results without any severe complications. Conclusions: Corrective surgery for scoliosis after a Fontan procedure becomes a stronger option if cardiac insufficiency is prevented during the perioperative period and a conservative plan is carried out with minimal invasiveness and operation time. © 2017 The Japanese Orthopaedic Association. Published by Elsevier B.V. All rights reserved.
1. Introduction The Fontan procedure is a functional repair technique anastomosing the superior vena cava and inferior vena cava to the pulmonary artery to treat congenital heart disease with cyanosis. Although several studies have linked scoliosis with congenital heart disease, the aetiology of this relationship remains unknown [1e3]. Khadhim et al. [4] identified a high prevalence of scoliosis in patients with Fontan circulation. The survival rate of patients with complex congenital heart diseases is improving, thus creating a subset of individuals with a complex physiology who require surgical treatment for scoliosis due to worsening curvature, back pain, secondary impairment of cardio-respiratory function, and the lack of conservative treatment options [5]. To date, there exist few case reports of surgical treatment for scoliosis after a Fontan procedure [5e7]. We herein describe the
* Corresponding author. Fax: þ81 263 35 8844. E-mail address: [email protected] (J. Takahashi). 1 Fax: þ81 263 35 8844. 2 Fax: þ81 263 37 3089.
clinical and radiological features of three patients with Fontan circulation who were surgically treated for severe scoliosis. 1.1. Case presentations This study was approved by the ethics committee of our hospital (No. 3765). This was a retrospective, consecutive case series study that included three patients aged 11, 12, and 14 years, respectively, with scoliosis and Fontan circulation who were treated by posterior spinal fusion using pedicle screws in tandem with careful monitoring of circulatory dynamics in the perioperative period. All cases received surgery for scoliosis subsequent to a Fontan procedure at our hospital. Written informed consent was obtained from the patients' parents. Perioperative findings are summarized in Table 1. Mean preoperative main curve Cobb angle was 83.7 ± 35.5 , mean surgical time was 233.0 ± 91.1 min, and mean blood loss was 1167 ± 764 g. The mean correction rate of the main curve was 48.0 ± 15.3%. Surgical outcome as evaluated by the Scoliosis Research Society-22 patient questionnaire (SRS-22) revealed favourable results without severe complications (Fig. 1).
https://doi.org/10.1016/j.jos.2017.11.004 0949-2658/© 2017 The Japanese Orthopaedic Association. Published by Elsevier B.V. All rights reserved.
Please cite this article in press as: Uehara M, et al., Posterior spinal fusion for scoliosis after Fontan procedure: A case series, Journal of Orthopaedic Science (2017), https://doi.org/10.1016/j.jos.2017.11.004
2
M. Uehara et al. / Journal of Orthopaedic Science xxx (2017) 1e5
Table 1 Perioperative patient data. Blood loss Fusion Age Gender Risser Follow-up Surgical volume (g) area time (years) sign period (months) (minutes) Case 1 Case 2 Case 3 Mean
11 12 14 12.3
F M F
2 0 4
36 16 9 20.3
167 337 195 233.0
1000 2000 500 1167
Post-op major Correction Pre-op T5-T12 Post-op T5-T12 Ponte Pre-op major rate (%) kyphotic angle kyphotic angle curve Cobb osteotomy curve Cobb (degrees) (degrees) angle (degrees) angle (degrees)
T5-L1 e T5-L4 e T5-T11 þ
77 (MT) 122 (MT) 52 (MT) 83.7
34 85 17 45.3
55.8 30.3 67.3 51.2
20 40 9 23
20 21 35 25.3
MT: main thoracic.
Case 1: An 11-year-old girl had received a Fontan procedure at the age of 3 years. We performed posterior spinal fusion from T5 to L1 for her preoperative main thoracic (MT) curve Cobb angle of 77. Surgical time was 167 min and blood loss volume was 1000 g. She suffered from pleural effusion immediately after surgery that improved a day later with conservative treatment. Postoperative Cobb angle of the MT curve improved to 34 . Thoracic kyphotic angle remained at 20 (Fig. 2). Forced vital capacity improved from 2.11 L before surgery to 2.51 L afterwards. Preoperative SRS-22 domain scores were 3.8, 4.4, 2.8, 4.2, and 3.8 for function, pain, self-image, mental health, and subtotal, respectively, which were improved at 50 months at 4.8, 5.0, 4.4, 5.0, and 4.8, respectively. Case 2: A 12-year-old boy had received a Fontan procedure at the age of 4 years. We performed posterior spinal fusion from T5 to L4 for his preoperative MT curve Cobb angle of 122 . Surgical time was 337 min and blood loss volume was 2000 g. He suffered from pleural effusion immediately after surgery that improved a week later with conservative treatment. Postoperative Cobb angle of the MT curve improved to 85 . Thoracic kyphotic angle improved from 40 before surgery to 21 afterwards (Fig. 3). Forced vital capacity improved from 0.36 L before surgery to 0.56 L afterwards. Preoperative SRS-22 domain scores were 4.4, 5.0, 2.4, 5.0, and 4.2 for function, pain, self-image, mental health, and subtotal, respectively,
Subtotal
Mental health
2. Discussion In the present case series of three patients with Fontan circulation receiving posterior spinal fusion for severe scoliosis, pedicle screw fixation improved both radiological and clinical parameters without severe perioperative complications, although two patients experienced pleural effusion that improved with conservative treatment.
Case 1
Case 2
Function 5 4 3 2 1 0
Function 5 4 3 2 1 0
Mental health
Subtotal
which were marginally improved at 16 months at 4.2, 5.0, 3.0, 4.8, and 4.25, respectively. Case 3: A 14-year-old girl had received a Fontan procedure at the age of 2 years. We performed posterior spinal fusion from T5 to T11 for her preoperative MT curve Cobb angle of 52 . Ponte osteotomy was added since this case had a rigid curve and thoracic hypokyphosis and there was little intraoperative bleeding. Surgical time was 195 min and blood loss volume was 500 g. There were no perioperative complications. Postoperative Cobb angle of the MT curve improved to 22 . Thoracic kyphotic angle (T5-T12) improved from 9 before surgery to 35 afterwards (Fig. 4). Preoperative SRS22 domain scores were 4.2, 4.6, 2.6, 4.6, and 4.0 for function, pain, self-image, mental health, and subtotal, respectively, which were improved at 1 year at 4.6, 5.0, 4.2, 4.6, and 4.6, respectively.
Pain
Self-image
Subtotal
Mental health
Mean
Function 5 4 3 2 1 0
Function 5 4 3 2 1 0
Self-image
Subtotal
Mental health
Final follow-up
Pain
Self-image
Case 3
Pain
Preoperative
Pain
Self-image
Fig. 1. Surgical outcome as evaluated by the Scoliosis Research Society-22 patient questionnaire.
Please cite this article in press as: Uehara M, et al., Posterior spinal fusion for scoliosis after Fontan procedure: A case series, Journal of Orthopaedic Science (2017), https://doi.org/10.1016/j.jos.2017.11.004
M. Uehara et al. / Journal of Orthopaedic Science xxx (2017) 1e5
3
Fig. 2. Case 1: an 11-year-old girl. (a) Preoperative Cobb angle of the MT curve was 77. (b) We performed posterior spinal fusion from T5 to L1. Postoperative Cobb angle of the MT curve improved to 34 .
Fig. 3. Case 2: a 12-year-old boy. (a) Preoperative Cobb angle of the MT curve was 122 . (b) We performed posterior spinal fusion from T5 to L4. Postoperative Cobb angle of the MT curve improved to 85 .
A relationship between spinal and major cardiac abnormalities is well known [8], with the frequency of scoliosis in patients with congenital heart disease ranging from 11% to 34% [9]. It has also been suggested that cyanosis associated with congenital heart anomalies is a risk factor for the development of scoliosis [10,11]. Owing to improvements in the survival rate of patients with complex congenital heart disease, a growing subset of individuals with Fontan circulation who require surgical treatment for severe scoliosis has emerged [5]. Herrera et al. reported that sagittal alignment in scoliosis patients after sternotomy for congenital heart disease tended towards kyphosis [1]. In our series, however, the thoracic kyphotic angles of all patients were almost normal. The management of surgical scoliosis correction after a Fontan procedure is very difficult due to the risk of various perioperative complications. Hedequist et al. observed that six of seven patients who received posterior correction surgery for scoliosis after a
Fontan procedure experienced postoperative pleural effusion [6], rez-Caballero et al. reported that one of eight patients with while Pe Fontan circulation died after scoliosis correction surgery due to hypovolemic shock [7]. In the present series, we encountered no severe complications in our careful monitoring of circulatory dynamics over the perioperative period. Two of three patients suffered from pleural effusion that improved with conservative treatment. Indeed, special considerations are necessary in posterior spinal fusion for scoliosis after a Fontan procedure to avoid heart failure. Hedequist et al. [6] postulated that a short operation time without prolonged attempts at instrumentation or correction in addition to a minor loss of blood led to better clinical outcomes. In our cohort, the mean surgical time was 233.0 min and mean blood loss was 1167 g, which were similar to those in previous study of adolescent idiopathic scoliosis (AIS; mean surgical time: 242 min,
Please cite this article in press as: Uehara M, et al., Posterior spinal fusion for scoliosis after Fontan procedure: A case series, Journal of Orthopaedic Science (2017), https://doi.org/10.1016/j.jos.2017.11.004
4
M. Uehara et al. / Journal of Orthopaedic Science xxx (2017) 1e5
Fig. 4. Case 3: a 14-year-old girl. (a) Preoperative Cobb angle of the MT curve was 52 . (b) We performed posterior spinal fusion from T5 to T11. Postoperative Cobb angle of the MT curve improved to 22 .
mean blood loss volume: 1060 g) [12]. Regarding intraoperative tissue bleeding, haemorrhage was more than usual for case 2 but comparable to that in normal scoliosis for the other two cases. Ponte osteotomy was generally avoided to minimize blood loss. However, case 3 had a rigid curve and thoracic hypokyphosis, for which Ponte osteotomy produced good thoracic kyphosis with little intraoperative bleeding through the use of Floseal® (Baxter, Deerfield, IL, USA), a haemostatic agent made of bovine-derived gelatin matrix and human-derived thrombin. Although the correction rate in this case series was lower than in previous AIS series (48.0% vs. 62.4% [12]), surgical outcome as evaluated by SRS-22 showed acceptable results without severe complications. Thus, conservative correction with minimal invasiveness is advised for patients having received a Fontan procedure. Chan et al. reported a case of severe kyphoscoliosis after Fontan surgery in which preoperative halo traction provided a correction rate of 64% with an operation time of 150 min and blood loss of 1050 mls and concluded that traction was useful for decreasing blood loss volume and operation time [13]. For our case 2 with severe scoliosis of more than 100 , bleeding and surgery time were roughly double those of Chan's case. Although we could safely correct the deformity without preoperative halo traction, better correction might have been possible with this technique. The key features of Fontan circulation are a high central venous pressure (CVP) required to maintain pulmonary circulation due to the absence of the right ventricle and a low cardiac output, even under normal conditions. Blood loss exacerbates the diminished cardiac function to increase the risk of circulatory failure, and so it is essential to limit the loss of blood with transfusions and catecholamines under careful monitoring. The high venous pressure necessary to drive the Fontan circulation, particularly whilst in the prone position, may result in significant blood loss [6,14]. It is therefore desirable to minimize operation time. Furthermore, since high positive end expiratory pressure from artificial ventilation is very disadvantageous for pulmonary circulation in patients with Fontan circulation, prompt extubation is also important after surgery. In our cohort, adjustment of transfusions and catecholamines based on circulation monitoring prevented postoperative hypotension. Thus, the main success factors appeared to be short
operation time, minimized blood loss, conservative planning of correction rate, and careful circulation monitoring and management. Specifically to shorten operative time, we avoided Ponte osteotomy when possible by using a rigid rod on the concave side and shortened the fusion area as much as possible. To reduce blood loss volume, we avoided Ponte osteotomy and actively used haemostatic agents, such as Floseal®. The precise cause of pleural effusion in patients after a Fontan procedure has not been identified. However, many potential factors, such as increased pulmonary artery pressure, reduced ventricular function, ventricular type, prolonged mechanical ventilation, moderate-to-severe systemic atrioventricular valve regurgitation, and longer cardiopulmonary bypass, may be involved [15]. In our series, two of three patients (case 1 and 2) exhibited pleural effusion that was successfully managed. Accordingly, perioperative precautions for patients after a Fontan procedure include: (1) proper monitoring of CVP, (2) extubation as soon as possible, (3) the use of catecholamines to enhance vasodilation and cardiac contractility, and (4) performing intensive intraoperative monitoring and moisture management in patients at high risk for postoperative pleural effusion, such as those with high CVP, poor cardiac function, severe atrioventricular valve regurgitation, and the necessity of large amounts of diuretics after Fontan procedures. With regard to surgical outcome, all SRS-22 domain scores were markedly improved in two of three patients, with the remaining patient having a preoperative curve of greater than 120 experiencing only modest gains. Nonetheless, surgery is advised in such cases before the curve becomes too large. The surgical procedure in case 2 was considered effective because forced vital capacity was improved to prevent possible future respiratory failure. Continued observation is necessary due to the short follow-up periods in two patients. It is important to understand the distinction between ordinal AIS and of post-Fontan procedures. Ordinal AIS normally has few perioperative complications, and so even if time or blood loss volume becomes elevated, optimal correction can still be pursued. On the other hand, patients with a Fontan procedure have poor cardiac function. To prevent serious complications, only the minimal
Please cite this article in press as: Uehara M, et al., Posterior spinal fusion for scoliosis after Fontan procedure: A case series, Journal of Orthopaedic Science (2017), https://doi.org/10.1016/j.jos.2017.11.004
M. Uehara et al. / Journal of Orthopaedic Science xxx (2017) 1e5
necessary correction is advised to minimize bleeding volume, and patients should be extubated as soon as possible after surgery. 3. Conclusion Based on the present case series, it is important to prevent cardiac insufficiency during the perioperative period, make a conservative correction plan, and minimize invasiveness in surgery for scoliosis following a Fontan procedure. Conflict of interest The authors declare that they have no conflict of interest. References [1] Herrera-Soto JA, Vander Have KL, Barry-Lane P, Myers JL. Retrospective study on development of spinal deformity following sternotomy for congenital heart disease. Spine (Phila Pa 1976) 2007 Aug 15;32(18):1998e2004. [2] Ruiz-Iban MA, Burgos J, Aguado HJ, Diaz-Heredia J, Roger I, Muriel A, Sanchez PA. Scoliosis after median sternotomy in children with congenital heart disease. Spine (Phila Pa 1976) 2005 Apr 15;30(8):E214e8. [3] Herrera-Soto JA, Vander Have KL, Barry-Lane P, Woo A. Spinal deformity after combined thoracotomy and sternotomy for congenital heart disease. J Pediatr Orthop 2006 MareApr;26(2):211e5. [4] Kadhim M, Pizarro C, Holmes Jr L, Rogers KJ, Kallur A, Mackenzie WG. Prevalence of scoliosis in patients with Fontan circulation. Arch Dis Child 2013 Mar;98(3):170e5.
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[5] Leichtle CI, Kumpf M, Gass M, Schmidt E, Niemeyer T. Surgical correction of scoliosis in children with congenital heart failure (Fontan circulation): case report and literature review. Eur Spine J 2008 Sep;17(Suppl. 2):S312e7. [6] Hedequist DJ, Emans JB, Hall JE. Operative treatment of scoliosis in patients with a Fontan circulation. Spine 2006 Jan 15;31(2):202e5. rez-Caballero Macarro n C, Sobrino Ruiz E, Burgos Flores J, Va zquez [7] Pe rez A, Alvarez Rojas E, Sa nchez Ruas J. Spinal surgery in the Martínez J, Coca Pe univentricular hearteis it viable? Cardiol Young 2014 Feb;24(1):73e8. [8] Ogilvie JW. Congenital heart disease and scoliosis. In: Lonstein JE, Winter RB, Bradford DS, Ogilvie JW, editors. Moe's textbook of scoliosis and other spinal deformities. Philadelphia, PA: W.B. Saunders Co; 1995. p. 564e5. [9] Bozcali E, Ucpunar H, Sevencan A, Balioglu MB, Albayrak A, Polat V. A retrospective study of congenital cardiac abnormality associated with scoliosis. Asian Spine J 2016 Apr;10(2):226e30. [10] Kawakami N, Mimatsu K, Deguchi M, Kato F, Maki S. Scoliosis and congenital heart disease. Spine (Phila Pa 1976) 1995 Jun 1;20(11):1252e5, discussion 1256. [11] Roth A, Rosenthal A, Hall JE, Mizel M. Scoliosis and congenital heart disease. Clin Orthop Relat Res 1973 Jun;93:95e102. [12] Uehara M, Takahashi J, Kuraishi S, Shimizu M, Ikegami S, Futatsugi T, Oba H, Kato H. Computer-assisted skip pedicle screw fixation for adolescent idiopathic scoliosis. J Orthop Sci 2017 Mar;22(2):218e23. [13] Chan CY, Lim CY, Shahnaz Hasan M, Kwan MK. The use of pre-operative halo traction to minimize risk for correction of severe scoliosis in a patient with Fontan circulation: a case report and review of literature. Eur Spine J 2016 May;25(Suppl. 1):245e50. [14] Rafique MB, Stuth EA, Tassone JC. Increased blood loss during posterior spinal fusion for idiopathic scoliosis in an adolescent with Fontan physiology. Pediatr Anaesth 2006 Feb;16(2):206e12. [15] Pike NA, Okuhara CA, Toyama J, Gross BP, Wells WJ, Starnes VA. Reduced pleural drainage, length of stay, and readmissions using a modified Fontan management protocol. J Thorac Cardiovasc Surg 2015 Sep;150(3): 481e7.
Please cite this article in press as: Uehara M, et al., Posterior spinal fusion for scoliosis after Fontan procedure: A case series, Journal of Orthopaedic Science (2017), https://doi.org/10.1016/j.jos.2017.11.004
Contents lists available at ScienceDirect
Journal of Orthopaedic Science journal homepage: http://www.elsevier.com/locate/jos
Original Article
Posterior spinal fusion for scoliosis after Fontan procedure: A case series Masashi Uehara a, 1, Jun Takahashi a, *, Yohei Akazawa b, 2, Shugo Kuraishi a, 1, Shota Ikegami a, 1, Toshimasa Futatsugi a, 1, Hiroki Oba a, 1, Hiroyuki Kato a, 1 a b
Department of Orthopaedic Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan Department of Pediatrics, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan
a r t i c l e i n f o
a b s t r a c t
Article history: Received 11 August 2017 Received in revised form 17 October 2017 Accepted 2 November 2017 Available online xxx
Background: As the surgical treatment of scoliosis after a Fontan procedure is very challenging due to the risk of various perioperative complications, case reports are scarce. We herein describe three patients who were successfully treated for scoliosis following a Fontan procedure and discuss their clinical and radiological outcomes. Methods: We retrospectively reviewed three cases of scoliosis treated by posterior spinal fusion after a Fontan procedure. Results: Mean preoperative major curve Cobb angle was 83.7, mean surgical time was 233.0 min, and mean blood loss was 1167 g. The mean correction rate of the major curve was 48.0%. Surgical outcome as evaluated by Scoliosis Research Society-22 patient questionnaires revealed acceptable results without any severe complications. Conclusions: Corrective surgery for scoliosis after a Fontan procedure becomes a stronger option if cardiac insufficiency is prevented during the perioperative period and a conservative plan is carried out with minimal invasiveness and operation time. © 2017 The Japanese Orthopaedic Association. Published by Elsevier B.V. All rights reserved.
1. Introduction The Fontan procedure is a functional repair technique anastomosing the superior vena cava and inferior vena cava to the pulmonary artery to treat congenital heart disease with cyanosis. Although several studies have linked scoliosis with congenital heart disease, the aetiology of this relationship remains unknown [1e3]. Khadhim et al. [4] identified a high prevalence of scoliosis in patients with Fontan circulation. The survival rate of patients with complex congenital heart diseases is improving, thus creating a subset of individuals with a complex physiology who require surgical treatment for scoliosis due to worsening curvature, back pain, secondary impairment of cardio-respiratory function, and the lack of conservative treatment options [5]. To date, there exist few case reports of surgical treatment for scoliosis after a Fontan procedure [5e7]. We herein describe the
* Corresponding author. Fax: þ81 263 35 8844. E-mail address: [email protected] (J. Takahashi). 1 Fax: þ81 263 35 8844. 2 Fax: þ81 263 37 3089.
clinical and radiological features of three patients with Fontan circulation who were surgically treated for severe scoliosis. 1.1. Case presentations This study was approved by the ethics committee of our hospital (No. 3765). This was a retrospective, consecutive case series study that included three patients aged 11, 12, and 14 years, respectively, with scoliosis and Fontan circulation who were treated by posterior spinal fusion using pedicle screws in tandem with careful monitoring of circulatory dynamics in the perioperative period. All cases received surgery for scoliosis subsequent to a Fontan procedure at our hospital. Written informed consent was obtained from the patients' parents. Perioperative findings are summarized in Table 1. Mean preoperative main curve Cobb angle was 83.7 ± 35.5 , mean surgical time was 233.0 ± 91.1 min, and mean blood loss was 1167 ± 764 g. The mean correction rate of the main curve was 48.0 ± 15.3%. Surgical outcome as evaluated by the Scoliosis Research Society-22 patient questionnaire (SRS-22) revealed favourable results without severe complications (Fig. 1).
https://doi.org/10.1016/j.jos.2017.11.004 0949-2658/© 2017 The Japanese Orthopaedic Association. Published by Elsevier B.V. All rights reserved.
Please cite this article in press as: Uehara M, et al., Posterior spinal fusion for scoliosis after Fontan procedure: A case series, Journal of Orthopaedic Science (2017), https://doi.org/10.1016/j.jos.2017.11.004
2
M. Uehara et al. / Journal of Orthopaedic Science xxx (2017) 1e5
Table 1 Perioperative patient data. Blood loss Fusion Age Gender Risser Follow-up Surgical volume (g) area time (years) sign period (months) (minutes) Case 1 Case 2 Case 3 Mean
11 12 14 12.3
F M F
2 0 4
36 16 9 20.3
167 337 195 233.0
1000 2000 500 1167
Post-op major Correction Pre-op T5-T12 Post-op T5-T12 Ponte Pre-op major rate (%) kyphotic angle kyphotic angle curve Cobb osteotomy curve Cobb (degrees) (degrees) angle (degrees) angle (degrees)
T5-L1 e T5-L4 e T5-T11 þ
77 (MT) 122 (MT) 52 (MT) 83.7
34 85 17 45.3
55.8 30.3 67.3 51.2
20 40 9 23
20 21 35 25.3
MT: main thoracic.
Case 1: An 11-year-old girl had received a Fontan procedure at the age of 3 years. We performed posterior spinal fusion from T5 to L1 for her preoperative main thoracic (MT) curve Cobb angle of 77. Surgical time was 167 min and blood loss volume was 1000 g. She suffered from pleural effusion immediately after surgery that improved a day later with conservative treatment. Postoperative Cobb angle of the MT curve improved to 34 . Thoracic kyphotic angle remained at 20 (Fig. 2). Forced vital capacity improved from 2.11 L before surgery to 2.51 L afterwards. Preoperative SRS-22 domain scores were 3.8, 4.4, 2.8, 4.2, and 3.8 for function, pain, self-image, mental health, and subtotal, respectively, which were improved at 50 months at 4.8, 5.0, 4.4, 5.0, and 4.8, respectively. Case 2: A 12-year-old boy had received a Fontan procedure at the age of 4 years. We performed posterior spinal fusion from T5 to L4 for his preoperative MT curve Cobb angle of 122 . Surgical time was 337 min and blood loss volume was 2000 g. He suffered from pleural effusion immediately after surgery that improved a week later with conservative treatment. Postoperative Cobb angle of the MT curve improved to 85 . Thoracic kyphotic angle improved from 40 before surgery to 21 afterwards (Fig. 3). Forced vital capacity improved from 0.36 L before surgery to 0.56 L afterwards. Preoperative SRS-22 domain scores were 4.4, 5.0, 2.4, 5.0, and 4.2 for function, pain, self-image, mental health, and subtotal, respectively,
Subtotal
Mental health
2. Discussion In the present case series of three patients with Fontan circulation receiving posterior spinal fusion for severe scoliosis, pedicle screw fixation improved both radiological and clinical parameters without severe perioperative complications, although two patients experienced pleural effusion that improved with conservative treatment.
Case 1
Case 2
Function 5 4 3 2 1 0
Function 5 4 3 2 1 0
Mental health
Subtotal
which were marginally improved at 16 months at 4.2, 5.0, 3.0, 4.8, and 4.25, respectively. Case 3: A 14-year-old girl had received a Fontan procedure at the age of 2 years. We performed posterior spinal fusion from T5 to T11 for her preoperative MT curve Cobb angle of 52 . Ponte osteotomy was added since this case had a rigid curve and thoracic hypokyphosis and there was little intraoperative bleeding. Surgical time was 195 min and blood loss volume was 500 g. There were no perioperative complications. Postoperative Cobb angle of the MT curve improved to 22 . Thoracic kyphotic angle (T5-T12) improved from 9 before surgery to 35 afterwards (Fig. 4). Preoperative SRS22 domain scores were 4.2, 4.6, 2.6, 4.6, and 4.0 for function, pain, self-image, mental health, and subtotal, respectively, which were improved at 1 year at 4.6, 5.0, 4.2, 4.6, and 4.6, respectively.
Pain
Self-image
Subtotal
Mental health
Mean
Function 5 4 3 2 1 0
Function 5 4 3 2 1 0
Self-image
Subtotal
Mental health
Final follow-up
Pain
Self-image
Case 3
Pain
Preoperative
Pain
Self-image
Fig. 1. Surgical outcome as evaluated by the Scoliosis Research Society-22 patient questionnaire.
Please cite this article in press as: Uehara M, et al., Posterior spinal fusion for scoliosis after Fontan procedure: A case series, Journal of Orthopaedic Science (2017), https://doi.org/10.1016/j.jos.2017.11.004
M. Uehara et al. / Journal of Orthopaedic Science xxx (2017) 1e5
3
Fig. 2. Case 1: an 11-year-old girl. (a) Preoperative Cobb angle of the MT curve was 77. (b) We performed posterior spinal fusion from T5 to L1. Postoperative Cobb angle of the MT curve improved to 34 .
Fig. 3. Case 2: a 12-year-old boy. (a) Preoperative Cobb angle of the MT curve was 122 . (b) We performed posterior spinal fusion from T5 to L4. Postoperative Cobb angle of the MT curve improved to 85 .
A relationship between spinal and major cardiac abnormalities is well known [8], with the frequency of scoliosis in patients with congenital heart disease ranging from 11% to 34% [9]. It has also been suggested that cyanosis associated with congenital heart anomalies is a risk factor for the development of scoliosis [10,11]. Owing to improvements in the survival rate of patients with complex congenital heart disease, a growing subset of individuals with Fontan circulation who require surgical treatment for severe scoliosis has emerged [5]. Herrera et al. reported that sagittal alignment in scoliosis patients after sternotomy for congenital heart disease tended towards kyphosis [1]. In our series, however, the thoracic kyphotic angles of all patients were almost normal. The management of surgical scoliosis correction after a Fontan procedure is very difficult due to the risk of various perioperative complications. Hedequist et al. observed that six of seven patients who received posterior correction surgery for scoliosis after a
Fontan procedure experienced postoperative pleural effusion [6], rez-Caballero et al. reported that one of eight patients with while Pe Fontan circulation died after scoliosis correction surgery due to hypovolemic shock [7]. In the present series, we encountered no severe complications in our careful monitoring of circulatory dynamics over the perioperative period. Two of three patients suffered from pleural effusion that improved with conservative treatment. Indeed, special considerations are necessary in posterior spinal fusion for scoliosis after a Fontan procedure to avoid heart failure. Hedequist et al. [6] postulated that a short operation time without prolonged attempts at instrumentation or correction in addition to a minor loss of blood led to better clinical outcomes. In our cohort, the mean surgical time was 233.0 min and mean blood loss was 1167 g, which were similar to those in previous study of adolescent idiopathic scoliosis (AIS; mean surgical time: 242 min,
Please cite this article in press as: Uehara M, et al., Posterior spinal fusion for scoliosis after Fontan procedure: A case series, Journal of Orthopaedic Science (2017), https://doi.org/10.1016/j.jos.2017.11.004
4
M. Uehara et al. / Journal of Orthopaedic Science xxx (2017) 1e5
Fig. 4. Case 3: a 14-year-old girl. (a) Preoperative Cobb angle of the MT curve was 52 . (b) We performed posterior spinal fusion from T5 to T11. Postoperative Cobb angle of the MT curve improved to 22 .
mean blood loss volume: 1060 g) [12]. Regarding intraoperative tissue bleeding, haemorrhage was more than usual for case 2 but comparable to that in normal scoliosis for the other two cases. Ponte osteotomy was generally avoided to minimize blood loss. However, case 3 had a rigid curve and thoracic hypokyphosis, for which Ponte osteotomy produced good thoracic kyphosis with little intraoperative bleeding through the use of Floseal® (Baxter, Deerfield, IL, USA), a haemostatic agent made of bovine-derived gelatin matrix and human-derived thrombin. Although the correction rate in this case series was lower than in previous AIS series (48.0% vs. 62.4% [12]), surgical outcome as evaluated by SRS-22 showed acceptable results without severe complications. Thus, conservative correction with minimal invasiveness is advised for patients having received a Fontan procedure. Chan et al. reported a case of severe kyphoscoliosis after Fontan surgery in which preoperative halo traction provided a correction rate of 64% with an operation time of 150 min and blood loss of 1050 mls and concluded that traction was useful for decreasing blood loss volume and operation time [13]. For our case 2 with severe scoliosis of more than 100 , bleeding and surgery time were roughly double those of Chan's case. Although we could safely correct the deformity without preoperative halo traction, better correction might have been possible with this technique. The key features of Fontan circulation are a high central venous pressure (CVP) required to maintain pulmonary circulation due to the absence of the right ventricle and a low cardiac output, even under normal conditions. Blood loss exacerbates the diminished cardiac function to increase the risk of circulatory failure, and so it is essential to limit the loss of blood with transfusions and catecholamines under careful monitoring. The high venous pressure necessary to drive the Fontan circulation, particularly whilst in the prone position, may result in significant blood loss [6,14]. It is therefore desirable to minimize operation time. Furthermore, since high positive end expiratory pressure from artificial ventilation is very disadvantageous for pulmonary circulation in patients with Fontan circulation, prompt extubation is also important after surgery. In our cohort, adjustment of transfusions and catecholamines based on circulation monitoring prevented postoperative hypotension. Thus, the main success factors appeared to be short
operation time, minimized blood loss, conservative planning of correction rate, and careful circulation monitoring and management. Specifically to shorten operative time, we avoided Ponte osteotomy when possible by using a rigid rod on the concave side and shortened the fusion area as much as possible. To reduce blood loss volume, we avoided Ponte osteotomy and actively used haemostatic agents, such as Floseal®. The precise cause of pleural effusion in patients after a Fontan procedure has not been identified. However, many potential factors, such as increased pulmonary artery pressure, reduced ventricular function, ventricular type, prolonged mechanical ventilation, moderate-to-severe systemic atrioventricular valve regurgitation, and longer cardiopulmonary bypass, may be involved [15]. In our series, two of three patients (case 1 and 2) exhibited pleural effusion that was successfully managed. Accordingly, perioperative precautions for patients after a Fontan procedure include: (1) proper monitoring of CVP, (2) extubation as soon as possible, (3) the use of catecholamines to enhance vasodilation and cardiac contractility, and (4) performing intensive intraoperative monitoring and moisture management in patients at high risk for postoperative pleural effusion, such as those with high CVP, poor cardiac function, severe atrioventricular valve regurgitation, and the necessity of large amounts of diuretics after Fontan procedures. With regard to surgical outcome, all SRS-22 domain scores were markedly improved in two of three patients, with the remaining patient having a preoperative curve of greater than 120 experiencing only modest gains. Nonetheless, surgery is advised in such cases before the curve becomes too large. The surgical procedure in case 2 was considered effective because forced vital capacity was improved to prevent possible future respiratory failure. Continued observation is necessary due to the short follow-up periods in two patients. It is important to understand the distinction between ordinal AIS and of post-Fontan procedures. Ordinal AIS normally has few perioperative complications, and so even if time or blood loss volume becomes elevated, optimal correction can still be pursued. On the other hand, patients with a Fontan procedure have poor cardiac function. To prevent serious complications, only the minimal
Please cite this article in press as: Uehara M, et al., Posterior spinal fusion for scoliosis after Fontan procedure: A case series, Journal of Orthopaedic Science (2017), https://doi.org/10.1016/j.jos.2017.11.004
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necessary correction is advised to minimize bleeding volume, and patients should be extubated as soon as possible after surgery. 3. Conclusion Based on the present case series, it is important to prevent cardiac insufficiency during the perioperative period, make a conservative correction plan, and minimize invasiveness in surgery for scoliosis following a Fontan procedure. Conflict of interest The authors declare that they have no conflict of interest. References [1] Herrera-Soto JA, Vander Have KL, Barry-Lane P, Myers JL. Retrospective study on development of spinal deformity following sternotomy for congenital heart disease. Spine (Phila Pa 1976) 2007 Aug 15;32(18):1998e2004. [2] Ruiz-Iban MA, Burgos J, Aguado HJ, Diaz-Heredia J, Roger I, Muriel A, Sanchez PA. Scoliosis after median sternotomy in children with congenital heart disease. Spine (Phila Pa 1976) 2005 Apr 15;30(8):E214e8. [3] Herrera-Soto JA, Vander Have KL, Barry-Lane P, Woo A. Spinal deformity after combined thoracotomy and sternotomy for congenital heart disease. J Pediatr Orthop 2006 MareApr;26(2):211e5. [4] Kadhim M, Pizarro C, Holmes Jr L, Rogers KJ, Kallur A, Mackenzie WG. Prevalence of scoliosis in patients with Fontan circulation. Arch Dis Child 2013 Mar;98(3):170e5.
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[5] Leichtle CI, Kumpf M, Gass M, Schmidt E, Niemeyer T. Surgical correction of scoliosis in children with congenital heart failure (Fontan circulation): case report and literature review. Eur Spine J 2008 Sep;17(Suppl. 2):S312e7. [6] Hedequist DJ, Emans JB, Hall JE. Operative treatment of scoliosis in patients with a Fontan circulation. Spine 2006 Jan 15;31(2):202e5. rez-Caballero Macarro n C, Sobrino Ruiz E, Burgos Flores J, Va zquez [7] Pe rez A, Alvarez Rojas E, Sa nchez Ruas J. Spinal surgery in the Martínez J, Coca Pe univentricular hearteis it viable? Cardiol Young 2014 Feb;24(1):73e8. [8] Ogilvie JW. Congenital heart disease and scoliosis. In: Lonstein JE, Winter RB, Bradford DS, Ogilvie JW, editors. Moe's textbook of scoliosis and other spinal deformities. Philadelphia, PA: W.B. Saunders Co; 1995. p. 564e5. [9] Bozcali E, Ucpunar H, Sevencan A, Balioglu MB, Albayrak A, Polat V. A retrospective study of congenital cardiac abnormality associated with scoliosis. Asian Spine J 2016 Apr;10(2):226e30. [10] Kawakami N, Mimatsu K, Deguchi M, Kato F, Maki S. Scoliosis and congenital heart disease. Spine (Phila Pa 1976) 1995 Jun 1;20(11):1252e5, discussion 1256. [11] Roth A, Rosenthal A, Hall JE, Mizel M. Scoliosis and congenital heart disease. Clin Orthop Relat Res 1973 Jun;93:95e102. [12] Uehara M, Takahashi J, Kuraishi S, Shimizu M, Ikegami S, Futatsugi T, Oba H, Kato H. Computer-assisted skip pedicle screw fixation for adolescent idiopathic scoliosis. J Orthop Sci 2017 Mar;22(2):218e23. [13] Chan CY, Lim CY, Shahnaz Hasan M, Kwan MK. The use of pre-operative halo traction to minimize risk for correction of severe scoliosis in a patient with Fontan circulation: a case report and review of literature. Eur Spine J 2016 May;25(Suppl. 1):245e50. [14] Rafique MB, Stuth EA, Tassone JC. Increased blood loss during posterior spinal fusion for idiopathic scoliosis in an adolescent with Fontan physiology. Pediatr Anaesth 2006 Feb;16(2):206e12. [15] Pike NA, Okuhara CA, Toyama J, Gross BP, Wells WJ, Starnes VA. Reduced pleural drainage, length of stay, and readmissions using a modified Fontan management protocol. J Thorac Cardiovasc Surg 2015 Sep;150(3): 481e7.
Please cite this article in press as: Uehara M, et al., Posterior spinal fusion for scoliosis after Fontan procedure: A case series, Journal of Orthopaedic Science (2017), https://doi.org/10.1016/j.jos.2017.11.004