- Email: [email protected]
Nuss procedure for a case of asymmetric pectus excavatum associated with Ehlers-Danlos syndrome
Journal of Pediatric Surgery Case Reports 22 (2017) 20e24
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.jpscasereports.com
Nuss procedure for a case of asymmetric pectus excavatum associated with Ehlers-Danlos syndrome Kazunori Masahata*, Chizu Yoneyama, Ryo Tsukada, Chiyoshi Toyama, Souji Ibuka, Keigo Nara, Hideki Soh, Noriaki Usui Department of Pediatric Surgery, Osaka Women's and Children's Hospital, Murodoucho 840, Izumi-shi, Osaka 594-1101, Japan
a r t i c l e i n f o
a b s t r a c t
Article history: Received 6 March 2017 Received in revised form 1 May 2017 Accepted 2 May 2017 Available online 3 May 2017
Ehlers-Danlos syndrome (EDS) is an inherited connective tissue disorder that is often associated with pectus excavatum (PE). The Nuss procedure, which is a minimally invasive approach for the treatment of PE achieves good functional and cosmetic outcomes. We experienced a case of EDS-associated asymmetric PE that was corrected by the Nuss procedure using chondrotomy of the costal cartilages. After this procedure, an excellent chest appearance was achieved. Our experience suggests that the repair of PE can be accomplished safely in EDS patients and that this procedure can achieve good cosmetic results in patients with asymmetric PE. © 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Keywords: Pectus excavatum Nuss procedure Ehlers-Danlos syndrome
1. Introduction Pectus excavatum (PE) is characterized by depression of the anterior chest wall including the lower sternum and costal cartilages [1]. Connective tissue disorders, such as Marfan syndrome and Ehlers-Danlos syndrome (EDS), are often associated with PE [2]. The Nuss procedure, which is a minimally invasive approach for the treatment of PE, can achieve good functional and cosmetic outcomes [3]. In the operative management of the patients with EDS, close attention should be paid to detect complications, such as hemorrhage and easy skin bruising, which may occur due to the patient's tissue fragility. To the best of our knowledge, only a few reports have addressed the surgical repair of PE in EDS patients [2,4]. We herein present a case of EDS that was associated with asymmetric PE and which was corrected by the Nuss procedure with costal cartilage chondrotomy. 2. Case report A 15-year-old male patient with PE was referred for surgery by a medical genetics specialist in our hospital. He has been diagnosed with EDS at 11 years of age based on the distinct craniofacial features, multiple congenital contractures, progressive joint and skin
* Corresponding author. Tel.: þ81 0742 93 7526. E-mail address: [email protected] (K. Masahata).
laxity, and multisystem fragility-related manifestations. Genetic testing revealed carbohydrate sulfotransferase 14/dermatan 4-Osulfotransferase-1(CHST14/D4ST1) deficiency, which represents a specific form of EDS caused by a recessive loss-of-function mutation in CHST14. Based on these findings, the patient was diagnosed with EDS Kosho-type (EDSKT), which is a heterogeneous group of heritable connective tissue disorders. His chest showed left-side depression with a sternal twist (Fig. 1a and b). Preoperative chest computed tomography showed an asymmetric PE with cardiac compression and rightward displacement with a Haller index of 9.5 and an asymmetry index of 119.4. The sternum of this patient was twisted clockwise; the angle of rotation was þ49.0 (Fig. 2a and b). Echocardiography with Doppler ultrasound revealed a mild degree of tricuspid valve regurgitation. Although the patient did not have any other associated symptoms, he had a cosmetic indication and strongly wished for surgical correction. The Nuss procedure was performed under thoracoscopic guidance to treat the patient's PE. Thoracoscopy showed that the most depressed part consisted of the left fifth and sixth costal cartilages. Thus, a small skin incision was made above the depressed left costal cartilages, and the costal cartilages were incised using an electrosurgical knife. When the small skin incisions were made on both mid-axillary lines, subcutaneous or submuscular dissection was difficult because the tissue was fragile and hemorrhagic due to the patient's connective tissue disorder. Double bars were inserted and rotated under the sternum through the fourth and fifth intercostal space. The sternum was
http://dx.doi.org/10.1016/j.epsc.2017.05.004 2213-5766/© 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
K. Masahata et al. / Journal of Pediatric Surgery Case Reports 22 (2017) 20e24
21
Fig. 1. Frontal (a) and lateral (b) views of the patient's chest.
then symmetrically elevated. A drainage tube was inserted into the left thoracic space to drain any collected fluid. No bleeding was observed from the pleural spaces or the subcutaneous tissue. The total amount of the bleeding during the operation was 400 ml. A postoperative chest X-ray showed no other complications (Fig. 3a and b). On the fifth postoperative day, the pleural drainage tube was removed because the pleural effusion, which initially had a bloody appearance, became serous and the amount of effusion fell to <50 ml per day; the initial amount of pleural effusion was >250 ml per day. After the removal of the drainage tube, mild pleural effusion was persistently detected on X-ray images; however, the amount of pleural effusion did not increase. On the 14th postoperative day, the patient suddenly developed mild dyspnea during rest. The breath sounds on the left side of his chest were decreased. Chest X-ray revealed an increase in the left-side pleural effusion (Fig. 4). Although the patient was observed conservatively with bed rest and hemostatic agents, his pleural effusion worsened and the laboratory findings demonstrated anemia with a hemoglobin level of 8.0 g/dl, suggesting the possibility of hemothorax. After drainage by thoracentesis, the pleural effusion which contained some blood resolved and no evidence of recurrence was found. The patient was closely observed until discharge on postoperative day 26. He was
Fig. 2. Chest computed tomography showed left-side asymmetric pectus excavatum with rightward cardiac displacement (a). An asymmetric deformity of the chest wall was caused by the sternal twist (b).
satisfied with the appearance of his chest (Fig. 5a and b) and has been observed for four months after surgery without further complications or a recurrence of the deformity.
3. Discussion EDS is an inherited connective tissue disorder that is characterized by skin hyperextensibility, joint hypermobility, and tissue fragility involving the skin, joints, blood vessels, and internal organs. EDS occurs due to genetic mutations that affect the structure or assembly of collagen. EDS is classified into six major types: classical-type, hypermobility-type, kyphoscoliosis-type,
22
K. Masahata et al. / Journal of Pediatric Surgery Case Reports 22 (2017) 20e24
Fig. 4. A postoperative chest X-ray showing the double bars with left pleural effusion at 14 days after the Nuss procedure.
Fig. 3. Postoperative chest X-rays in frontal (a) and lateral (b) views showed no other complications.
arthrochalasia-type, dermatosparaxis-type and vascular-type [5]. The latter type is the most serious form of EDS; affected patients are at risk of artery, bowel, and uterus rupture. Recently, a case of EDSKT characterized by CHST14/D4ST1 deficiency was reported, the main feature was the recurrence of subcutaneous hematoma, in
addition to joint and skin laxity, and tissue fragility [6]. The condition causes multisystem fragility through the impaired assembly of collagen fibrils, which occurs due to the loss of dermatan sulfate in the decorin glycosaminoglycan side chain, which promotes electrostatic binding between collagen fibrils. This type of EDS is extremely rare; only 31 affected patients from 21 families have been described to date [7]. Marfanoid habitus and PE have also been noted in patients with EDS. Tocchioni et al. reported that connective tissue disorders, including Marfan syndrome and EDS are more likely to be associated with chest wall deformities [2]. Although there have been a few surgical reports on PE in EDS patients, the details of the individual cases were not described. The previous reports found only slightly higher rates of bar displacement after pectus repair in patients with connective tissue disorders [3,4,8]. In contrast, Olbrecht et al. suggested that bar repairs could be safely and effectively performed in patients with connective tissue disorder and that the rate of postoperative complications and the operative characteristics were equivalent to those in patients without such diseases [9]. PE is characterized by the anomalous growth of several ribs and the sternum, which results in the deformity of the thoracic cavity. Although most young PE patients have symmetrical deformities, adolescent patients with PE have a high incidence of asymmetrical deformities [10,11]. Yoshida et al. reported that half of the patients with PE who were >13 years of age showed a moderate or severe asymmetrical deformity [10]. Generally, the patients with asymmetrical PE exhibit an anticlockwise twist of the sternum, which results in a depression of the right-side chest wall, which excludes the heart to the left side. However, in this case, an uncommon clockwise twist of the sternum resulted in the depression of the left-side chest wall, which excluded the heart to the right side. Although the compression of the heart was released by the elevation of the sternum in the Nuss procedure, the displacement of the heart remained; this was possibly a result of heart dislocation due to immobilization. In 1998, Nuss et al. reported an original procedure for the correction of PE [3]; the procedure was less invasive and achieved good cosmetic outcomes. In both pediatric and adult patients with
K. Masahata et al. / Journal of Pediatric Surgery Case Reports 22 (2017) 20e24
23
Pleural effusion is reported to occur as a complication of the Nuss procedure in 6e17% of patients [13e16]. Cheng et al. reported that adult patients were at significant risk of developing pleural effusion after the Nuss procedure with the placement of double bars [15]. When pleural effusion fails to improve after initial conservative management, thoracentesis or continuous drainage is required. In our case, persistent pleural effusion, including late-phase bleeding, was observed and thoracentesis, which was performed due to the increase in pleural effusion, led to the detection of a hemothorax. We presume that the hemothorax, which occurred after the Nuss procedure, resulted from an injury to the intercostal vein due to the friction between the pectus bars and ribs. It is possible that vessel wall fragility causes postoperative hemothorax, because the connective tissue of the vessel walls in EDS shows hypoplasia. In patients with EDSKT, collagen types Ⅰ and Ⅲ are poorly organized and more diffuse and the linear arrangement is disrupted [7]. In conclusion, we presented a case of asymmetric PE that was associated with EDSKT, which was corrected by the Nuss procedure with costal cartilage chondrotomy. Our experience suggested that the repair of PE can be accomplished safely even in EDSKT patients. Although this procedure provided good cosmetic results for a patient with asymmetric PE, surgeons should be aware of the possibility of persistent pleural effusion after the Nuss procedure in patients with EDS. Disclosure of funding None. Conflict of interest The authors declare that they have no conflicts of interest. Sources of support None. References
Fig. 5. Frontal (a) and lateral (b) views of the patient's chest after the Nuss procedure with double bars.
asymmetric PE, it is difficult to achieve good results with the Nuss procedure alone, because the twisted sternum is elevated by the support bar, which thereby maintains the twisted form. Thus, the imbalance of the chest wall along the sternal border remains. In 2015, Uemura et al. reported that a modified Nuss procedure using chondrotomy achieved good cosmetic results in adult patients with asymmetric deformities [12]. Thus, in the present case, we performed the modified procedure, which resulted in a good cosmetic appearance. Although the subcutaneous and submuscular dissections in our case were fragile and hemorrhagic, the Nuss procedure using chondrotomy of the costal cartilages was safely performed to treat a patient with EDSKT. Postoperative complications have been reported to occur after the Nuss procedure. These include pneumothorax, hemothorax, pleural effusion, cardiac injury and bar dislocation [4,13e16].
[1] Kelly RE, Lawson ML, Paidas CN, Hruban RH. Pectus excavatum in a 112-year autopsy series: anatomic findings and the effect on survival. J Pediatr Surg 2005;40:1275e8. [2] Croitoru DP, Kelly Jr RE, Goretsky MJ, Lawson ML, Swoveland B, Nuss D. Experience and modification update for the minimally invasive Nuss technique for pectus excavatum repair in 303 patients. J Pediatr Surg 2002;37: 437e45. [3] Nuss D, Kelly Jr RE, Croitoru DP, Katz ME. A 10-year review of a minimally invasive technique for the correction of pectus excavatum. J Pediatr Surg 1998;33:545e52. [4] Tocchioni F, Ghionzoli M, Messineo A, Romagnoli P. Pectus excavatum and heritable disorders of the connective tissue. Pediatr Rep 2013;5(3):e15. [5] Beighton P, Bristow J. The Ehlers-Danlos syndrome: on beyond collagens. J Clin Invest 2001;107:1063e9. [6] Kosho T. Discovery and delineation of dermatan 4-O-sulfotransferase-1 (D4ST1)-deficient Ehlers-Danlos syndrome. In: Oiso N, Kawada A, editors. Current genetics in dermatology. Croatia: InTech; 2013. p. 73e86. [7] Kosho T. CHST14/D4ST1 deficiency: new form of Ehlers-Danlos syndrome. Pediatr Int 2016;58:88e99. [8] Nuss D. Recent experiences with minimally invasive pectus excavatum repair “Nuss procedure”. Jpn J Thorac Cardiovasc Surg 2005;53:338e44. [9] Olbrecht VA, Nabaweesi R, Arnold MA, Chandler N, Chang DC, McIltrot KH, et al. Pectus bar repair of pectus excavatum in patients with connective tissue disease. J Pediatr Surg 2009;44:1812e6. [10] Yoshida A, Uemura S, Yamamoto M, Nouso H, Kuyama H, Muta Y. Correlation of asymmetric chest wall deformity and growth in patients with pectus excavatum. J Pediatr Surg 2013;48:771e5. [11] Park HJ, Sung SW, Park JK, Kim JJ, Jeon HW, Wang YP. How early can we repair pectus excavatum: the earlier the better? Eur J Cardiothorac Surg 2012;42: 667e72. [12] Uemura S, Yoshida A, Yamamoto M, Kuyama H. Thoracoscopic costal
24
K. Masahata et al. / Journal of Pediatric Surgery Case Reports 22 (2017) 20e24
chondrotomy and the Nuss procedure for women with asymmetric pectus excavatum. Jpn J Pediatr Surg 2015;47:14e8. [13] Park HJ, Lee SY, Lee CS. Complications associated with the Nuss procedure: analysis of risk factors and suggested measures for prevention of complications. J Pediatr Surg 2004;39:391e5. [14] Yoon YS, Kim HK, Choi YS, Kim K, Shim YM, Kim J. A modified Nuss procedure for late adolescent and adult pectus excavatum. World J Surg 2010;34:
1475e80. [15] Cheng YL, Lin CT, Wang HB, Chang H. Pleural effusion complicating after Nuss procedure for pectus excavatum. Ann Thorac Cardiovasc Surg 2014;20:6e11. [16] Croitoru DP, Kelly Jr RE, Goretsky MJ, Gustin T, Keever R, Nuss D. The minimally invasive Nuss technique for recurrent or failed pectus excavatum repair in 50 patients. J Pediatr Surg 2005;40:181e6.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.jpscasereports.com
Nuss procedure for a case of asymmetric pectus excavatum associated with Ehlers-Danlos syndrome Kazunori Masahata*, Chizu Yoneyama, Ryo Tsukada, Chiyoshi Toyama, Souji Ibuka, Keigo Nara, Hideki Soh, Noriaki Usui Department of Pediatric Surgery, Osaka Women's and Children's Hospital, Murodoucho 840, Izumi-shi, Osaka 594-1101, Japan
a r t i c l e i n f o
a b s t r a c t
Article history: Received 6 March 2017 Received in revised form 1 May 2017 Accepted 2 May 2017 Available online 3 May 2017
Ehlers-Danlos syndrome (EDS) is an inherited connective tissue disorder that is often associated with pectus excavatum (PE). The Nuss procedure, which is a minimally invasive approach for the treatment of PE achieves good functional and cosmetic outcomes. We experienced a case of EDS-associated asymmetric PE that was corrected by the Nuss procedure using chondrotomy of the costal cartilages. After this procedure, an excellent chest appearance was achieved. Our experience suggests that the repair of PE can be accomplished safely in EDS patients and that this procedure can achieve good cosmetic results in patients with asymmetric PE. © 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Keywords: Pectus excavatum Nuss procedure Ehlers-Danlos syndrome
1. Introduction Pectus excavatum (PE) is characterized by depression of the anterior chest wall including the lower sternum and costal cartilages [1]. Connective tissue disorders, such as Marfan syndrome and Ehlers-Danlos syndrome (EDS), are often associated with PE [2]. The Nuss procedure, which is a minimally invasive approach for the treatment of PE, can achieve good functional and cosmetic outcomes [3]. In the operative management of the patients with EDS, close attention should be paid to detect complications, such as hemorrhage and easy skin bruising, which may occur due to the patient's tissue fragility. To the best of our knowledge, only a few reports have addressed the surgical repair of PE in EDS patients [2,4]. We herein present a case of EDS that was associated with asymmetric PE and which was corrected by the Nuss procedure with costal cartilage chondrotomy. 2. Case report A 15-year-old male patient with PE was referred for surgery by a medical genetics specialist in our hospital. He has been diagnosed with EDS at 11 years of age based on the distinct craniofacial features, multiple congenital contractures, progressive joint and skin
* Corresponding author. Tel.: þ81 0742 93 7526. E-mail address: [email protected] (K. Masahata).
laxity, and multisystem fragility-related manifestations. Genetic testing revealed carbohydrate sulfotransferase 14/dermatan 4-Osulfotransferase-1(CHST14/D4ST1) deficiency, which represents a specific form of EDS caused by a recessive loss-of-function mutation in CHST14. Based on these findings, the patient was diagnosed with EDS Kosho-type (EDSKT), which is a heterogeneous group of heritable connective tissue disorders. His chest showed left-side depression with a sternal twist (Fig. 1a and b). Preoperative chest computed tomography showed an asymmetric PE with cardiac compression and rightward displacement with a Haller index of 9.5 and an asymmetry index of 119.4. The sternum of this patient was twisted clockwise; the angle of rotation was þ49.0 (Fig. 2a and b). Echocardiography with Doppler ultrasound revealed a mild degree of tricuspid valve regurgitation. Although the patient did not have any other associated symptoms, he had a cosmetic indication and strongly wished for surgical correction. The Nuss procedure was performed under thoracoscopic guidance to treat the patient's PE. Thoracoscopy showed that the most depressed part consisted of the left fifth and sixth costal cartilages. Thus, a small skin incision was made above the depressed left costal cartilages, and the costal cartilages were incised using an electrosurgical knife. When the small skin incisions were made on both mid-axillary lines, subcutaneous or submuscular dissection was difficult because the tissue was fragile and hemorrhagic due to the patient's connective tissue disorder. Double bars were inserted and rotated under the sternum through the fourth and fifth intercostal space. The sternum was
http://dx.doi.org/10.1016/j.epsc.2017.05.004 2213-5766/© 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
K. Masahata et al. / Journal of Pediatric Surgery Case Reports 22 (2017) 20e24
21
Fig. 1. Frontal (a) and lateral (b) views of the patient's chest.
then symmetrically elevated. A drainage tube was inserted into the left thoracic space to drain any collected fluid. No bleeding was observed from the pleural spaces or the subcutaneous tissue. The total amount of the bleeding during the operation was 400 ml. A postoperative chest X-ray showed no other complications (Fig. 3a and b). On the fifth postoperative day, the pleural drainage tube was removed because the pleural effusion, which initially had a bloody appearance, became serous and the amount of effusion fell to <50 ml per day; the initial amount of pleural effusion was >250 ml per day. After the removal of the drainage tube, mild pleural effusion was persistently detected on X-ray images; however, the amount of pleural effusion did not increase. On the 14th postoperative day, the patient suddenly developed mild dyspnea during rest. The breath sounds on the left side of his chest were decreased. Chest X-ray revealed an increase in the left-side pleural effusion (Fig. 4). Although the patient was observed conservatively with bed rest and hemostatic agents, his pleural effusion worsened and the laboratory findings demonstrated anemia with a hemoglobin level of 8.0 g/dl, suggesting the possibility of hemothorax. After drainage by thoracentesis, the pleural effusion which contained some blood resolved and no evidence of recurrence was found. The patient was closely observed until discharge on postoperative day 26. He was
Fig. 2. Chest computed tomography showed left-side asymmetric pectus excavatum with rightward cardiac displacement (a). An asymmetric deformity of the chest wall was caused by the sternal twist (b).
satisfied with the appearance of his chest (Fig. 5a and b) and has been observed for four months after surgery without further complications or a recurrence of the deformity.
3. Discussion EDS is an inherited connective tissue disorder that is characterized by skin hyperextensibility, joint hypermobility, and tissue fragility involving the skin, joints, blood vessels, and internal organs. EDS occurs due to genetic mutations that affect the structure or assembly of collagen. EDS is classified into six major types: classical-type, hypermobility-type, kyphoscoliosis-type,
22
K. Masahata et al. / Journal of Pediatric Surgery Case Reports 22 (2017) 20e24
Fig. 4. A postoperative chest X-ray showing the double bars with left pleural effusion at 14 days after the Nuss procedure.
Fig. 3. Postoperative chest X-rays in frontal (a) and lateral (b) views showed no other complications.
arthrochalasia-type, dermatosparaxis-type and vascular-type [5]. The latter type is the most serious form of EDS; affected patients are at risk of artery, bowel, and uterus rupture. Recently, a case of EDSKT characterized by CHST14/D4ST1 deficiency was reported, the main feature was the recurrence of subcutaneous hematoma, in
addition to joint and skin laxity, and tissue fragility [6]. The condition causes multisystem fragility through the impaired assembly of collagen fibrils, which occurs due to the loss of dermatan sulfate in the decorin glycosaminoglycan side chain, which promotes electrostatic binding between collagen fibrils. This type of EDS is extremely rare; only 31 affected patients from 21 families have been described to date [7]. Marfanoid habitus and PE have also been noted in patients with EDS. Tocchioni et al. reported that connective tissue disorders, including Marfan syndrome and EDS are more likely to be associated with chest wall deformities [2]. Although there have been a few surgical reports on PE in EDS patients, the details of the individual cases were not described. The previous reports found only slightly higher rates of bar displacement after pectus repair in patients with connective tissue disorders [3,4,8]. In contrast, Olbrecht et al. suggested that bar repairs could be safely and effectively performed in patients with connective tissue disorder and that the rate of postoperative complications and the operative characteristics were equivalent to those in patients without such diseases [9]. PE is characterized by the anomalous growth of several ribs and the sternum, which results in the deformity of the thoracic cavity. Although most young PE patients have symmetrical deformities, adolescent patients with PE have a high incidence of asymmetrical deformities [10,11]. Yoshida et al. reported that half of the patients with PE who were >13 years of age showed a moderate or severe asymmetrical deformity [10]. Generally, the patients with asymmetrical PE exhibit an anticlockwise twist of the sternum, which results in a depression of the right-side chest wall, which excludes the heart to the left side. However, in this case, an uncommon clockwise twist of the sternum resulted in the depression of the left-side chest wall, which excluded the heart to the right side. Although the compression of the heart was released by the elevation of the sternum in the Nuss procedure, the displacement of the heart remained; this was possibly a result of heart dislocation due to immobilization. In 1998, Nuss et al. reported an original procedure for the correction of PE [3]; the procedure was less invasive and achieved good cosmetic outcomes. In both pediatric and adult patients with
K. Masahata et al. / Journal of Pediatric Surgery Case Reports 22 (2017) 20e24
23
Pleural effusion is reported to occur as a complication of the Nuss procedure in 6e17% of patients [13e16]. Cheng et al. reported that adult patients were at significant risk of developing pleural effusion after the Nuss procedure with the placement of double bars [15]. When pleural effusion fails to improve after initial conservative management, thoracentesis or continuous drainage is required. In our case, persistent pleural effusion, including late-phase bleeding, was observed and thoracentesis, which was performed due to the increase in pleural effusion, led to the detection of a hemothorax. We presume that the hemothorax, which occurred after the Nuss procedure, resulted from an injury to the intercostal vein due to the friction between the pectus bars and ribs. It is possible that vessel wall fragility causes postoperative hemothorax, because the connective tissue of the vessel walls in EDS shows hypoplasia. In patients with EDSKT, collagen types Ⅰ and Ⅲ are poorly organized and more diffuse and the linear arrangement is disrupted [7]. In conclusion, we presented a case of asymmetric PE that was associated with EDSKT, which was corrected by the Nuss procedure with costal cartilage chondrotomy. Our experience suggested that the repair of PE can be accomplished safely even in EDSKT patients. Although this procedure provided good cosmetic results for a patient with asymmetric PE, surgeons should be aware of the possibility of persistent pleural effusion after the Nuss procedure in patients with EDS. Disclosure of funding None. Conflict of interest The authors declare that they have no conflicts of interest. Sources of support None. References
Fig. 5. Frontal (a) and lateral (b) views of the patient's chest after the Nuss procedure with double bars.
asymmetric PE, it is difficult to achieve good results with the Nuss procedure alone, because the twisted sternum is elevated by the support bar, which thereby maintains the twisted form. Thus, the imbalance of the chest wall along the sternal border remains. In 2015, Uemura et al. reported that a modified Nuss procedure using chondrotomy achieved good cosmetic results in adult patients with asymmetric deformities [12]. Thus, in the present case, we performed the modified procedure, which resulted in a good cosmetic appearance. Although the subcutaneous and submuscular dissections in our case were fragile and hemorrhagic, the Nuss procedure using chondrotomy of the costal cartilages was safely performed to treat a patient with EDSKT. Postoperative complications have been reported to occur after the Nuss procedure. These include pneumothorax, hemothorax, pleural effusion, cardiac injury and bar dislocation [4,13e16].
[1] Kelly RE, Lawson ML, Paidas CN, Hruban RH. Pectus excavatum in a 112-year autopsy series: anatomic findings and the effect on survival. J Pediatr Surg 2005;40:1275e8. [2] Croitoru DP, Kelly Jr RE, Goretsky MJ, Lawson ML, Swoveland B, Nuss D. Experience and modification update for the minimally invasive Nuss technique for pectus excavatum repair in 303 patients. J Pediatr Surg 2002;37: 437e45. [3] Nuss D, Kelly Jr RE, Croitoru DP, Katz ME. A 10-year review of a minimally invasive technique for the correction of pectus excavatum. J Pediatr Surg 1998;33:545e52. [4] Tocchioni F, Ghionzoli M, Messineo A, Romagnoli P. Pectus excavatum and heritable disorders of the connective tissue. Pediatr Rep 2013;5(3):e15. [5] Beighton P, Bristow J. The Ehlers-Danlos syndrome: on beyond collagens. J Clin Invest 2001;107:1063e9. [6] Kosho T. Discovery and delineation of dermatan 4-O-sulfotransferase-1 (D4ST1)-deficient Ehlers-Danlos syndrome. In: Oiso N, Kawada A, editors. Current genetics in dermatology. Croatia: InTech; 2013. p. 73e86. [7] Kosho T. CHST14/D4ST1 deficiency: new form of Ehlers-Danlos syndrome. Pediatr Int 2016;58:88e99. [8] Nuss D. Recent experiences with minimally invasive pectus excavatum repair “Nuss procedure”. Jpn J Thorac Cardiovasc Surg 2005;53:338e44. [9] Olbrecht VA, Nabaweesi R, Arnold MA, Chandler N, Chang DC, McIltrot KH, et al. Pectus bar repair of pectus excavatum in patients with connective tissue disease. J Pediatr Surg 2009;44:1812e6. [10] Yoshida A, Uemura S, Yamamoto M, Nouso H, Kuyama H, Muta Y. Correlation of asymmetric chest wall deformity and growth in patients with pectus excavatum. J Pediatr Surg 2013;48:771e5. [11] Park HJ, Sung SW, Park JK, Kim JJ, Jeon HW, Wang YP. How early can we repair pectus excavatum: the earlier the better? Eur J Cardiothorac Surg 2012;42: 667e72. [12] Uemura S, Yoshida A, Yamamoto M, Kuyama H. Thoracoscopic costal
24
K. Masahata et al. / Journal of Pediatric Surgery Case Reports 22 (2017) 20e24
chondrotomy and the Nuss procedure for women with asymmetric pectus excavatum. Jpn J Pediatr Surg 2015;47:14e8. [13] Park HJ, Lee SY, Lee CS. Complications associated with the Nuss procedure: analysis of risk factors and suggested measures for prevention of complications. J Pediatr Surg 2004;39:391e5. [14] Yoon YS, Kim HK, Choi YS, Kim K, Shim YM, Kim J. A modified Nuss procedure for late adolescent and adult pectus excavatum. World J Surg 2010;34:
1475e80. [15] Cheng YL, Lin CT, Wang HB, Chang H. Pleural effusion complicating after Nuss procedure for pectus excavatum. Ann Thorac Cardiovasc Surg 2014;20:6e11. [16] Croitoru DP, Kelly Jr RE, Goretsky MJ, Gustin T, Keever R, Nuss D. The minimally invasive Nuss technique for recurrent or failed pectus excavatum repair in 50 patients. J Pediatr Surg 2005;40:181e6.