Introduction: Chylothorax, a relatively rare congenital heart disease early postoperative complication, is occurring more frequently due to complexity of cardiac surgeries.
Presentation of case: We present a 9-month-old boy who had hypoplastic left heart (HLH) syndrome with interrupted inferior vena cava (IVC) and bilateral superior vena cava (SVC) palliated with left sided modified Blalock-Taussig (MBT) shunt during neonatal period and second stage palliation with left sided bidirectional glen (BDG) procedure and right sided Kawashima procedure develop bilateral chylothorax two weeks after discharge.
Discussion: This is the first reported case in the literature of a patient who developed chylothorax with relatively low Fontan systemic venous pressures after a Kawashima procedure. Clinically important chylothorax may be a marker of poor long-term outcomes, demonstrating an inability to handle overwhelming lymphatic congestion.
Conclusion: Early diagnosis of chylothorax in complex cardiac surgeries may permit successful conservative management

Adams, Jennifer

Raj, Shekhar S.

Yarrabolu, Tharak

English

Scholarworks@UTRGV Univ. of Texas RioGrande Valley

University of Texas Rio Grande Valley ScholarWorks @ UTRGV School of Medicine Publications and Presentations School of Medicine 4-9-2022 Bilateral chylothorax: A late complication of Kawashima procedure despite normal pulmonary pressures Jennifer Adams The University of Texas Rio Grande Valley Tharak Yarrabolu Shekhar S. Raj Follow this and additional works at: https://scholarworks.utrgv.edu/som_pub  Part of the Medicine and Health Sciences Commons Recommended Citation Adams, J., Yarrabolu, T., & Raj, S. S. (2022). Bilateral chylothorax: A late complication of Kawashima procedure despite normal pulmonary pressures. International journal of surgery case reports, 93, 106981. https://doi.org/10.1016/j.ijscr.2022.106981 This Article is brought to you for free and open access by the School of Medicine at ScholarWorks @ UTRGV. It has been accepted for inclusion in School of Medicine Publications and Presentations by an authorized administrator of ScholarWorks @ UTRGV. For more information, please contact justin.white@utrgv.edu, william.flores01@utrgv.edu. International Journal of Surgery Case Reports 93 (2022) 106981Available online 28 March 20222210-2612/© 2022 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license(http://creativecommons.org/licenses/by-nc-nd/4.0/).Case report Bilateral chylothorax: A late complication of Kawashima procedure despite normal pulmonary pressures Jennifer Adams c,*, Tharak Yarrabolu a, Shekhar S. Raj b a Department of Pediatrics, Section of Pediatric Cardiology, Driscoll Children's Hospital, 3533 South Alameda St, Corpus Christi, USA b Department of Pediatrics, Section of Pediatric Critical Care, Driscoll Children's Hospital, 3533 South Alameda St, Corpus Christi, USA c University of Texas Rio Grande Valley - School of Medicine, 1201 W University Dr, Edinburg, TX, USA   A R T I C L E  I N F O   Keywords: Chylothorax Cardiac surgery Congenial heart disease Post operative complications Kawashima procedure Bidirectional glen procedure A B S T R A C T   Introduction: Chylothorax, a relatively rare congenital heart disease early postoperative complication, is occur-ring more frequently due to complexity of cardiac surgeries. Presentation of case: We present a 9-month-old boy who had hypoplastic left heart (HLH) syndrome with inter-rupted inferior vena cava (IVC) and bilateral superior vena cava (SVC) palliated with left sided modified Blalock- Taussig (MBT) shunt during neonatal period and second stage palliation with left sided bidirectional glen (BDG) procedure and right sided Kawashima procedure develop bilateral chylothorax two weeks after discharge. Discussion: This is the first reported case in the literature of a patient who developed chylothorax with relatively low Fontan systemic venous pressures after a Kawashima procedure. Clinically important chylothorax may be a marker of poor long-term outcomes, demonstrating an inability to handle overwhelming lymphatic congestion. Conclusion: Early diagnosis of chylothorax in complex cardiac surgeries may permit successful conservative management.   1. Introduction Chylothorax after cardiac surgery is a known complication and its incidence is around 2.8% [1], 9.2% in high- risk patients [2]. Chylo-thorax is an early postoperative complication with median time to diagnosis of 6 days [3]. Common etiologies are injury to thoracic duct, high central venous pressure, or presence of thrombus in Superior vena cava (SVC) system [1–3]. Our patient developed chylothorax following left sided bidirectional glen and right Kawashima procedure with mul-tiple interesting features. Chylothorax happened late in the post oper-ative period after patient was discharged home from the hospital. Effusions were large and occurred in the absence of high venous pres-sures or thrombus in the SVC system and were resolved with conser-vative management. This work has been reported in line with the SCARE 2020 criteria [9]. 2. Presentation of case Our patient is a 9-month-old boy with complex cyanotic congenital heart disease including hypoplastic left heart (HLH), double outlet right ventricle (DORV), interrupted inferior vena cava (IVC), bilateral superior vena cava (SVC), mitral valve atresia and pulmonary stenosis. Patient has confirmed diagnosis of heterotaxy syndrome with left atrial isomerism, an interrupted inferior vena cava with azygos continuation. Family history and medication history is noncontributory. At 10 days of age, patient had palliation with left sided modified Blalock-Taussig (MBT) shunt with 3.5 mm Gore-Tex graft. At 8 months of age patient had cardiac catheterization to evaluate for bidirectional glen (BDG) procedure. The patient's hemodynamics data and cardiac anatomy are shown in Fig. 1. One week after cardiac catheterization, he underwent second stage palliative cardiac surgery of takedown of left MBT shunt, right sided Kawashima procedure, left sided BDG, right pulmonary arterioplasty and atrial septectomy. Cardiopulmonary bypass time was 174 min with aortic cross clamp time of 106 min. His Glen pressures during post operative periods were 9 to 10 mm Hg. Patient was discharged home on postoperative day 5. Three days after cardiac surgery in outpatient setting, patient was asymptomatic and SpO2 on room air was 92%. Fifteen days after cardiac surgery, patient developed tachypnea, cyanosis, SpO2 of 75%. Chest X-ray showed bilateral plural effusions with right side predominance and worsening of pulmonary opacification (Fig. 2). On arrival in PICU, oxygen saturation was 80% with high-flow * Corresponding author. E-mail address: Jennifer.adams01@utrgv.edu (J. Adams).  Contents lists available at ScienceDirect International Journal of Surgery Case Reports journal homepage: www.elsevier.com/locate/ijscr https://doi.org/10.1016/j.ijscr.2022.106981 Received 13 February 2022; Received in revised form 15 March 2022; Accepted 24 March 2022   International Journal of Surgery Case Reports 93 (2022) 1069812nasal cannula at 10 lpm. Right side chest tube was placed and 500 ml of chylous pleural fluid was drained with significant improvement in res-piratory symptoms. Pleural effusion analysis revealed elevated Triglyc-eride (TG) level (864 mg/dl), protein level (3 g/dl), LDH of 210, confirming chylothorax. Left sided chest tube was placed next day draining 110 ml of fluid and chest tube additionally drained 55 ml of fluid over next 24 h. The procedures were performed by the on-call intensivist. Internal jugular ultrasound ruled out evidence of clot. Pa-tient was initially managed with low fat diet (Enfaport™, Mead Johnson nutrition, Evansville, IN. USA), diuretics, empiric sildenafil with partial improvement then maintained on total parenteral nutrition and octreotide infusion for complete resolution. A significant reduction in pleural output was noted. With continued improvement the chest drain was removed at day 10 which allowed for incremental reduction of octreotide therapy and establishment of a low- fat oral diet and close observation for rebound chylous accumulation. Patient at one week follow-up in outpatient setting by pediatric cardi-ologist showed no recurrence of pleural effusion. He was found to be stable with appropriate growth and oxygen saturations. At the time of this writing, he continued to have a gastrostomy tube but had begun supplementing tube feeds with oral feeds which he tolerated well. 3. Discussion The Bidirectional Glenn (BDG) procedure, which creates an anasto-mosis between the superior vena cava (SVC) and ipsilateral pulmonary artery (PA), is the second stage of repair in Fontan completion indicated for patients with anatomic or functional single ventricle. The Kawa-shima procedure is similar to BDG but adapted for interrupted inferior vena cava (IVC) anatomy. The Fontan, which is the final procedure in the series, connects the IVC to the PA. Patients with interrupted IVC will have a Fontan-like circulation after undergoing the bidirectional Glenn/ Kawashima due to the influx of splanchnic venous blood from azygous circulation. Chylothorax is less likely to occur after BDG (2.2%) compared to post-Fontan (12%) due to relatively lower postoperative systemic venous pressure [4]. Reported complications from the Kawa-shima procedure in the literature include severe cyanosis secondary to anomalous systemic venous return or unrecognized congenital porto-systemic venous connections [10]. This case is unique in that it is the first documented case chylothorax after the Kawashima procedure despite the presence of relatively low Fontan systemic venous pressures. The mechanisms of chylothorax after cardiac surgery can be due direct trauma to the lymphatic vessels, less commonly secondary to central venous hypertension after cavopulmonary connection [5]. Relatively good lymphatic blood flow is maintained at a systemic venous pressure <15 mm Hg [6] and an animal study also has shown preserved thoracic lymph flow at relatively high systemic venous pressures [7]. Lymphatic abnormalities are also common in children with single ventricle physiology with palliative surgeries [8]. This evidence in-dicates that development of chylothorax after cardiac surgery is multi-factorial. It is difficult to predict which patients develop chylothorax after high-risk cardiac surgery [6] and early identification and treatment is crucial for good outcome [4]. Our patient developed chylothorax relatively late in the post oper-ative course and effusions were bilateral. Patient recovery in the hospital with conservative management and no recurrence after few months of follow up indicate temporary cause for chylothorax, most likely excess burden on lymphatic drainage. Despite high volume of chylothorax (>15 ml/kg/day), child recovered without surgical intervention. Patient perspective N/A. Informed consent Written informed consent was obtained from the patient for publi-cation of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request. Provenance and peer review Not commissioned, externally peer-reviewed. Ethical approval N/A. Funding This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Guarantor Raj Shekhar M.D. Research registration number N/A. Fig. 1. Cardiac diagram showing anatomy, oxygen saturation and pressures. Numbers indicate systolic/diastolic and mean pressures in mm Hg. Circled numbers indicate oxygen saturations. A: Right atrium. B: Right and left superior vena cava (SVC). C: Right and left pulmonary arteries. D: Ascending aorta. E: Main pulmonary artery. F: Absent inferior vena cava (IVC). G: Vertical vein draining into right SVC. J. Adams et al.                                                                                                                                                                                                                                  International Journal of Surgery Case Reports 93 (2022) 1069813CRediT authorship contribution statement JA was involved in the writing of the manuscript. RS and YT were involved in the editing/supervision of the manuscript. Declaration of competing interest The authors declare no conflicts of interests. References [1] C.M. Mery, B.S. Moffett, M.S. Khan, W. Zhang, F.A. Guzmán-Pruneda, C. D. Fraser Jr., A.G. Cabrera, Incidence and treatment of chylothorax after cardiac surgery in children: analysis of a large multi-institution database, J. Thorac. Cardiovasc. Surg. 147 (2) (2014 Feb) 678–686. [2] E.S. Biewer, C. Zürn, R. Arnold, et al., Chylothorax after surgery on congenital heart disease in newborns and infants -risk factors and efficacy of MCT-diet, J. Cardiothorac. Surg. 5 (2010) 127. [3] E.H. Chan, J.L. Russell, W.G. Williams, G.S. Van Arsdell, J.G. Coles, B. W. McCrindle, Postoperative chylothorax after cardiothoracic surgery in children, Ann. Thorac. Surg. 80 (5) (2005 Nov) 1864–1870. [4] P. Akbari Asbagh, M.A. Navabi Shirazi, A. Soleimani, et al., Incidence and etiology of chylothorax after congenital heart surgery in children, J. Tehran Heart Cent. 9 (2) (2014) 59–63. [5] M.A. Ahmed Sr., Post-operative chylothorax in children undergoing congenital heart surgery, Cureus 13 (3) (2021 Mar 10), e13811. [6] M. Lo Rito, O.O. Al-Radi, A. Saedi, Y. Kotani, V. Ben Sivarajan, J.L. Russell, C. A. Caldarone, G.S. Van Arsdell, O. Honjo, Chylothorax and pleural effusion in contemporary extracardiac fenestrated fontan completion, J. Thorac. Cardiovasc. Surg. 155 (5) (2018 May) 2069–2077. [7] R.A. Brace, G.J. Valenzuela, Effects of outflow pressure and vascular volume loading on thoracic duct lymph flow in adult sheep, Am. J. Phys. 258 (1 Pt 2) (1990 Jan) R240–R244. [8] Y. Dori, M.S. Keller, M.A. Fogel, J.J. Rome, K.K. Whitehead, M.A. Harris, M. Itkin, MRI of lymphatic abnormalities after functional single-ventricle palliation surgery, AJR Am. J. Roentgenol. 203 (2) (2014 Aug) 426–431. [9] R.A. Agha, T. Franchi, C. Sohrabi, G. Mathew, for the SCARE Group, The SCARE 2020 guideline: updating consensus Surgical CAse REport (SCARE) guidelines, Int. J. Surg. 84 (2020) 226–230. [10] Y. Nakamura, M. Aoki, I. Hagino, H. Nakajima, Severe cyanosis after kawashima operation resulting from congenital portosystemic shunt, Ann. Thorac. Surg. 93 (4) (2012 Apr) 1315–1317, https://doi.org/10.1016/j.athoracsur.2011.08.058. PMID: 22450089. Fig. 2. Chest X-ray at various time periods of the disease process. A: After cardiac surgery at the time of discharge. B: At the time of admission showing large right plural effusion. C. After chest tube insertion on the right side. D. After left chest tube placement. J. Adams et al.                                                                                                                                                                                                                                  

Bilateral chylothorax: A late complication of Kawashima procedure despite normal pulmonary pressures

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Bilateral chylothorax: A late complication of Kawashima procedure despite normal pulmonary pressures

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