- Email: [email protected]
Successful treatment of liver abscesses with corticosteroids and antibiotics in an infant with chronic granulomatous disease
Journal of Pediatric Surgery Case Reports 35 (2018) 48–51
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Successful treatment of liver abscesses with corticosteroids and antibiotics in an infant with chronic granulomatous disease
T
Jennifer Tama, David Fahmyb, Jeffrey Traubicic, Jacob C. Langerd, Shaun K. Morrisa, Julia E.M. Uptone,∗ a
Division of Infectious Diseases, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada Division of Immunology and Allergy, Department of Medicine, McMaster University, Hamilton, Ontario, Canada c Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, University of Toronto, Ontario, Canada d Division of General and Thoracic Surgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada e Division of Immunology and Allergy, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada b
A R T I C LE I N FO
A B S T R A C T
Keywords: Chronic granulomatous disease Liver abscess Corticosteroids
Liver abscesses occur in up to one-third of patients with chronic granulomatous disease (CGD). Surgery has traditionally been considered the cornerstone of management. Emerging reports in older children and adults, however, have demonstrated successful treatment of CGD-related liver abscesses with corticosteroids and antibiotics alone. We describe the safe and effective use of corticosteroids and antibiotics for CGD-related liver abscesses in an infant with X-linked CGD who had high surgical risk for hepatic resection. Medical management was started at 11 months of age and the abscesses resolved without requiring any subsequent invasive surgical procedures. This case report highlights that corticosteroids and antibiotics may be considered in the management of CGD-related liver abscesses in the infant age group, especially when surgical management is not an option.
1. Introduction Liver abscesses occur in up to 35% of patients with chronic granulomatous disease (CGD), with Staphylococcus aureus being the most frequently cultured pathogen [1,2]. The management of liver abscesses in CGD patients is not well-defined, but surgery has been a cornerstone of therapy [3,4]. A small number of reports in older children and young adults have described successful treatment of CGD-related liver abscesses with corticosteroids [2,5–7], with a more recent case series describing this approach as a “changing paradigm of management of liver abscesses” in CGD [8]. We report a case of an infant with X-linked CGD and a liver abscess successfully treated with corticosteroid and targeted antibiotic therapy without surgical resection. Written informed consent was obtained from the patient's family for this case report. 1.1. Case report A term male infant with a longstanding history of fevers and lymphadenitis developed abdominal distension and right upper quadrant
pain at 10 months of age. Abdominal ultrasound demonstrated a 5.0 × 1.8 × 2.0 cm hepatic subcapsular complex collection. He was empirically started on parenteral piperacillin-tazobactam and ciprofloxacin. CT abdomen (Fig. 1) confirmed a large subcapsular abscess (4.5 × 2.1 × 3.9 cm) on the right side of the liver anteriorly along segment V with extension into the hepatic parenchyma (Fig. 1A), as well as a second ill-defined lesion (0.8 × 0.6 × 0.9 cm) on the left side of the liver anteriorly along the falciform ligament (Fig. 1B). The subcapsular abscess was drained via image-guided therapy the following day, yielding 8 mL of pus, which grew methicillin-sensitive Staphylococcus aureus (MSSA). Piperacillin-tazobactam was changed to parenteral cloxacillin (50 mg/kg every 6 h) and ciprofloxacin was continued for one week while a percutaneous drain remained in situ. Neutrophil oxidative burst index was low on two occasions (9 and 1.1, with normal range 32–300). A clinical diagnosis of CGD was made, later confirmed to be due to a hemizygous nonsense mutation in the CYBB gene ((c.931C > T, p.Gln 311, Denver Genetic Laboratories)), which was classified as pathogenic. At 4 weeks of admission (11 months of age), the patient continued to have intermittent fever, abdominal pain, and elevated inflammatory
∗
Corresponding author. The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. E-mail addresses: [email protected] (J. Tam), [email protected] (D. Fahmy), jeff[email protected] (J. Traubici), [email protected] (J.C. Langer), [email protected] (S.K. Morris), [email protected] (J.E.M. Upton). https://doi.org/10.1016/j.epsc.2018.06.001 Received 30 May 2018; Accepted 2 June 2018 Available online 04 June 2018 2213-5766/ © 2018 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/).
Journal of Pediatric Surgery Case Reports 35 (2018) 48–51
J. Tam et al.
Abbreviations CGD cm CRP CT CYBB ESR
h IV kg L mg mL MSSA
chronic granulomatous disease centimetre C-reactive protein computed tomography cytochrome b-235 beta chain (also known as p91-phox) erythrocyte sedimentation rate
hour intravenous kilogram litre milligram millilitre methicillin-sensitive Staphylococcus aureus
prophylactic itraconazole. Prophylactic trimethoprim-sulfamethoxazole was also initiated. Serial abdominal ultrasound imaging continued to be reassuring with gradually decreasing size of left hepatic lesions with increasing internal calcifications. The right subcapsular abscess did not re-accumulate and no new hepatic lesions occurred. Prednisolone wean started 6 weeks after initiation by approximately 20% weekly. IV cloxacillin was changed to oral cephalexin 150 mg/kg/day divided three times daily at just over 3 months of antibiotic therapy, and continued for an additional 4 months until abdominal imaging only showed small clusters of tiny calcifications. At 2 years of age, the patient underwent bone marrow transplantation for CGD. At over one year post-transplant, there was no evidence of recurrence of liver abscesses. His clinical course is summarized (Table 1).
markers (CRP 66.6 mg/L and ESR 53 mm/h) despite continuing IV cloxacillin. While the right subcapsular abscess had decreased in size post-drainage to 2.1 × 1.9 × 0.4 cm, the lesion in the left hepatic lobe had increased to 1.3 × 1.7 × 1.3 cm with a 2.8 × 1.9 × 2 cm area of surrounding hypoechoic halo. Surgical resection of the abscess was considered; however, based on the location of the lesions and the otherwise stable clinical status, the procedure was deemed to be high risk. Therefore, medical management was attempted with methylprednisolone intravenously (IV) (1 mg/kg/day), while continuing IV cloxacillin. His fevers abated following initiation of corticosteroids. Within one week, his inflammatory markers normalized and abdominal ultrasound demonstrated the right subcapsular abscess had decreased to 3 mm thickness and the left hepatic lobe lesion had decreased to 2.4 × 0.9 × 1.2 cm. Following 2 weeks of IV methylprednisolone, he was discharged home on oral prednisolone (1.5 mg/kg/day divided twice daily), IV cloxacillin, and itraconazole for fungal prophylaxis. Five days after discharge, the patient developed new fever. A CT abdomen showed the right subcapsular liver abscess had almost completely resolved (Fig. 1-2A). However, the left hepatic lesion increased to 1.4 × 1.7 × 2.7 cm (Fig. 1-2B); a biopsy showed only sterile fibrotic granulation tissue. Blood cultures remained negative and he was discharged home to continue oral prednisolone, IV cloxacillin and
2. Discussion We report a case description of an infant with CGD successfully treated for a liver abscess with antibiotic and corticosteroid therapy, thereby avoiding surgical resection. The management of liver abscesses in CGD patients has often included hepatic resection, due to disappointing results from only drainage of the abscess [4]. The risks of Fig. 1. Axial contrast enhanced CT images initially (1) and two months later (2). 1A. A heterogeneous sub-capsular liver lesion (thick white arrow) along segment V demonstrates a low attenuation region with peripheral rim enhancement concerning for an abscess with surrounding phlegmon. It appears to involve the underlying liver parenchyma and likely the adjacent abdominal wall. 1B. A smaller 0.8 × 0.6 × 0.9 cm hypoattenuating lesion (thin white arrow) is seen adjacent to the falciform ligament. 2A. The sub-capsular lesion resolved (thick white arrow) and attenuation changes are improved. 2B. The lesion increased in size (thin white arrow), measuring 1.4 × 1.7 × 2.7 cm.
49
50
Liver lesions diagnosed; admitted
4.5 × 2.1 × 3.9 cm
0.8 × 0.6 x 0.9 (CT)
Present Pip/tazo IV Ciprofloxacin IV
Event
Sub-capsular liver lesions
Left-sided liver lesion (cm)
Fever Anti-microbials
3
Drains removed
7
Itraconazole PO
Cloxacillin IV
Subcapsula- CGD diagnosed r drainage → MSSA
1
Methylpred day IV 14.6 66.6 53
2.1 × 1.9 × 0.4 cm 2.8 × 1.9 x 2.0 (US)
Steroids started
11
28
21.1 6.2 1
1 mg/kg/
2.4 × 0.9 x 1.2 (US)
3 mm thickness
Fevers gone x > 72h
35
Present
Almost completely resolved 2.7 × 1.4 x 1.7 (CT)
Re-admitted for fever
47
Left-sided lesion drainage → cultures negative
12
53
Discharged home
62
2.5 × 1.5 x 1.2 (US)
Re-solved
Prednisolone wean started
69
Cephalexin PO
IV cloxacillin changed to PO cephalexin
13
100
Trimethoprim-sulfamethoxazole PO Prednisolone 1.5 mg/kg/day PO divided BID Prednisolone ↓ by 20% per week
Discharged home on PO steroids
42
Cephalexin stopped
Steroids stopped
1.0 × 0.8 x 0.5 + calcifi-cations (US)
Re-solved
18
231
15
152
1.3 × 1 x 0.9 + calcifications (US)
Re-solved
Bone marrow transplant
23
410
BID = twice daily; CGD = chronic granulomatous disease; CT: computed tomography; IV = intravenous; Methylpred = methylprednisolone; MSSA = methicillin-sensitive Staphylococcus aureus; PO = per oral; Pip/ tazo = piperacillin-tazobactam; US: ultrasound.
WBC (x100/L) CRP (mg/L) ESR (mm/h)
24.3 204.4 93
10
Age (months)
Steroids
0
Days after diagnosis
Table 1 Timeline of Key Events in case of infant with chronic granulomatous disease and liver abscesses treated with corticosteroids and antibiotics.
J. Tam et al.
Journal of Pediatric Surgery Case Reports 35 (2018) 48–51
Journal of Pediatric Surgery Case Reports 35 (2018) 48–51
J. Tam et al.
Appendix A. Supplementary data
surgery, however, are high with a surgical complication rate of up to 56% described in a case series of patients aged 2–31 years old [3]. The most common complications included persistence of the abscess, wound dehiscence, pancreatitis, and pneumonia [2,3]. In our patient, surgery was not an optimal option given the anatomical position and ill-defined lesions. Our case also highlights that increasing size of intrahepatic lesions is not necessarily an indication of failure of medical treatment. The patient's clinical status, inflammatory markers, and lesion biopsy were helpful to determine that the lesion was fibrotic granulation tissue rather than an active infection. A concern of an increased risk of infection with glucocorticoid use was mitigated by directed treatment against the initial proven pathogenic organism (MSSA) and the use of bacterial and fungal prophylaxis with antimicrobials. Case reports and case series have described successful treatment of CGD liver abscesses with corticosteroids [2,5–8]. Although the corticosteroid doses varied amongst the patients, the average starting dose was methylprednisolone 1 mg/kg for 2–3 weeks with an average taper of 3 months [2]. Only one case series has included any infant. Straughan et al. [8] reported a retrospective case series at the National Institutes of Health (Bethesda, Maryland) of 26 patients from 1980 to 2014 with CGD and liver abscesses in which 8 patients were treated with only corticosteroids and antibiotics. The median age at initiation of corticosteroids was 19 years (range 0.5–46) with the youngest being 6 months at corticosteroid initiation. Our patient differs from this series in that he had partially successful percutaneous interventional radiologic drainage followed by high dose corticosteroid management. Furthermore, we describe that our patient underwent successful hematopoietic stem cell transplantation after the medical management of his abscess. Our case report contributes to the collective experience of using corticosteroids and antibiotics in the management of CGD-related liver abscesses in the infant age group.
Supplementary data related to this article can be found at http://dx. doi.org/10.1016/j.epsc.2018.06.001. Patient consent Written informed consent was obtained from the patient's family for this case report. Funding This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Authorship All authors attest that they meet the current ICMJE criteria for Authorship. Conflicts of interest Julia Upton is on the Medical Advisory Board for Immunodeficiency Canada (non-remunerated). None of the other authors have any conflicts of interest to declare. References [1] Holland SM. Chronic granulomatous disease. Hematol Oncol Clin N Am 2013;27:89–99. http://dx.doi.org/10.1016/j.hoc.2012.11.002. [2] Leiding JW, Freeman AF, Marciano BE, Anderson VL, Uzel G, Malech HL, et al. Corticosteroid therapy for liver abscess in chronic granulomatous disease. Clin Infect Dis 2012;54:694–700. http://dx.doi.org/10.1093/cid/cir896. [3] Lublin M, Bartlett DL, Danforth DN, Kauffman H, Gallin JI, Malech HL, et al. Hepatic abscess in patients with chronic granulomatous disease. Ann Surg 2002;235:383–91. http://dx.doi.org/10.1097/00000658-200203000-00010. [4] Chen LE, Minkes RK, Shackelford PG, Strasberg SM, Kuo EY, Langer JC. Cut it out: managing hepatic abscesses in patients with chronic granulomatous disease. J Pediatr Surg 2003;38:709–13. http://dx.doi.org/10.1016/jpsu.2003.50189. [5] Shin K-S, Lee MS. Concomitant use of corticosteroid and antimicrobials for liver abscesses in patients with chronic granulomatous disease. Korean J Pediatr 2016;59:196–201. http://dx.doi.org/10.3345/kjp.2016.59.4.196. [6] Yamazaki-Nakashimada MA, Stiehm ER, Pietropaolo-Cienfuegos D, HernandezBautista V, Espinosa-Rosales F. Corticosteroid therapy for refractory infections in chronic granulomatous disease: case reports and review of the literature. Ann Allergy Asthma Immunol 2006;97:257–61. http://dx.doi.org/10.1016/S1081-1206(10) 60023-3. [7] Tewari D, Tewari V, Siegel S, Berezin SH, Bostwick HE. Successful management of liver abscesses in a patient with chronic granulomatous disease (CGD) using corticosteroids. J Pediatr Surg Case Rep 2014;2:257–60. http://dx.doi.org/10.1016/j. epsc.2014.05.003. [8] Straughan DM, McLoughlin KC, Mullinax JE, Marciano BE, Freeman AF, Anderson VL, et al. The changing paradigm of management of liver abscesses in chronic granulomatous disease. Clin Infect Dis 2018;66:1427–34. http://dx.doi.org/10. 1093/cid/cix1012.
3. Conclusion In conclusion, this case report is to our knowledge the second report of safe and effective use of antibiotics and corticosteroids in the management of an infant with CGD and intrahepatic lesions who had a high surgical risk for hepatic resection. This case highlights the paradigm change away from open surgical procedures in the management of abscesses in patients with CGD. Corticosteroids plus antibiotics, in conjunction with close clinical and radiologic follow-up, may allow for resolution of liver abscesses without surgery in some infants. Acknowledgements We would like to thank the patient and his family for being an important part of this case report.
51
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Successful treatment of liver abscesses with corticosteroids and antibiotics in an infant with chronic granulomatous disease
T
Jennifer Tama, David Fahmyb, Jeffrey Traubicic, Jacob C. Langerd, Shaun K. Morrisa, Julia E.M. Uptone,∗ a
Division of Infectious Diseases, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada Division of Immunology and Allergy, Department of Medicine, McMaster University, Hamilton, Ontario, Canada c Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, University of Toronto, Ontario, Canada d Division of General and Thoracic Surgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada e Division of Immunology and Allergy, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada b
A R T I C LE I N FO
A B S T R A C T
Keywords: Chronic granulomatous disease Liver abscess Corticosteroids
Liver abscesses occur in up to one-third of patients with chronic granulomatous disease (CGD). Surgery has traditionally been considered the cornerstone of management. Emerging reports in older children and adults, however, have demonstrated successful treatment of CGD-related liver abscesses with corticosteroids and antibiotics alone. We describe the safe and effective use of corticosteroids and antibiotics for CGD-related liver abscesses in an infant with X-linked CGD who had high surgical risk for hepatic resection. Medical management was started at 11 months of age and the abscesses resolved without requiring any subsequent invasive surgical procedures. This case report highlights that corticosteroids and antibiotics may be considered in the management of CGD-related liver abscesses in the infant age group, especially when surgical management is not an option.
1. Introduction Liver abscesses occur in up to 35% of patients with chronic granulomatous disease (CGD), with Staphylococcus aureus being the most frequently cultured pathogen [1,2]. The management of liver abscesses in CGD patients is not well-defined, but surgery has been a cornerstone of therapy [3,4]. A small number of reports in older children and young adults have described successful treatment of CGD-related liver abscesses with corticosteroids [2,5–7], with a more recent case series describing this approach as a “changing paradigm of management of liver abscesses” in CGD [8]. We report a case of an infant with X-linked CGD and a liver abscess successfully treated with corticosteroid and targeted antibiotic therapy without surgical resection. Written informed consent was obtained from the patient's family for this case report. 1.1. Case report A term male infant with a longstanding history of fevers and lymphadenitis developed abdominal distension and right upper quadrant
pain at 10 months of age. Abdominal ultrasound demonstrated a 5.0 × 1.8 × 2.0 cm hepatic subcapsular complex collection. He was empirically started on parenteral piperacillin-tazobactam and ciprofloxacin. CT abdomen (Fig. 1) confirmed a large subcapsular abscess (4.5 × 2.1 × 3.9 cm) on the right side of the liver anteriorly along segment V with extension into the hepatic parenchyma (Fig. 1A), as well as a second ill-defined lesion (0.8 × 0.6 × 0.9 cm) on the left side of the liver anteriorly along the falciform ligament (Fig. 1B). The subcapsular abscess was drained via image-guided therapy the following day, yielding 8 mL of pus, which grew methicillin-sensitive Staphylococcus aureus (MSSA). Piperacillin-tazobactam was changed to parenteral cloxacillin (50 mg/kg every 6 h) and ciprofloxacin was continued for one week while a percutaneous drain remained in situ. Neutrophil oxidative burst index was low on two occasions (9 and 1.1, with normal range 32–300). A clinical diagnosis of CGD was made, later confirmed to be due to a hemizygous nonsense mutation in the CYBB gene ((c.931C > T, p.Gln 311, Denver Genetic Laboratories)), which was classified as pathogenic. At 4 weeks of admission (11 months of age), the patient continued to have intermittent fever, abdominal pain, and elevated inflammatory
∗
Corresponding author. The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. E-mail addresses: [email protected] (J. Tam), [email protected] (D. Fahmy), jeff[email protected] (J. Traubici), [email protected] (J.C. Langer), [email protected] (S.K. Morris), [email protected] (J.E.M. Upton). https://doi.org/10.1016/j.epsc.2018.06.001 Received 30 May 2018; Accepted 2 June 2018 Available online 04 June 2018 2213-5766/ © 2018 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/).
Journal of Pediatric Surgery Case Reports 35 (2018) 48–51
J. Tam et al.
Abbreviations CGD cm CRP CT CYBB ESR
h IV kg L mg mL MSSA
chronic granulomatous disease centimetre C-reactive protein computed tomography cytochrome b-235 beta chain (also known as p91-phox) erythrocyte sedimentation rate
hour intravenous kilogram litre milligram millilitre methicillin-sensitive Staphylococcus aureus
prophylactic itraconazole. Prophylactic trimethoprim-sulfamethoxazole was also initiated. Serial abdominal ultrasound imaging continued to be reassuring with gradually decreasing size of left hepatic lesions with increasing internal calcifications. The right subcapsular abscess did not re-accumulate and no new hepatic lesions occurred. Prednisolone wean started 6 weeks after initiation by approximately 20% weekly. IV cloxacillin was changed to oral cephalexin 150 mg/kg/day divided three times daily at just over 3 months of antibiotic therapy, and continued for an additional 4 months until abdominal imaging only showed small clusters of tiny calcifications. At 2 years of age, the patient underwent bone marrow transplantation for CGD. At over one year post-transplant, there was no evidence of recurrence of liver abscesses. His clinical course is summarized (Table 1).
markers (CRP 66.6 mg/L and ESR 53 mm/h) despite continuing IV cloxacillin. While the right subcapsular abscess had decreased in size post-drainage to 2.1 × 1.9 × 0.4 cm, the lesion in the left hepatic lobe had increased to 1.3 × 1.7 × 1.3 cm with a 2.8 × 1.9 × 2 cm area of surrounding hypoechoic halo. Surgical resection of the abscess was considered; however, based on the location of the lesions and the otherwise stable clinical status, the procedure was deemed to be high risk. Therefore, medical management was attempted with methylprednisolone intravenously (IV) (1 mg/kg/day), while continuing IV cloxacillin. His fevers abated following initiation of corticosteroids. Within one week, his inflammatory markers normalized and abdominal ultrasound demonstrated the right subcapsular abscess had decreased to 3 mm thickness and the left hepatic lobe lesion had decreased to 2.4 × 0.9 × 1.2 cm. Following 2 weeks of IV methylprednisolone, he was discharged home on oral prednisolone (1.5 mg/kg/day divided twice daily), IV cloxacillin, and itraconazole for fungal prophylaxis. Five days after discharge, the patient developed new fever. A CT abdomen showed the right subcapsular liver abscess had almost completely resolved (Fig. 1-2A). However, the left hepatic lesion increased to 1.4 × 1.7 × 2.7 cm (Fig. 1-2B); a biopsy showed only sterile fibrotic granulation tissue. Blood cultures remained negative and he was discharged home to continue oral prednisolone, IV cloxacillin and
2. Discussion We report a case description of an infant with CGD successfully treated for a liver abscess with antibiotic and corticosteroid therapy, thereby avoiding surgical resection. The management of liver abscesses in CGD patients has often included hepatic resection, due to disappointing results from only drainage of the abscess [4]. The risks of Fig. 1. Axial contrast enhanced CT images initially (1) and two months later (2). 1A. A heterogeneous sub-capsular liver lesion (thick white arrow) along segment V demonstrates a low attenuation region with peripheral rim enhancement concerning for an abscess with surrounding phlegmon. It appears to involve the underlying liver parenchyma and likely the adjacent abdominal wall. 1B. A smaller 0.8 × 0.6 × 0.9 cm hypoattenuating lesion (thin white arrow) is seen adjacent to the falciform ligament. 2A. The sub-capsular lesion resolved (thick white arrow) and attenuation changes are improved. 2B. The lesion increased in size (thin white arrow), measuring 1.4 × 1.7 × 2.7 cm.
49
50
Liver lesions diagnosed; admitted
4.5 × 2.1 × 3.9 cm
0.8 × 0.6 x 0.9 (CT)
Present Pip/tazo IV Ciprofloxacin IV
Event
Sub-capsular liver lesions
Left-sided liver lesion (cm)
Fever Anti-microbials
3
Drains removed
7
Itraconazole PO
Cloxacillin IV
Subcapsula- CGD diagnosed r drainage → MSSA
1
Methylpred day IV 14.6 66.6 53
2.1 × 1.9 × 0.4 cm 2.8 × 1.9 x 2.0 (US)
Steroids started
11
28
21.1 6.2 1
1 mg/kg/
2.4 × 0.9 x 1.2 (US)
3 mm thickness
Fevers gone x > 72h
35
Present
Almost completely resolved 2.7 × 1.4 x 1.7 (CT)
Re-admitted for fever
47
Left-sided lesion drainage → cultures negative
12
53
Discharged home
62
2.5 × 1.5 x 1.2 (US)
Re-solved
Prednisolone wean started
69
Cephalexin PO
IV cloxacillin changed to PO cephalexin
13
100
Trimethoprim-sulfamethoxazole PO Prednisolone 1.5 mg/kg/day PO divided BID Prednisolone ↓ by 20% per week
Discharged home on PO steroids
42
Cephalexin stopped
Steroids stopped
1.0 × 0.8 x 0.5 + calcifi-cations (US)
Re-solved
18
231
15
152
1.3 × 1 x 0.9 + calcifications (US)
Re-solved
Bone marrow transplant
23
410
BID = twice daily; CGD = chronic granulomatous disease; CT: computed tomography; IV = intravenous; Methylpred = methylprednisolone; MSSA = methicillin-sensitive Staphylococcus aureus; PO = per oral; Pip/ tazo = piperacillin-tazobactam; US: ultrasound.
WBC (x100/L) CRP (mg/L) ESR (mm/h)
24.3 204.4 93
10
Age (months)
Steroids
0
Days after diagnosis
Table 1 Timeline of Key Events in case of infant with chronic granulomatous disease and liver abscesses treated with corticosteroids and antibiotics.
J. Tam et al.
Journal of Pediatric Surgery Case Reports 35 (2018) 48–51
Journal of Pediatric Surgery Case Reports 35 (2018) 48–51
J. Tam et al.
Appendix A. Supplementary data
surgery, however, are high with a surgical complication rate of up to 56% described in a case series of patients aged 2–31 years old [3]. The most common complications included persistence of the abscess, wound dehiscence, pancreatitis, and pneumonia [2,3]. In our patient, surgery was not an optimal option given the anatomical position and ill-defined lesions. Our case also highlights that increasing size of intrahepatic lesions is not necessarily an indication of failure of medical treatment. The patient's clinical status, inflammatory markers, and lesion biopsy were helpful to determine that the lesion was fibrotic granulation tissue rather than an active infection. A concern of an increased risk of infection with glucocorticoid use was mitigated by directed treatment against the initial proven pathogenic organism (MSSA) and the use of bacterial and fungal prophylaxis with antimicrobials. Case reports and case series have described successful treatment of CGD liver abscesses with corticosteroids [2,5–8]. Although the corticosteroid doses varied amongst the patients, the average starting dose was methylprednisolone 1 mg/kg for 2–3 weeks with an average taper of 3 months [2]. Only one case series has included any infant. Straughan et al. [8] reported a retrospective case series at the National Institutes of Health (Bethesda, Maryland) of 26 patients from 1980 to 2014 with CGD and liver abscesses in which 8 patients were treated with only corticosteroids and antibiotics. The median age at initiation of corticosteroids was 19 years (range 0.5–46) with the youngest being 6 months at corticosteroid initiation. Our patient differs from this series in that he had partially successful percutaneous interventional radiologic drainage followed by high dose corticosteroid management. Furthermore, we describe that our patient underwent successful hematopoietic stem cell transplantation after the medical management of his abscess. Our case report contributes to the collective experience of using corticosteroids and antibiotics in the management of CGD-related liver abscesses in the infant age group.
Supplementary data related to this article can be found at http://dx. doi.org/10.1016/j.epsc.2018.06.001. Patient consent Written informed consent was obtained from the patient's family for this case report. Funding This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Authorship All authors attest that they meet the current ICMJE criteria for Authorship. Conflicts of interest Julia Upton is on the Medical Advisory Board for Immunodeficiency Canada (non-remunerated). None of the other authors have any conflicts of interest to declare. References [1] Holland SM. Chronic granulomatous disease. Hematol Oncol Clin N Am 2013;27:89–99. http://dx.doi.org/10.1016/j.hoc.2012.11.002. [2] Leiding JW, Freeman AF, Marciano BE, Anderson VL, Uzel G, Malech HL, et al. Corticosteroid therapy for liver abscess in chronic granulomatous disease. Clin Infect Dis 2012;54:694–700. http://dx.doi.org/10.1093/cid/cir896. [3] Lublin M, Bartlett DL, Danforth DN, Kauffman H, Gallin JI, Malech HL, et al. Hepatic abscess in patients with chronic granulomatous disease. Ann Surg 2002;235:383–91. http://dx.doi.org/10.1097/00000658-200203000-00010. [4] Chen LE, Minkes RK, Shackelford PG, Strasberg SM, Kuo EY, Langer JC. Cut it out: managing hepatic abscesses in patients with chronic granulomatous disease. J Pediatr Surg 2003;38:709–13. http://dx.doi.org/10.1016/jpsu.2003.50189. [5] Shin K-S, Lee MS. Concomitant use of corticosteroid and antimicrobials for liver abscesses in patients with chronic granulomatous disease. Korean J Pediatr 2016;59:196–201. http://dx.doi.org/10.3345/kjp.2016.59.4.196. [6] Yamazaki-Nakashimada MA, Stiehm ER, Pietropaolo-Cienfuegos D, HernandezBautista V, Espinosa-Rosales F. Corticosteroid therapy for refractory infections in chronic granulomatous disease: case reports and review of the literature. Ann Allergy Asthma Immunol 2006;97:257–61. http://dx.doi.org/10.1016/S1081-1206(10) 60023-3. [7] Tewari D, Tewari V, Siegel S, Berezin SH, Bostwick HE. Successful management of liver abscesses in a patient with chronic granulomatous disease (CGD) using corticosteroids. J Pediatr Surg Case Rep 2014;2:257–60. http://dx.doi.org/10.1016/j. epsc.2014.05.003. [8] Straughan DM, McLoughlin KC, Mullinax JE, Marciano BE, Freeman AF, Anderson VL, et al. The changing paradigm of management of liver abscesses in chronic granulomatous disease. Clin Infect Dis 2018;66:1427–34. http://dx.doi.org/10. 1093/cid/cix1012.
3. Conclusion In conclusion, this case report is to our knowledge the second report of safe and effective use of antibiotics and corticosteroids in the management of an infant with CGD and intrahepatic lesions who had a high surgical risk for hepatic resection. This case highlights the paradigm change away from open surgical procedures in the management of abscesses in patients with CGD. Corticosteroids plus antibiotics, in conjunction with close clinical and radiologic follow-up, may allow for resolution of liver abscesses without surgery in some infants. Acknowledgements We would like to thank the patient and his family for being an important part of this case report.
51