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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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Year : 2021  |  Volume : 26  |  Issue : 5  |  Page : 338-341

Isolated renal mucormycosis in children: A case report and review of the literature

1 Department of Paediatric Surgery, All India Institute of Medical Sciences, Delhi, India
2 Department of Paediatrics, All India Institute of Medical Sciences, Delhi, India
3 Department of Pathology, All India Institute of Medical Sciences, Delhi, India
4 Department of Radiodiagnosis, All India Institute of Medical Sciences, Delhi, India

Date of Submission07-May-2020
Date of Decision30-Jul-2020
Date of Acceptance14-May-2021
Date of Web Publication16-Sep-2021

Correspondence Address:
Dr. Anjan Kumar Dhua
Room No. 4002, Department of Pediatric Surgery, Teaching Block, AIIMS, Ansari Nagar, Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_142_20

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Isolated renal mucormycosis is rare in children. A case of 3-month-old infant presenting with right-sided flank lump and fever is being reported. Investigations revealed a nonfunctioning right kidney with intrarenal abscesses and a low percent-frequency of T-cells. He made an uneventful recovery following surgery and systemic antifungal therapy. Compared to the other forms of deep mucormycosis, a review of literature suggests favorable prognosis in children with isolated renal involvement.

Keywords: Amphotericin B, antifungal, renal mucormycosis

How to cite this article:
Anand S, Kainth D, Dhua AK, Sehgal M, Bajpai M, Das P, Kandasamy D. Isolated renal mucormycosis in children: A case report and review of the literature. J Indian Assoc Pediatr Surg 2021;26:338-41

How to cite this URL:
Anand S, Kainth D, Dhua AK, Sehgal M, Bajpai M, Das P, Kandasamy D. Isolated renal mucormycosis in children: A case report and review of the literature. J Indian Assoc Pediatr Surg [serial online] 2021 [cited 2023 Jun 2];26:338-41. Available from: https://www.jiaps.com/text.asp?2021/26/5/338/326058

   Introduction Top

Renal mucormycosis usually occurs as a part of disseminated fungal infection in immunocompromised patients. Isolated renal involvement is sporadic in children and can present as a spectrum of clinical features ranging from nonspecific abdominal pain to anuria. Histopathological diagnosis is the gold standard. Early surgical treatment combined with systemic antifungal therapy remains the mainstay of treatment. Herein, we report a case of isolated renal mucormycosis in a 3-month-old boy with low percent-frequency of T-cells. The case was successfully managed by surgical evacuation of necrotic tissue occupying the right renal fossa, followed by systemic antifungal therapy (amphotericin-B).

   Case Report Top

The index case is a 3-month-old boy who was born by vaginal delivery at term. He had meconium aspiration at birth and was kept in neonatal intensive care unit (NICU) for a month. During the NICU stay, he required mechanical ventilation for 1 week but had no features of multiorgan dysfunction. He made an uneventful recovery and was discharged at 1 month of age. Subsequently, he had a stable course at home until 2 weeks back when he was brought to outpatient department with a right-sided flank lump and fever.

Upon examination, he was febrile (102°F) with tachycardia. There was a 5 cm × 6 cm fluctuant swelling in the right lumbar region. Ultrasound revealed a collection in the right renal fossa with internal echoes. The right kidney was not visualized. Subsequently, a contrast-enhanced computed tomography scan of the abdomen revealed homogeneous fluid-attenuating lesion with a peripheral enhancing wall [Figure 1]a and [Figure 1]b entirely replacing the right kidney (thin arrow). Left kidney (outlined arrow) and rest of the abdominal organs were normal.
Figure 1: (a) Contrast enhanced computed tomography scan of the child showing a homogeneous fluid attenuating lesion (b) Peripheral enhancing wall. The lesion completely replaces the right kidney (thin arrow in a and b). Left kidney is normal (outlined arrow)

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A clinicoradiological diagnosis of right nonfunctioning kidney with intrarenal abscess extending into perirenal space was made, and the child was taken for surgery. Upon exploration through the lumbotomy approach, the entire renal parenchyma was replaced by necrotic tissue and pus. The pus was drained, and necrotic tissue was removed. Histopathologic examination revealed necrotic tissue [Figure 2]a along with numerous fungal hyphae with obtuse-angled branching [Figure 2]b and angioinvasion. These features were consistent with renal mucormycosis. After 10 weeks of the operation, a 99mTc-dimercaptosuccinic acid scan (DMSA) showed no functioning renal tissue in the right renal fossa [Figure 3]. The child received amphotericin-B for 4 weeks and had an uneventful recovery. His immunological workup revealed low percent-frequencies of T (CD3+) cells. However, the percent frequency of B (CD19+/CD20+) cells was within the normal range. Similarly, a normal percent frequency of granulocytes expressing leukocyte adhesion markers and a normal neutrophil oxidative index ruled out leukocyte adhesion disorder and chronic granulomatous disease. After a year of follow-up, the child is asymptomatic and gaining appropriate milestones.
Figure 2: (a) Photomicrograph showing extensive necrosis including the blood vessels (H and E, ×40). (b) It shows angioinvasion by broad, aseptate fungal hyphae (thin arrow) with right-angle branching (H and E, ×100)

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Figure 3: 99mTc-dimercaptosuccinic acid scan scan shows no evidence of any functional renal tissue in the right renal fossa

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   Discussion Top

Fungi belonging to zygomycetes class are opportunistic organisms having the propensity to cause a fatal infection in immunocompromised states.[1],[2] Within this taxonomic class lies Mucorales order, which encompasses fungi causing mucormycosis.[1] Common forms of mucormycosis include rhinocerebral, pulmonary, gastrointestinal, and disseminated. Around 20% of cases with a disseminated infection have renal mucormycosis.[3]

Isolated renal involvement [Table 1] is rarely seen in children. On literature review, only 13 cases (from 10 reports) within the pediatric age group (14 years or younger) had isolated renal mucormycosis. Out of these, five (38%) had bilateral involvement. Furthermore, only two children had underlying risk factors – one with aplastic anemia and other with low natural killer cell activity.[6],[8] One child had undergone sequential bilateral pyeloplasty for bilateral pelviureteric junction obstruction.[3] Similarly, in a literature review done by Dhua et al. in the pediatric population, the presence of an underlying risk was uncommon.[3] Mathew et al. have suggested some unique property of the immune system in children might have contributed to this occurrence;[2] however, this hypothesis needs further evaluation before definite conclusions are drawn. Majority of the children presented with fever (11/13; 85%) and flank or abdominal pain (9/13; 69%). Flank mass and urinary symptoms (hematuria, pyuria, etc.) were seen in 15% (2/13) and 54% (7/13) of cases, respectively. Apart from these, four children (31%) had features of acute renal failure at the time of presentation. All four were later found to have bilateral involvement. It has been shown that angio-invasion by the fungal hyphae causes occlusion of the renal arteries, leading to renal infarction and subsequent renal failure in bilateral disease.[1]
Table 1: Summary of reports in the literature on intrarenal mucormycosis in the paediatric age-group (14 years or younger)

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The radiological features of renal mucormycosis are characteristic including large nonenhancing kidneys with an absence of contrast excretion.[1] The presence of low-attenuation areas suggesting intrarenal abscesses and peri-ephric collections are also frequently seen in renal disease. However, in the index case, the entire right kidney was destroyed by the fulminant disease and was replaced by slough and purulent collection [Figure 1]. Histopathological features of the presence of broad (10–20 mm), irregularly branched, and rarely septate hyphae with angio-invasion are consistent with the diagnosis of mucormycosis.[1]

Early diagnosis and prompt treatment are the cornerstones in the management of these cases. Treatment includes thorough surgical drainage or nephrectomy and systemic antifungal therapy. Although surgical strategies remain the mainstay, it has been shown that systemic antifungal therapy alone can also treat these patients.[5] In this review, five children received systemic antifungal therapy alone. Out of these, only two recovered, highlighting the importance of surgical debulking.

The overall outcome of renal mucormycosis is poor, with mortality rates of above 50% described in the literature.[2] However, survival rates in isolated renal disease are around 65%.[3] Similar figures were observed in this review, with a mortality rate of around 38% (5/13). Four of them, who died, had a bilateral disease. Gupta et al. have also reported a poorer outcome in cases with bilateral involvement.[1] Of the five who died, two had risk factors, while the remaining three were managed by a nonsurgical approach only. Therefore, possible risk factors of mortality include bilateral disease, the presence of underlying immunocompromised state, and treatment with nonsurgical modalities.

   Conclusion Top

Isolated renal mucormycosis, an extremely rare entity in children, has a relatively favorable outcome as compared to other forms of mucormycosis. Timely diagnosis followed by surgical debulking combined with systemic antifungal therapy offers the best fate.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


   References Top

Gupta KL, Joshi K, Sud K, Kohli HS, Jha V, Radotra BD, et al. Renal zygomycosis: An under-diagnosed cause of acute renal failure. Nephrol Dial Transplant 1999;14:2720-5.  Back to cited text no. 1
Mathew G, Arumugam V, Murugesan S, Duhli N, Agarwal I. Renal Mucormycosis: A Rare Cause of Urinary Tract Infection Leading to End-stage Renal Disease (ESRD). J Trop Pediatr 2019;65:405-8.  Back to cited text no. 2
Dhua AK, Sinha S, Sarin YK, Khurana N. Isolated mucormycosis in a post-pyeloplasty kidney in an immuno-competent child. J Indian Assoc Pediatr Surg 2012;17:132-4.  Back to cited text no. 3
[PUBMED]  [Full text]  
Chakrabarti A, Das A, Sharma A, Panda N, Das S, Gupta KL, et al. Ten years' experience in zygomycosis at a tertiary care centre in India. J Infect 2001;42:261-6.  Back to cited text no. 4
Jianhong L, Xianliang H, Xuewu J. Isolated renal mucormycosis in children. J Urol 2004;171:387-8.  Back to cited text no. 5
Sharma R, Shivanand G, Kumar R, Prem S, Kandpal H, Das CJ, et al. Isolated renal mucormycosis: An unusual cause of acute renal infarction in a boy with aplastic anaemia. Br J Radiol 2006;79:e19-21.  Back to cited text no. 6
Sobti P, Rakheja G, Mittal A, Khurana N, Aggarwal S. Isolated renal mucormycosis in a pediatric patient. J Case Rep 2013;3:390-92.  Back to cited text no. 7
Sathe KP, Mehta KP. Irreversible fatal renal failure resulting from isolated renal mucormycosis. Saudi J Kidney Dis Transpl 2014;25:1312-4.  Back to cited text no. 8
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Nayagam LS, Vijayanand B, Balasubramanian S. Isolated renal mucormycosis in an immunocompetent child. Indian J Nephrol 2014;24:321-3.  Back to cited text no. 9
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Saran S, Naranje K, Gurjar M, Bhadauria D, Kaul A, Poddar B. Isolated Renal Mucormycosis in Immunocompetent Children: A Report of Two Cases. Indian J Crit Care Med 2017;21:457-9.  Back to cited text no. 10
[PUBMED]  [Full text]  


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]

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