|Year : 2015 | Volume
| Issue : 4 | Page : 271-274
Vertebral osteomyelitis and epidural abscess due to mucormycosis in a neonate with esophageal Atresia
Ankur Mandelia1, Rajan Garg1, Sandeep Agarwala1, Shashank Sharad Kale2
1 Department of Pediatric Surgery, All Institute of Medical Sciences, New Delhi, India
2 Department of Neurosurgery, All Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||16-Oct-2015|
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
Vertebral osteomyelitis due to mucormycosis is a rare but fulminant and fatal disease. Only three cases have been reported in adults with none reported in neonates till date. The present paper reports the first case of a neonate who developed vertebral osteomyelitis and epidural abscess due to mucormycosis following repair of esophageal atresia.
Keywords: Esophageal atresia, mucormycosis, neonate, vertebral osteomyelitis
|How to cite this article:|
Mandelia A, Garg R, Agarwala S, Kale SS. Vertebral osteomyelitis and epidural abscess due to mucormycosis in a neonate with esophageal Atresia. J Clin Neonatol 2015;4:271-4
|How to cite this URL:|
Mandelia A, Garg R, Agarwala S, Kale SS. Vertebral osteomyelitis and epidural abscess due to mucormycosis in a neonate with esophageal Atresia. J Clin Neonatol [serial online] 2015 [cited 2019 Sep 21];4:271-4. Available from: http://www.jcnonweb.com/text.asp?2015/4/4/271/161708
| Introduction|| |
Mucormycosis in neonates is rare, difficult to recognize, and hence, usually fatal. There is usually a delay in diagnosis and specific treatment. The presence of mucormycosis at rare sites, like the vertebrae, can be easily missed by a physician who is unaware of such a presentation. We report a case of a neonate with esophageal atresia (EA) who developed acute onset paraplegia resulting from vertebral osteomyelitis and epidural abscess due to mucormycosis.
| Case Report|| |
A 2-day-old, 2.6 kg female infant was referred to our institute as a case of EA with distal trachea-esophageal fistula (TEF). Chest radiograph revealed bilateral bronchopneumonia and echocardiography revealed atrial septal defect, ventricular septal defect, and patent ductus arteriosus with left to right flow. Plain radiograph frontal and lateral view [Figure 1]a and [Figure 1]b revealed segmentation anomaly involving multiple thoracic and lumbar vertebrae. Right posterolateral thoracotomy with the division of TEF and end-to-end anastomosis of esophageal ends was performed on the 3rd day of life. A contrast study on the 7th postoperative day revealed no anastomotic leak or hold up, and oral feeding was started. There was persistent bilateral bronchopneumonia, which was managed with intravenous antibiotics, chest physiotherapy, and saline nebulization. Postoperatively, the child also developed features of congestive cardiac failure and was managed with digoxin, furosemide, and fluid restriction.
|Figure 1: Frontal (a) and lateral (b) radiograph of the spine of the child reveals multiple vertebral segmentation anomaly involving the lower thoracic and lumbar vertebrae (arrows), (c) sagittal T1-weighted turbo spin-echo (TSE) fat-suppressed magnetic resonance image (MRI) after administration of gadolinium shows intraspinal extension of the lesion (arrows), (d) coronal T1-weighted TSE fat-suppressed MRI after administration of gadolinium shows the extraspinal lesion with intraspinal extension (arrows)|
Click here to view
The child developed bilateral lower limb weakness on the 12th postoperative day, which progressed to complete paraparesis over the next 3 days. Neurological examination revealed no spinal mass or tenderness. Both the lower limbs were flaccid and abducted. Muscle strength grading was 0/5 in both the lower limb muscles. A sensory examination revealed a complete loss of sensation below the level of the umbilicus. The patella and ankle reflexes were totally lost.
Magnetic resonance imaging (MRI) of the whole spine and pelvis revealed a large lesion involving the left sacroiliac joint, the lumbosacral spine, and adjacent iliac bone; having a large intraspinal component. The lesion had a central part, which showed hyperintense signal on T2-weighted images, isointensity to the adjacent muscles on T1-weighted images, and peripheral rim enhancement after administration of gadolinium [Figure 1]c and [Figure 1]d.
Based on the acute clinical presentation and imaging, surgical decompression of the spine was planned. Surgical exploration revealed large epidural abscess from D12-L3. The paraspinal muscles were edematous with areas of necrosis and calcification. There was a complete destruction of the D12-L1 disc and interspinal ligaments from D12-L3. Pus was also found inside the vertebral canal where the extradural fat became liquefied, and the dura was grayish black in color and friable. The spinal cord was not pulsatile. Purulent pus could also be seen within the laminae and spinous processes. Frozen section analysis of the tissue revealed mucormycosis. Samples from different locations were sent for bacterial and fungal culture and for pathologic analysis. Debridement of vertebral elements and affected disc tissue was carried out until the bleeding bone was encountered. The wound was irrigated and closed thereafter. Postoperatively, the child was started on intravenous amphotericin B and ceftriaxone. There was purulent wound discharge with no improvement in the neurological deficit in the lower limbs. The child developed signs of systemic sepsis, progressively deteriorated, and expired on the 8th postoperative day.
A microscopic examination of the histological section confirmed the presence of broad-based, aseptate branching fungal hyphae, morphologically consistent with mucormycosis [Figure 2]a and [Figure 2]b. Aerobic and anaerobic bacterial cultures were negative for pathogens. Cultures rapidly developed thick white mycelia forms that were identified as Rhizopus species.
|Figure 2: (a) H and E stained section at ×400 showing broad-based, aseptate branching fungal hyphae, (b) silver methenamine stain at ×400|
Click here to view
| Discussion|| |
Mucormycosis is the most acute, fulminant, and fatal of all fungal infections in humans. Mucormycosis, also known as phycomycosis or zygomycosis, is an infection caused by fungi of the class zygomycosis and order mucorales, usually Rhizopus, Absidia, Mortierella, and Mucor. They exist in the soil and air. Most of these pathogenic fungi require oxygen but are also capable of growth in anaerobic and microaerophilic conditions. Although the fungi show minimal intrinsic pathogenicity to normal persons, they can initiate aggressive and fulminant infections under certain clinical conditions. Most reported cases of mucormycosis in adults occurred in patients who were immunologically predisposed to infection. These conditions include hematologic malignancy, organ transplants, severe burns, end-stage renal disease or diabetes mellitus.,, The portals of entry in these cases were the nasopharynx or paranasal sinuses, with direct involvement of the orbit, meninges, and the brain. The predisposing conditions in children are prematurity, neutropenia, acidosis, and corticosteroid therapy. They could cause a diversity of pathologic clinical manifestation, including rhinocerebral, pulmonary, cutaneous, gastrointestinal, central nervous system disseminated, and miscellaneous syndromes.,,, Construction activity has been reported as an independent risk factor for invasive mucormycosis in a population of immunosuppressed patients. Hospitals caring for such patients should take precautions, which minimize exposure of these patients to construction or renovation activity.
Although mucormycosis has been reported in neonates, the disease is still considered rare in this population. Robertson et al. reviewed 31 cases of neonatal mucormycosis reported in literature till 1997. Of the 31 cases, 22 were premature infants with an average gestational age of 27 weeks and birth weight of 1154 g. In 12 of the premature infants, the original site was the skin, in 9 the gastrointestinal tract and in 1 the lungs. 16 of the premature infants died, almost equally distributed between the 2 major sites. In the 9 term infants, 4 of the cases involved the gastrointestinal tract, 4 the skin, and 1 the nasopharynx. Eight of the term infants died.
Vertebral osteomyelitis, spondylodiscitis, and epidural abscess due to mucormycosis have not been reported in neonates till date. A search of the literature from 1975 to 2011 in the English language revealed only three case reports in adults.,,
Buruma et al. reported about a patient with a history of pain in the neck, followed by a slow progressive loss of muscle strength in both the arms and finally resulted in tetraplegia; medical history included laryngectomy with partial hypopharyngectomy preceded by radiotherapy because of carcinoma. The patient died as a consequence of massive pulmonary embolism, and the autopsy showed the cause of the neurological deficit to be vertebral osteomyelitis and epidural abscess due to mucormycosis.
Von Pohle  reported the case of a 43-year-old male patient who presented with ascending paralysis, new onset diabetes mellitus, and a necrotizing pneumonia. The patient progressively deteriorated with the development of multiorgan dysfunction and ultimately died. At necropsy, disseminated phycomycosis-Rhizopus species with severe bilateral necrotizing cavitary pneumonia, bilateral pleural effusions, and direct extension of the left upper lobe abscess to the vertebral bodies at the T3-T4 level was noted. There was an associated purulent leptomeningitis of the spinal canal at levels T3-T8. At the T3 level, there was transverse necrosis of the spinal cord with Rhizopus organisms within feeding blood vessels.
Chen et al. reported a 57-year-old female case of mucormycosis spondylodiscitis and vertebral osteomyelitis after lumbar disc puncture and radio frequency nucleoplasty. She subsequently underwent two surgical debridements, continuous local irrigation and drainage, together with local and systemic amphotericin B treatments. The infection was controlled 4 months after the second debridement; however, there was no improvement in the neurological function.
The final diagnosis of mucormycosis is usually difficult to make before ischemia and necrosis are well advanced. The routine laboratory tests, X-ray, computed tomography, and MRI are all nonspecific. A definitive diagnosis requires either histological identification or growth of the organism on culture. Mucormycosis is characterized histologically by broad, irregularly shaped, nonseptate hyphae with right angle branching. Staining techniques include hematoxylin and eosin and periodic acid-Schiff. Speciation requires culture of the fungus and the advice of a mycologist.
Mucormycosis is notoriously difficult to treat. Experience with adults emphasizes the importance of surgical debridement and excision of the affected, necrotic tissue. Amphotericin B is generally used although resistance to the drug has been reported. In some cases, the use of a topical solution of amphotericin B may be helpful. Rifampin has been used with amphotericin B since in vitro synergy is reported. Ketoconazole was reported to be effective in one adult case.
The present case is thefirst reported case of vertebral osteomyelitis with an epidural abscess in neonates due to mucormycosis. The purpose of this report was to highlight a very rare presentation of this fulminant infection. Early diagnosis and an aggressive approach of combined medical and surgical treatment may improve the outcome of patients with this potentially lethal invasive disease.
Source of funding
Conflicts of interest
There are no conflicts of interest.
| References|| |
Lehrer RL, Howard DH, Sypherd PS. Mucormycosis. Ann Intern Med 1980;93:93-108.
Cocanour CS, Miller-Crotchett P, Reed RL 2nd
, Johnson PC, Fischer RP. Mucormycosis in trauma patients. J Trauma 1992;32:12-5.
Boelaert JR. Mucormycosis (zygomycosis): Is there news for the clinician? J Infect 1994;28 Suppl 1:1-6.
Ingram CW, Sennesh J, Cooper JN, Perfect JR. Disseminated zygomycosis: Report of four cases and review. Rev Infect Dis 1989;11:741-54.
Maertens J, Demuynck H, Verbeken EK, Zachée P, Verhoef GE, Vandenberghe P, et al.
Mucormycosis in allogeneic bone marrow transplant recipients: Report of five cases and review of the role of iron overload in the pathogenesis. Bone Marrow Transplant 1999;24:307-12.
Morduchowicz G, Shmueli D, Shapira Z, Cohen SL, Yussim A, Block CS, et al.
Rhinocerebral mucormycosis in renal transplant recipients: Report of three cases and review of the literature. Rev Infect Dis 1986;8:441-6.
Weems JJ Jr, Davis BJ, Tablan OC, Kaufman L, Martone WJ. Construction activity: An independent risk factor for invasive aspergillosis and zygomycosis in patients with hematologic malignancy. Infect Control 1987;8:71-5.
Robertson AF, Joshi VV, Ellison DA, Cedars JC. Zygomycosis in neonates. Pediatr Infect Dis J 1997;16:812-5.
Buruma OJ, Craane H, Kunst MW. Vertebral osteomyelitis and epidural abcess due to mucormycosis, a case report. Clin Neurol Neurosurg 1979;81:39-44.
Von Pohle WR. Disseminated mucormycosis presenting with lower extremity weakness. Eur Respir J 1996;9:1751-3.
Chen F, Lü G, Kang Y, Ma Z, Lu C, Wang B, et al.
Mucormycosis spondylodiscitis after lumbar disc puncture. Eur Spine J 2006;15:370-6.
Parfrey NA. Improved diagnosis and prognosis of mucormycosis. A clinicopathologic study of 33 cases. Medicine (Baltimore) 1986;65:113-23.
Barnert J, Behr W, Reich H. An amphotericin B-resistant case of rhinocerebral mucor mycosis. Infection 1985;13:134-6.
Christenson JC, Shalit I, Welch DF, Guruswamy A, Marks MI. Synergistic action of amphotericin B and rifampin against Rhizopus
species. Antimicrob Agents Chemother 1987;31:1775-8.
[Figure 1], [Figure 2]