Infection-Related Glomerulonephritis Revealed as Shunt Nephritis: A Case Report

Diffuse glomerulonephritis with the nephrotic syndrome was observed in two patients aged 3 years 8 months and 27 years, both of whom had ventriculo-atrial shunts. Removal of the shunts resulted in marked improvement in the first and complete recovery in the second patient. Serum β1C-globulin concentrations were initially low and returned to normal values. Cultures taken from cerebrospinal fluid and valve grew Staphylococcus albus in the pediatric patient and were negative in the adult patient. Initial renal biopsies obtained immediately before shunt removal showed diffuse intra- and extracapillary glomerulonephritis type III (crescent formation in over 90 percent of the glomeruli) in the first patient, and diffuse intracapillary proliferative and exudative glomerulonephritis in the second patient. Repeat biopsies were performed, on the first patient 22 months, and on the second patient 7 months after shunt removal. In the first patient, half of the glomeruli were sclerosed, and the others exhibited slight mesangial proliferation with some capsular adhesions. The repeat renal biopsy from the adult patient showed mesangial proliferation with lobulation. Immunofluorescence studies in both patients revealed positive staining in a coarsely granular pattern against IgG, IgM, and β1C globulin. On electron microscopy, subendothelial deposits were still visible in the second biopsies of both patients. Glomerulonephritis associated with infected ventriculo-atrial shunt is probably mediated by antigen-antibody complexes. Recovery is possible in spite of severe renal lesions since the antigen can be eliminated at once by removal of the shunt.

Shunt nephritis is an immune-complex-mediated glomerulonephritis (GN) associated with chronically infected ventriculoatrial shunts inserted for treatment of hydrocephalus. Six patients aged 5-22 years with shunt nephritis are reported who have been observed between 1971 and 1994. The clinical course and long-term outcome are analysed in relation to the time of diagnosis and renal histopathology. The time of diagnosis of shunt nephritis ranged from 0.3 to 4.5 years after the last shunt operation. Diagnosis was delayed up to 1.5 years after the first clinical manifestations. All patients had signs of infection, i.e. recurrent fever, hepatosplenomegaly, anaemia, and cerebral symptoms. Renal manifestations consisted of haematuria (macroscopic in 3 patients), proteinuria (heavy in 5), renal insufficiency (4) and hypertension (2). Decreased C3 levels, cryoglobulins, and antinuclear factors were frequent. Cultures of blood and cerebrospinal fluid provided growth mainly of S. epidermidis. Renal biopsy revealed endocapillary GN (1), membranoproliferative GN (1) and endocapillary/extracapillary GN with crescents (2). All patients received antibiotics i.v. Complete recovery was observed in three of four patients in whom the shunt was totally removed, supported by transient external drainage of cerebrospinal fluid, and followed by placement of a ventriculoperitoneal shunt. One child with delayed diagnosis, presenting with a serum creatinine of 3.2 mg/dl, hypertension, and severe scarring on renal biopsy, rapidly progressed to irreversible ESRD within 5 months. Two patients without and only partial removal of the shunt died subsequently from sepsis. The renal outcome of shunt nephritis is good if early diagnosis and treatment is provided including i.v. antibiotics and total removal of the infected shunt. The possible progression to ESRD requires frequent nephrological monitoring of patients with ventriculoatrial shunts.

Timing of ventriculoatrial shunt removal on renal function recovery of patients with shunt nephritis: Case report and systematic review

Shunt nephritis is a rare complication of ventriculoatrial and ventriculoperitoneal shunt infection. The clinical manifestations are nonspecific and include asthenia, arthralgias, anorexia, weight loss, hematuria, proteinuria, and progressive renal impairment. Therefore, diagnosis can be delayed up for to several years after the first clinical manifestations, which increases the risk of progressive renal impairment and neurological dysfunction. A 57-year-old woman who had undergone ventriculoperitoneal shunt placement in 1990 was admitted to the nephrology department to perform an elective kidney biopsy due to hematuria, proteinuria, and rapidly progressive renal failure. The patient presented with asthenia and weight loss with a duration of one year. No other symptoms were reported. The kidney biopsy was suggestive of infection associated glomerulonephritis, particularly a subacute infection, as a membranoproliferative pattern was seen in light microscopy, and IgM staining was predominant in immunofluorescence imaging. During the hospital stay, the patient developed neurological symptoms. Cerebrospinal fluid examination showed pleocytosis. Shunt nephritis was suspected, and antibiotic treatment was initiated. Due to the patient’s worsening neurological status, an urgent surgical shunt removal was performed. Two months later, her renal function was normal, and the urinalysis result was unremarkable, even though her neurological status did not improve. Although rare, shunt nephritis should be considered in patients that have a history of ventriculoperitoneal shunt placement and present with a proliferative or membranoproliferative glomerulonephritis and prominent IgM deposits in immunofluorescence imaging. Early diagnosis and shunt removal are critical for improving the neurological and renal prognosis.

Transient arthritis with positive tests for rheumatoid factor as presenting sign of shunt nephritis.

weeks. A neurosurgical consultant refused to accept Case report our recommendation of shunt removal as he was not convinced of the diagnosis. A week after the initiation In August 1995, an 18-year-old youth was initially of antibiotic therapy, 3 and 4 levels increased to 205 referred for the evaluation of microscopic haematuria and 11 mg/dl, respectively. However, a fortnight foldiscovered on a routine army pre-recruitment urinlowing the cessation of antibiotics, levels decreased to alysis. At the age of 8, he had undergone radiotherapy pretreatment values. A repeat CSF culture was sterile. for a brain tumour. Hydrocephalus was managed by The above antibiotic regimen was recommended folthe insertion of a ventriculoatrial (VA) shunt. On lowing which, a month later, our neurosurgeons were, presentation, physical examination was unremarkable finally, persuaded into removing the VA shunt, replawith a body temperature of 37.1°C and blood pressure cing it with a ventriculoperitoneal (VP) shunt. Culture 122/72 mmHg. At this stage, the only notable laboratof the tip of the removed shunt produced a growth of ory data were a mild leucocytosis (WBC 13 200/mm3), coagulase negative staphylococci, sensitive to both a decreased C4 level of 11 mg/dl (normal>16 mg/dl) vancomycin and rifampicin. Two weeks after shunt and urinalysis that showed numerous RBC/HPF with replacement C3 and C4 levels were 230 and 37 mg/dl, no casts or proteinuria. C3 level was 130 (normal> respectively, and have since remained in the normal 90 mg/dl ). Antistreptolysin titre, rheumatoid factor range. Currently (1 month post-surgery), microscopic and cryoglobulins were all negative. Repeated blood haematuria and proteinuria persist. cultures yielded no growth. Ultrasound showed two kidneys of normal size and echogenicity. In April 1996, proteinuria ranging from 1.4–2.8 g/day developed. 3 Comment level had decreased to 54 mg/dl, and 4 to 6 mg/dl. Serum creatinine was unchanged at 1.0 mg/dl while The introduction of shunting procedures for the treathaemoglobin had dropped from 13.4 to 10.8 g/dl. ment of hydrocephalus has led to the wide application Clinically, the patient felt well, was afebrile with no of this device in clinical practice. Shunt infection has, elevation of blood pressure. however, proved to be a troublesome complication. It Percutaneous renal biopsy was performed. On light usually presents with fever, almost a universal occurmicroscopy, accentuated lobular structure of the glomrence, often with accompanying signs of raised intraeruli with mesangial cell proliferation and thickening cranial pressure [1]. In general, either blood or CSF of the glomerular basement membrane (GBM ) was cultures are positive. Whereas, the incidence of shunt shown (Fig. 1). Immunoperoxidase demonstrated infection may range as high as 27% [1], the incidence intense staining for IgM, IgG and 3 in a granular of glomerulonephritis associated with shunt infection pattern along the GBM. Electron microscopy revealed approximates only 3% of cases [2]. This last entity, the presence of subendothelial and mesangial electron now known as shunt nephritis, was first described in dense deposits with duplication of the GBM (Fig. 2). 1965 by Black et al. [3 ] who reported two cases of the A spinal tap yielded a normal, culture negative cerebronephrotic syndrome and macroscopic haematuria assospinal fluid (CSF). Blood cultures were persistently ciated with Staphylococcus albus bacteraemia after sterile. Despite this, presenting a working diagnosis of shunt insertion. Since then, more than 80 cases have shunt nephritis, intravenous vancomycin (2 g/day) and been reported in the literature. The mean interval oral rifampicin (600 mg/day) were administered for 6 between shunt insertion and nephritis is 4.4 years, but, as in our case, much longer intervals of 10 to 14 years Correspondence and o print requests to: J. Bernheim, Department of Nephrology, Meir Hospital, Kfar-Saba, Israel. have been documented [2].

The management of hydrocephalus represents a neurosurgical challenge. Ventriculoperitoneal (VP) shunts are usually the treatment of choice for hydrocephalus. However, when VP shunt is not an option, ventriculoatrial (VA) shunt becomes a second choice. VA shunts have special complications such as postoperative neck hematomas, shunt nephritis, and migration of the distal segment. There are two main techniques for the retrieval of migrated VA shunt: either by retrieval of the broken segment by thoracotomy, which is highly invasive, or by transvenous approach. A 9-year-old boy presented with severe cerebral palsy, who was VP shunt dependent since birth. The patient developed generalized cerebrospinal fluid (CSF) ascites with peritonitis. The shunt was externalized and infection was treated with intravenous antibiotics. Two weeks later, and due to previous multiple abdominal procedures, it was decided to go for VA shunt and the procedure went well without complications. One month later, the patient presented to emergency department with neck swelling; shunt x-ray showed shunt breakage at clavicular level and the tip of the shunt was dislodged into the pulmonary artery. Under general anesthesia and after getting informed consent, through femoral vein, VA shunt was retrieved successfully without complications and new VP shunt was inserted. Migration of the distal segment of a broken atrial catheter is rare, but has a significant complication and is major cause of mortality. Endovascular retrieval of migrated shunts is less invasive, is feasible, and prevents further complications. There has been similar case in the adult English literature; however, to the best of our knowledge, this is the first reported case in the English literature of a successful endovascular retrieval of migrated dislodged VA shunt in pediatrics.

Ventriculoatrial Shunts in Adults, Incidence of Infection, and Significant Risk Factors: A Single-Center Experience

Nephritis associated with a chronically infected ventriculoatrial shunt is known as shunt nephritis. A 6-year-old girl with prior history of a ventriculoatrial shunt presented complaining of fever and gross hematuria. Serum complement levels were decreased and a coagulase-negative S. epidermidis was cultured from her blood. The renal biopsy specimen showed features of membranoproliferative glomerulonephritis type I. Hydrocephalus was so severe that shunt removal was impossible. With antibiotic therapy, clinical symptoms and laboratory findings include complement levels were normalized although microscopic hematuria persisted. To our knowledge, this is the first case of shunt nephritis in Korea. In addition to the case report, a brief review of shunt nephritis has been added.

Shunt nephritis with positive titers for ANCA specific for proteinase 3

Of 5 children with glomerulonephritis with infected ventriculoatrial shunt, 3 had improved renal function after antibiotic therapy and removal of the infected shunt. 1 patient with endoextracapillary proliferative glomerulonephritis with 70% glomerular crescents developed a rapidly progressive renal insufficiency. Renal failure was successfully managed by hemodialysis and kidney transplantation. 1 patient died from extrarenal causes in the course of a septic episode.

Fourteen years after shunt implantation, a 26-year-old patient with myelomeningocele, concomitant hydrocephalus and a ventriculoatrial cerebrospinal fluid (CSF) shunt presented with brief but recurrent episodes of fever predominantly when taking...

Long-Term Outcomes in Ventriculoatrial Shunt Surgery in Patients with Pediatric Hydrocephalus: Retrospective Single-Center Study

Preface

Contamination of a ventriculoatrial shunt (VAS) with skin organisms that are usually nonpathogenic may be followed by an immunologically mediated renal injury. The bacteria characteristically involved are coagulase-negative Staphylococci (e.g., Staphylococcus epidermidis), which strongly adhere to the plastic surface of the VAS. These bacteria are protected from the body's natural defense mechanisms and respond only poorly to antibiotics. As a result, their growth persists and produces a continuous antigenic stimulation. Circulating immune complexes (CIC) are an appropriate tool to screen for chronically infected VASs. We followed CIC in 138 VAS patients. An infected VAS was seen in 20 of the 24 patients with highly elevated CIC and in 1 of the 19 patients with moderately elevated CIC, but none of the 95 patients with normal CIC had evidence of shunt infection. Of the 21 patients with shunt infections, 8 had renal involvement (4 requiring dialysis, and 4 with proteinuria, hematuria, and/or elevated creatinine). Results from kidney biopsy specimens available from 4 patients confirmed glomerulonephritis. Of the 4 patients requiring dialysis at diagnosis, renal function recovered sufficiently to stop dialysis after successful VAS exchange in all but 1. In the other 4 patients, renal symptoms (proteinuria, creatinine) also improved after VAS revision. Chronic infection with S. epidermidis or other bacteria is a continuing problem in patients with VASs and can lead to an immune-mediated renal injury. However, the prognosis for reversal of the renal injury is relatively good if the VAS infection is treated promptly.