Shunt nephritis with crescent formation

The case of a 17-year-old girl is reported, who presented shunt nephritis, 8 years after the ventriculo-atrial shunt insertion was made. Renal biopsy showed membranoproliferative glomerulonephritis. The symptoms of uremia mingled with those of chronic septic state. Persistent hypocomplementemia and cryoglobulinemia dominated the picture of the disease. At the beginning of the disease Staphylococcus aureus abunded in the blood culture. In spite of the therapy the general status of the patient declined steadily.     

Lassoed liver: hepatic pseudo-fissure caused by ventriculoperitoneal shunt

A 2-year-old child with a ventriculoperitoneal shunt and a shunt revision 18 months previously developed a shunt infection. A CT scan of the abdomen was performed soon after the shunt catheter was removed. A peculiar invagination in the liver was demonstrated along the same course of a recently removed intraperitoneal catheter, presumably caused by the catheter. There have been no reported cases of deformity and pseudo-fissure of the liver, which may mimic pathologic processes on ultrasound or CT, caused by a ventriculoperitoneal shunt.     

Chronic granulomatous disease associated with chronic glomerulonephritis

A boy with chronic granulomatous disease (CGD) developed glomerulonephritis at the age of 12 years. The glomerulonephritis progressed to terminal uraemia at age 15 when maintenance haemodialysis was started. The clinical course was complicated by pulmonary aspergillosis and Pseudomonas septicaemia from which he eventually died. The glomerulonephritis was of unknown origin, and a possible relationship between CGD and glomerulonephritis is discussed.     

Chronic Granulomatous Disease Associated with Chronic Glomerulonephritis

ABSTRACT. A boy with chronic granulomatous disease (CGD) developed glomerulonephritis at the age of 12 years. The glomerulonephritis progressed to terminal uraemia at age 15 when maintenance haemodialysis was started. The clinical course was complicated by pulmonary aspergillosis and Pseudomonas septicaemia from which he eventually died. The glomerulonephritis was of unknown origin, and a possible relationship between CGD and glomerulonephritis is discussed.     

Peritoneal metastasis of third ventricular atypical teratoid/rhabdoid tumor after VP shunt implantation for unexplained hydrocephalus

Background

Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) is a highly malignant neoplasm seen frequently in infancy and early childhood. This report presents a 9-year-old girl of primary third ventricular AT/RT with peritoneal metastasis after ventriculoperitoneal (VP) shunt catheter implantation for hydrocephalus before the identification of the CNS tumor.

Methods

The data of clinical course, laboratory and imaging studies were obtained and carefully reviewed. Serial imaging studies including enhanced CT and MRI were performed at the first admission, during which the patient was diagnosed with a non-malignant communicating hydrocephalus. Secondary radiological studies were carried out 5 months after VP shunt, during which the patient demonstrated worsening clinical signs of intracranial hypertension. An imaging study identified a tumor in the third ventricle.

Results

The patient was treated by a surgical resection, showing the specimen was pathologically consistent with AT/RT 5 months after VP shunt. Systematic chemotherapy and radiotherapy were prescribed for the patient. After 6 months, PET/CT revealed peritoneal metastasis but negative findings in the CNS. The parents of the patient refused further intervention, and she died one month later.

Conclusion

VP shunt in a patient with AT/RT may cause distant seeding of the tumor in unrelated areas of the body, even after intensive multimodality treatment. Further studies on shunt related metastases are needed.     

Synchronization of plasmapheresis and pulse therapy in the complex treatment of children with highly active glomerulonephritis]

To suppress the activity of glomerulonephritis, lupus and primary chronic mixed one, 13 children received plasmapheresis synchronously with pulse therapy with cyclophosphamide or prednisolone. Plasmapheresis was carried out daily for 3 days. Six hours after the last session and on days 4 and 5 of the treatment pulse therapy was provided, followed by conventional intake of prednisolone per os in combination with azathioprine or cyclophosphamide. Beneficial therapeutic results were obtained in 10 patients within 3 to 6 weeks. The effect turned out insufficient in a patient with associated systemic lupus erythematosus, and rapid-progressing nephritis and in a child with primary chronic glomerulonephritis of the mesangiocapillary type with fibroplastic transformation and persistent nephrotic syndrome. No therapeutic effect was attained in a patient with focal segmental glomerulosclerosis running its course with long persistent nephrotic syndrome.     

A case of Sotos syndrome with subduroperitoneal shunt

The authors present a case of Sotos syndrome with increasing severity of subdural hygroma from the age of 5 months, which was managed with a subduroperitoneal shunt at 10 months of age. The patient had been followed up until 30 months of age with continuing improvement of symptoms. The patient initially presented with dolichocephaly accompanied by macrocrania, early tooth development, repeated pneumonia infections and developmental retardation concerning crawling, sitting, walking and speaking at 5 months of age. Magnetic resonance imaging (MRI) demonstrated partial hypoplasia of the corpus callosum and bifrontal subdural hygroma. The patient underwent subduroperitoneal shunting at 10 months of age with partial improvement of symptoms. At 18 months of age, the patient showed increased irritability and sweating, and development of spinal kyphosis, which resulted from shunt malfunction as shown in the shuntogram. The appearance of cervical syringomyelia was also seen in the MRI. After shunt revision, the irritability, sweating and kyphosis improved along with disappearance of the syringomyelia. The authors describe a case of Sotos syndrome with subduroperitoneal shunt that showed syringomyelia which developed with shunt malfunction but disappeared after shunt revision. We emphasize the importance of active management such as subduroperitoneal shunting to drain the cerebrospinal fluid in the Sotos syndrome.     

Ventriculoperitoneal shunt block: what are the best predictive clinical indicators?

AIMS: To evaluate the predictive value of symptoms, signs, and radiographic findings accompanying presumed ventriculoperitoneal (VP) shunt malfunction, by comparing presentation with operative findings and subsequent clinical course. METHODS: Prospective study of all 53 patient referrals to a paediatric neurosurgical centre between April and November 1999 with a diagnosis of presumed shunt malfunction. Referral pattern, presenting symptoms and signs, results of computed tomography (CT) scanning, operative findings, and clinical outcome were recorded. Two patient groups were defined, one with proven shunt block, the other with presumed normal shunt function. Symptomatology, CT scan findings, and the subsequent clinical course for each group were then compared. RESULTS: Common presenting features were headache, drowsiness, and vomiting. CT scans were performed in all patients. Thirty seven had operatively proven shunt malfunction, of whom 34 had shunt block and three shunt infection; 84% with shunt block had increased ventricle size when compared with previous imaging. For the two patient groups (with and without shunt block), odds ratios with 95% confidence intervals on their presenting symptoms were headache 1.5 (0.27 to 10.9), vomiting 0.9 (0.25 to 3.65), drowsiness 10 (0.69 to 10.7), and fever 0.19 (0.03 to 6.95). Every patient with ventricular enlargement greater than their known baseline had a proven blocked shunt. CONCLUSIONS: Drowsiness is by far the best clinical predictor of VP shunt block. Headache and vomiting were less predictive of acute shunt block in this study. Wherever possible CT scan findings should be interpreted in the context of previous imaging. We would caution that not all cases of proven shunt blockage present with an increase in ventricle size.     

Fulminant hepatitis and glomerulonephritis--a rare presentation of hepatitis A virus infection

Aim: We report a case of fulminant hepatitis and glomerulonephritis by Hepatitis A virus infection. Methods: We observed the patient’s clinical course and analyzed his data retrospectively. Results: The three‐year‐old boy presented with features of acute renal failure and stage 2 hepatic encephalopathy. Renal biopsy showed diffuse mesangioproliferative glomerulonephritis with immunoglobulin M and complement 3 deposition. After receiving supportive treatment for over 6 weeks, the hepatic and renal function became normal. Renal biopsy after 3 months was normal. Conclusion: Acute renal failure is a rare complication of Hepatitis A virus infection. Physicians should be aware of the potential renal involvement of Hepatitis A virus infection as the prognosis is not always favourable.     

Wegener granulomatosis in pediatric patients

Wegener granulomatosis is more easily recognized as a distinct clinical entity than other vasculitides because the initial clinical features frequently include granulomatous vasculitis of the upper and lower respiratory tract and glomerulonephritis. Although the disease has been lethal in the past, prolonged survival and avoidance of end-stage kidney disease can now be expected when cyclophosphamide therapy is introduced early in the course. We report four children with Wegener granulomatosis in whom the initial clinical findings suggested Henoch-Sch?nlein purpura. In two of the patients Wegener granulomatosis was not recognized until after end-stage kidney disease had developed. The course in these patients emphasizes the need for attention to even scant evidence of inflammation of the upper or lower respiratory tract in patients with glomerulonephritis. Appropriate diagnostic studies may then lead to recognition of Wegener granulomatosis and the prompt institution of appropriate treatment.     

Infections in cerebrospinal fluid shunts

Bacterial colonization of cerebrospinal fluid shunts is a cause of significant morbidity, causing not only shunt malfunction and chronic ill-health but has also been implicated in an immune-complex glomerulonephritis. Almost all shunt colonizations involve Staphylococcus albus which gains access to the shunt during surgery and grows in microcolonies inside the shunt. The most reliable means of diagnosis at present is serological surveillance. Medical management is usually ineffective in eradicating colonization and colonized shunts must be replaced. Recent work on the impregnation of Silastic with antimicrobial substances to prevent colonization has provided encouraging results.     

Spectrum of the syndrome of the isolated fourth ventricle in posthemorrhagic hydrocephalus of the premature infant

Ten patients who were premature born and had intracranial/intraventricular hemorrhage and developed hydrocephalus in early life were treated with ventriculoperitoneal shunts because of progression of the hydrocephalus. The hydrocephalus remained well controlled but in a follow-up period of 2 months to 7 years after shunt placement the patients developed an isolated fourth ventricle that required treatment. The presenting symptoms varied: increasing head size, fontanelle fullness, irritability difficulty with swallowing, vomiting, hypoactivity, headaches and lethargy. One patient presented with full cardiorespiratory arrest and expired. The remainder on neuroimaging studies revealed an extremely large fourth ventricle creating a mass effect and very small (slit-like) lateral ventricles, indicating a functioning lateral ventricle-peritoneal shunt. These patients were treated with the placement of a fourth ventricle shunt catheter and connection to the existing shunt, with resolution of symptoms. Premature infants with hydrocephalus need to be followed and assessed for findings of the syndrome of the isolated fourth ventricle in an attempt to prevent sudden neurological deterioration.     

A rare case of shunt malfunction attributable to a broken Codman-Hakim programmable shunt valve after a blow to the head

We report a rare case of shunt malfunction resulting from breakage of the Codman-Hakim programmable shunt valve system following a blow to the head. Originally, the patient had suffered intraventricular hemorrhage a month after birth; ventriculoperitoneal shunt was performed at 7 months. After that, the shunt valve functioned well for 7 years. The patient presented with headaches after receiving a blow behind the right ear. The headache became exacerbated with activity and improved with rest. Damage to the shunt valve was identified during surgery, with a crack at the top of the valve housing. Symptoms resolved following a change of the complete shunt system. The possibility of damage to the shunt valve following a blow should be considered in shunt patients presenting with headaches.     

Late resistance to corticosteroids in nephrotic syndrome

Corticosteroid resistance appeared late in the course of relapsing nephrotic syndrome in 12 patients who previously had steroid-sensitive relapses for 0.8 to 13 years. In 11 patients, renal histology performed earlier in the course of the disease showed minimal change in eight, mesangial proliferative glomerulonephritis (MesPGN) in two, and focal segmental glomerulosclerosis (FSGS) in one. Renal biopsy in another patient and a repeat procedure in four of eight patients who initially showed minimal change was done after they had developed steroid resistance, and showed FSGS. Cyclophosphamide was given to 11 patients after they became steroid resistant, and induced remission in eight that continued for 1 to 2 years in two patients. The other six had relapses that were steroid sensitive, but three of them (two with FSGS and one with MesPGN) later became resistant to steroids as well as to cyclophosphamide. Of six patients with FSGS, four with initial or subsequent resistance to cyclophosphamide eventually developed renal insufficiency. The other two have remained in remission for 12 to 16 years; one of these did not receive cyclophosphamide. Our observations suggest that patients with late steroid resistance comprise a heterogeneous group; those with FSGS and resistance to cyclophosphamide therapy may have a poor outcome.     

OLIGURIC GLOMERULONEPHRITIS IN AN INFANT OF FOUR MONTHS

A case of oliguric glomerulonephritis in an infant of 4 months is described. The patient was initially treated with steroids during 3 days. When the diagnosis glomerulonephritis had been settled, the treatment comprised fluid restriction and peritoneal dialysis. The patient was discharged after 6 weeks with slight proteinuria, a serum creatinine concentration of 0.5 mg per 100 ml and a systolic blood pressure of 90 mm Hg. Diagnosis and treatment is discussed. It is concluded that treatment with dialysis is well indicated in cases of acute oliguric glomerulonephritis while the beneficial effects of steroids is more doubtful.     

Late presentation of massive pleural effusion from intrathoracic migration of a ventriculoperitoneal shunt catheter: case report and review of the literature

We report an unusual case of ventriculoperitoneal (VP) shunt intrathoracic migration, associated with massive symptomatic hydrothorax. The VP shunt was inserted 10 years before presentation, after hemorrhagic hydrocephalus caused by prenatal intraventricular hemorrhage. The pleural fluid was drained via tube thoracostomy and the shunt was externalized, with full resolution of symptoms and signs. The patient was subsequently managed with shunt revision with drainage into the abdominal cavity. We review the 10 pediatric cases of cerebrospinal fluid hydrothorax reported in the literature and discuss the mechanism of shunt tip migration. Pleural effusion secondary to VP shunt insertion is a rare and potentially life-threatening occurrence, and it should be suspected in any patient with a VP shunt and respiratory distress.     

Open thoracic meningocele associated with amniotic band syndrome.

We present a case of an infant born with an open thoracic meningocele secondary to an amniotic band. Although there have been several case reports of encephalocele, anencephaly, closed meningocele and a case of tethered cord associated with amniotic band syndrome (ABS), there has yet to be a report of an open meningocele associated with ABS. The patient remained neurologically intact after the repair of his meningocele. His postoperative course was complicated by meningitis with subsequent hydrocephalus necessitating ventriculoperitoneal shunt placement.     

Predictive value of fractional excretion of filtered sodium for hypertension in acute post-streptococcal glomerulonephritis

The course of acute post-streptococcal glomerulonephritis was followed in 81 children, 66 of whom were hypertensive on admission. Sixty-one hypertensive patients were available for follow-up; in seven (11.5%), hypertension recurred 1 to 9 days after initial blood pressure elevation had returned to normal. Thirteen initially normotensive patients were available for follow-up; six (46%) developed hypertension 1 to 9 days after admission. Initial FENa was less than or equal to 0.5 in all 13 patients with recurrence of initial hypertension or who developed initial hypertension while under observation, and in another 25 patients who did not have this course. On the other hand, FENa was greater than 0.5 in 36 patients, none of whom had recurrence of initial hypertension or developed hypertension while under observation. Therefore, an admission FENa less than or equal to 0.5 seems to be an accurate predictor for development of hypertensive episodes.     

Transjugular intrahepatic portosystemic shunt prior to renal transplantation in a child with autosomal-recessive polycystic kidney disease and portal hypertension: A case report

Autosomal-recessive polycystic kidney disease (ARPKD) can cause renal failure and portal hypertension in children. Portal hypertension may complicate the course of renal transplantation (Tx). We report the successful outcome of a patient with end-stage renal disease (ESRD) and portal hypertension treated with transjugular intrahepatic portosystemic shunt (TIPS), a minimally invasive endovascular technique of portosystemic shunt, prior to renal Tx.