Sterile cerebrospinal fluid ascites presenting as high SAAG ascites: a case report
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Cerebrospinal fluid ascites is a rare complication of ventriculoperitoneal shunting and is the result of infection and subsequent peritonitis in the majority of cases. Sterile cerebrospinal fluid ascites in which no known infectious etiology is identified, is even more unusual.
A 26-year-old female with Loeys-Dietz syndrome and congenital hydrocephalus treated with a ventriculoperitoneal shunt, was evaluated after developing new-onset ascites of unclear etiology after abdominal surgery for repair of an aortic aneurysm requiring multiple therapeutic paracenteses. Her serum ascites albumin gradient (SAAG) was greater than 1.1, suggestive of a portal hypertensive etiology. Gram stain as well as multiple cultures of her ascites fluid were both negative. Extensive investigation including hepatic venous portal gradient measurement and liver biopsy revealed no evidence of hepatic disease or portal hypertension. She was ultimately found to have sterile cerebrospinal fluid ascites which was treated successfully with a peritoneovenous shunt.
Sterile cerebrospinal fluid ascites is a rare clinical entity that has only been reported approximately 50 times in the medical literature. In this report, we also highlight it as a rare cause of high SAAG ascites. Moreover, we describe the use of a peritoneovenous shunt as a novel therapeutic option in the management of this condition.
KeywordsAscites Cerebrospinal fluid Serum ascites albumin gradient Peritoneovenous shunt Case report
Serum ascites albumin gradient
Ascites is a common clinical problem for which hepatic cirrhosis represents ~ 80% of all cases, while non-hepatic causes including peritoneal malignancy (12%), cardiac failure (5%), and peritoneal tuberculosis (2%) constitute most of the remaining etiologies . A thorough evaluation, including assessment of the serum ascites albumin gradient (SAAG), is crucial to identifying the cause of the ascites and implementing an appropriate course of treatment. Low SAAG (< 1.1 g/dL) ascites is most frequently caused by peritoneal malignancy or tuberculosis and can also be caused by nephrotic syndrome, pancreatic ascites, or protein-losing enteropathy. A SAAG of ≥1.1 g/dL has been shown to be effective at identifying patients with ascites secondary to sinusoidal portal hypertension. Interestingly, the SAAG has been shown to correlate with hepatic sinusoidal pressure and the threshold of 1.1 g/dL roughly corresponds to a portal pressure gradient of 12 mmHg , the pressure that is thought to be necessary for the development of ascites in patients with cirrhosis. High SAAG ascites can also be the result of other disease processes including cardiac failure, alcoholic hepatitis, and Budd-Chiari syndrome. In this report, we describe a case of high SAAG ascites secondary to sterile cerebrospinal fluid accumulation.
Discussion and conclusions
Published cases of CSF ascites with reported SAAG values
Predisposing pathology for CSF ascites formation
Denver shunt, paracenteses
Longstreth and Buckwalter 
Inflammatory reaction to silicone tubing
Das et al. 
High (not specified)
Comba et al. 
Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder first described in 2005, characterized by aortic aneurysms, generalized arterial tortuosity, hypertelorism, and cleft palate. LDS has been described due to mutations in the transforming growth factor β I (TGFBR1), transforming growth factor β II (TGFBR2), transforming growth factor β 2 ligand gene (TGFB2), and the decapentaplegic homolog 3 (SMAD3) . Clinical complications of LDS are primarily a result of vascular complications including aortic dissection and aneurysmal rupture, which often require aortic surgery as was the case in our patients. Gastrointestinal complications including constipation as well as higher prevalence of eosinophilic gastrointestinal disease have been reported, but hepatic complications specifically related to LDS have not been reported.
Our case is also notable for the successful use of a Denver shunt in the management of CSF ascites, which has not been previously reported. Denver shunts have been successfully used in the management of portal hypertensive and malignant ascites and rely on the principle that ascites flows down a pressure gradient from the peritoneal cavity into the central venous circulation [9, 10]. Backflow of blood is prevented through the use of a one-way valve chamber that lies in the subcutaneous tissue that can be compressed to promote flow and avoid blockage. Complications include infection, blockage, venous thrombosis, and disseminated intravascular coagulation. In the literature, CSF ascites has been treated primarily with conversion of the existing VP shunt to a VA shunt . Our case demonstrates that the use of a Denver shunt may be a safe and viable alternative treatment modality to VA shunting for this condition.
In this report, we characterize sterile cerebrospinal fluid ascites as a rare cause of high SAAG ascites, highlighting the need for clinician awareness for this rare entity in the differential diagnosis of ascites. Moreover, we describe the use of a peritoneovenous shunt for the first time as a novel and viable therapeutic option in the management of this condition.
DKL – wrote manuscript, acquisition and analysis of data; JMP – acquisition and analysis of data; JES – acquisition and analysis of data; JCY – acquisition and analysis of data; all authors have read and approved the manuscript for submission.
Ethics approval and consent to participate
Consent for publication
Written informed consent has been obtained from the patient for their personal and clinical details to be included in the publication of this case report.
The authors declare that they have no competing interests.
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