To the editor:
Chyloperitoneum is a rare condition in patients on peritoneal dialysis.1 It has been reported in patients diagnosed with systemic amyloidosis. We present a patient on peritoneal dialysis, with familial amyloidosis Andrade type with intermittent and recurrent chyloperitoneum.
The patient was a 69 year-old woman who began peritoneal dialysis in April of 2006. She had renal amyloidosis in the setting of familial amyloid polyneuropathy (FAP), which had manifested with nephrotic syndrome.
The clinical picture onset at 64 yearold with hyperestesia of the lower limbs. The electromyography was compatible with mixed polyneuropathy. She had the transthyretin transthyretin (TTR) mutation (Val-30-Met). Rectal biopsy study showed amyloid deposition, and the diagnosis of FAP was confirmed. At presentation, renal function was normal and the proteinuria was negative. She required a pacemaker because of cardiac amyloid infiltration.
Mallorca is the fifth world endemic focus of FAP or Andrade¿s disease,2 a hereditary systemic amyloidosis, with mixed polyneuropathy being the main manifestation. The inheritance is autosomal dominant. The disease is due to a transthyretin mutation, which is synthesized in the liver and forms systemic depositions, which cause the clinical picture.
The diagnosis is made by means of rectal, fat or sural nerve biopsy, that discloses the TTR variant or the DNA mutation.
The only efficacious treatment is liver transplantation.
In January of 2004 proteinuria appeared, which reached nephrotic range. Since April of 2005, the renal function deteriorated progressively, and in February of 2006 a catheter for peritoneal dialysis was placed.
During peritoneal catheter implantation peritoneal lavage was performed and a white fluid was obtained.
Chyloperitoneum is the appearance of a turbid milk-like fluid due to the presence of chylomicrons. Among the causes that can be implicated are: micro-traumas, sometimes during catheter insertion;3 malignant diseases, mainly lymphomas;4 hepatic cirrhosis, acute pancreatitis;5 systemic amyloidosis6 as in the case reported; lymphatic obstruction due to fibrotic adherences of tuberculosis;7 dihydropiridinic calcium channel antagonists;8 and lymphatic vessels malformations.9
During the training period, chyloperitoneum was obtained that attenuated with lavage.
The suspicion of chyloperitoneum arises in the presence of milk-like fluid and the diagnosis is confirmed when chylomicrons are detected, ortriglycerides levels in the peritoneal fluid are higher than those in plasma, and protein value in the peritoneal fluid is higher than half of the protein value in plasma, like in the reported case. Differential diagnosis should be made with conditions accompanied with turbid fluid, normal cellularity and negative cultures.
A expectant attitude was taken. Regular oil was replaced by mediumchain triglycerides (MCT)-containing oil.
Treatment is usually conservative. A protein and carbohydrate rich diet is recommended with low fat content. MTC oils are preferred. The ligation of the thoracic duct can prevent nutritional complications.
Currently the patient presents intermittent and self-limited chyloperitoneum (every 10 days approximately). Nutritional parameters are preserved with daily intake of 2 liters of Nutrineal. The patient is wheel chair-bound due to FAP-associated disability.
