increased risk of pyogenic liver abscess in children with papillon-lefevre syndrome

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  • Increased Risk of Pyogenic Liver Abscess in Children With Papillon-Lefevre Syndrome

    By Pelin OQuzkurt, F. Cahit Tanyel, Nebil Bijyiikpamukqu, and Akgiin Hiqstinmez Ankara, Turkey

    0 Among 16 with pyogenic liver abscess, two were found to have Papillon-Lefevre syndrome. The two cases are re- ported herein. Sacteremia from involved periodontal tissues

    and a possible impaired immune response could indicate an increased risk of pyogenic liver abscess among children with Papillon-Lefevre syndrome.

    Copkright o 1996 by WA Saunders Company

    INDEX WORDS: Liver abscess, Papillon-Lefevre syndrome.

    P YOGENIC LIVER ABSCESS (PLA), which is an uncommon surgical problem among children, generally occurs in a compromised host who has a malignancy, septicemia, or chronic granulomotous disease.l Other factors that reportedly increase the risk of PLA are trauma, catheterization of the umbili- cal vein, omphalitis, sickle cell disease, and biliary tract surgery.2

    An evaluation of 16 patients diagnosed to have PLA during a 19-year period (1976-1994) showed that two of them had Papillon-Lefevre syndrome (12.5 %). The high incidence in this series indicates that there is an increased risk of Papillon-Lefevre syndrome among children with PLA.


    Case I

    An &year-old boy had a 2-week history of fever, abdominal pain and distension, malaise, and weight loss. At 2 years of age, because of the presence of typical components, he had been diagnosed as having Papillon-Lefevre syndrome. At the time of physical exami- nation, his temperature was 39.5C. The liver was palpable 8 cm below the right costal margin, and there was tenderness in the right upper quadrant. Breath sounds were diminished in the right hemithorax. There was a predominance of polymorphonuclear leukocytes in the peripheric blood smear. He was mildly anemic. Through sulfur colloid scintigraphy, a filling defect was noted in the right lobe of the liver, with diffuse hepatosplenomegaly. A right paramedian incision was made, and a right lobe abscess was drained; a sump drain was left in the abscess cavity. Staphylococcus uureus was present in the culture. He was treated with broad- spectrum antibiotics for 2.5 months. He recovered and was dis- charged, and has been well for 2 years.

    Case 2

    A 12-year-old girl had a l-week history of malaise, fever, and abdominal pain. She had palmoplantar keratoderma and diffuse periodontal inflammation, diagnostic of Papillon-Lefevre syn- drome, for 9 years. At the time of admission her temperature was 38C. Breath sounds were diminished in the lower zone of the right hemithorax. There was tenderness in the right upper quadrant of

    Journal ofPediafric Surgery, Vol31, No 7 (July), 1996: pp 955-956

    the abdomen. The complete blood count showed a hemoglobin level of 9 g/dL with leukocytosis. The right hemidiaphragm was found to be elevated on the posteroanterior chest x-ray. Ultrasono- graphic (US) and contrast-enhanced computed tomographic (CT) evaluations suggested the presence of an abscess (measuring 5 x 3 cm) in the right lobe of the liver. She underwent laparotomy through a subcostal incision. The abscess cavity that had been defined by US and CT evaluations was encountered and drained. The pus culture was positive for Escherichia co/i. Broad-spectrum antibiotics were administered for 22 days. The early postoperative period was uneventful. However, during the fifth postoperative month, the symptoms recurred and US showed an abscess of 8 x 10 cm at the site of the initial abscess. US-guided percutaneous drainage was performed, and antibiotic treatment was begun. The recovery period was uneventful. The patient is free of symptoms and findings related to hver abscess after 1.5 years of follow-up.


    Papillon-Lefevre syndrome is an autosomal-reces- sive genodermatosis characterized by the association of palmoplantar keratoderma and severe periodon- topathy, which results in premature loss of deciduous and permanent dentition3 Contrary to the rarity of Papillon-Lefevre syndrome in the general population, we found that two of 16 patients with PLA had this condition, which suggests that the risk is greater among patients with PLA.

    Although a 23-year-old girl with Papillon-Lefevre syndrome was reported to have two liver abscesses caused by pyogenic bacteria and a subphrenic abscess during Etretinate (Roche, Basel, Switzerland) therapy,4 the increased risk has not been mentioned in the literature.

    It has been demonstrated through light and elec- tron microscopic and bacteriological studies that in periodontal disease, invading bacteria are present in the cementum and in the radicular dentin of the teeth.5 Among patients with Papillon-Lefevre syn- drome, significant bacteremia has occurred during extensive periodontal inflammation, and antibiotic prophylaxis has been suggested.6

    Although no specific immune deficiency has been

    From the Department of Pediatric Surgery, Hacettepe University School of Medicine, Ankara, Turkey.

    Address reprint requests to Professor Dr F. Cahit Tanyel, Hucettepe oniversitesi Tzp Fakiiltesi, qocuk Cerruhisi Anabilim Dab, 06100

    Slhhiye, Ankara, Turkq,. Copyright o 1996 by L%B. Saunders Company 0022-3468/96/3107-0021$03.00l0



    described in cases of Papillon-Lefevre syndrome, have been found through immunohistochemical evalu- impairments in chemotaxis of polymorphonuclear ation of neutrophils.* leukocytes (PMNL), phagocytosis of opsonized S Therefore, bacteremia from involved periodontal aureus, and production of superoxide radicals by tissues, with the addition of a possible impaired PMNL have been reported. Additionally, leukocyte immune response, appear to correlate with an in- chemotaxis deficiency and a decrease in T lympho- creased incidence of Papillon-Lefevre syndrome cytes, with reduction of myeloperoxidase content, among children with PLA.


    1. Harrington E, Bleicher MA: Cryptogenic hepatic abscess in two uncompromised children. J Pediatr Surg 15:660-662, 1980

    2. Rowe IM, ONeill JA, Grosfeld JL, et al: Essentials of Pediatric Surgery (ed 1). St Louis, MO, Mosby-Year Book, 1995, p 623

    3. Gelmetti C, Nazzaro V, Cerri D, et al: Long term preserva- tion of permanent teeth in a patient with Papillon-Lefevre syn- drome treated with etretinate. Pediatr Dermatol6:222-225, 1989

    4. Tosti A, Manuzzi P, Bardazzi F, et al: Is etretinate dangerous in Papillon-Lefevre syndrome? Dermatologica 176:148-150,1988

    5. Adriaens PA: Bacterial invasion in periodontitis, is it impor- tant in periodontal treatment? Rev Belge Med Dent 449-30, 1989

    6. Yusof ZA: Prevention of bacterial endocarditis in localized juvenile periodontitis and Papillon-Lefevre syndrome patients. Dent J Malays 10:31-35, 1988

    7. Bullon P, Pascual A, Fernandez Novoa MC, et al: Late onset Papillon-Lefevre syndrome? A chromosomic, neutrophil function and microbiological study. J Clin Periodontol20:662-667, 1993

    8. DAngelo M, Margiotta V, Franc0 V, et al: Neutrophil dysfunction in prepubertal periodontitis associated with Papillon- Lefevre syndrome. Minerva Stomatol41:591-596, 1992


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