07/31/09 Periodontal Changes Associated With Chronic Idiopathic Neutropenia

Date: 07/31/09
Resident: Jason Hencler
Article title: Periodontal Changes Associated with Chronic Idiopathic Neutropenia
Author(s): Kenneth L. Kalkwarf, DDS, MS; Dennis P. Gutz, DDS, MS
Journal: Pediatric Dentistry
Volume #3; Number 2; Page 189-195 Year: 1981

Major topic: Chronic Idiopathic Neutropenia

Background: Chronic Idiopathic Neutropenia is a relatively rare blood disorder characterized by severe decrease in circulating neutrophils. Infants and children exhibiting severe neutropenias report hx of persistent infections of the skin and respiratory tract as well as severe gingivitis, oral ulcers, and destruction of the alveolar bone. Only antibiotics have had predictable success in controlling systemic sequel.

Type of Article: Case Report

Main Purpose: Present a case report of a child exhibiting chronic neutropenia, discuss classification of such neuropenias and review dental management for children with such problems.

Overview of method of research: A four year clinical course of this patient was monitored and is presented in this paper.

Findings: Patient was a 3 ½ yo white female in no pain referred for eval and tx of her gingival condition. Med hx revealed initial problems appeared at 2 ½ wks with the development of an intense papular diaper rash. Dx was staphylococcal dermatitis and was tx with ampicillin. The rash cleared but recurred 2 wks after antibiotics discontinued. Patient also experienced many bouts of respiratory distress and frequent febrile episodes. Each clinical picture recurred with cessation of antibiotics. A bone marrow examination was consistent with congenital neutropenia. Dental findings were swollen edematous gingival with a distinct granulomatous collar at the cervical region that bled easily when manipulated. Gingival recession had occurred in the anterior areas resulting in root exposure. No caries detected. Mother noted patient complained of a sore mouth and resisted tooth brushing. Radiographs revealed significant bone loss in the anterior regions of the mouth but no caries. OHI was given and patient was put on 3 mo recare over the next 4 yrs. During the 4 yrs of observation the patient’s OH varied from fair to poor. Progressive gingival recession eventually resulted in advanced root exposure throughout the primary dentition especially in the anterior regions. Radiographic eval revealed alveolar destruction in anterior regions and furcations of primary molars. Soft tissue lesions, appearing to be aphthous ulcers were occasionally present. No radiographic caries was observed. Mandibular primary incisors were exfoliated at 5 yr 7 mo. Eruption of permanent incisors followed a normal pattern with normal alveolar support through age 7.

Key points in the article discussion: Numerous systemic conditions can result in gingival inflammation, alveolar bone loss, and mucosal ulceration. Diff Dx should include: juvenile perio, histiocytosis X, Chediak-Lefevre syndrome, acatalasia, chronic granulomatous disease, hypophosphatasia, diabetes, leukemia, and neutropenia. Neutropenic conditions in children are classified according to clinical symptoms, age of onset, duration, white cell counts, immune function, familial tendencies, and bone marrow alterations. The patient in this case was dx as chronic idiopathic neutropenia on the following basis. The clinical course of neutropenia was present from birth with no etiology, no familial tendencies discovered. Clinical symptoms consisted of persistent, recurrent infections including gingivitis, throughout the patient’s life. An interesting finding was that while periodontal destruction, gingival recession, tooth discoloration and moderate to heavy plaque accumulation were present, no evidence of carious activity was ever found. Tooth discoloration was due to intrinsic staining, probably due to antibiotics during formative periods and extrinsic staining due to plaque control attempts. Therapy modalities other that antibiotics to control recurrent episodes of infection were not successful. The lack of carious activity is difficult to explain but may be due to a relative lack of certain inflammatory cells that alters the microbial population to one incapable of initiating a carious process. At the time this article was published the exact role(s) of the neutrophil in protecting the periodontal tissues were not discovered but investigators had determined that the cell may pass through the junct epi and reside in the sulcus in a viable state. It was certainly plausible that the periodontal destruction seen in patients with chronic idiopathic neutropenia is a result of decreased neutrophil count. We now know that the neutrophil play an important role in contolling the periodontal microbiota. They are the first leukocyte to arrive at the site of inflammation and are always the dominant cell type in the junct epi and the gingival sulcus. Disorders of neutrophils are associated with invasive perio infections and aggressive periodontitis. This patient exhibited a normal eruption sequence of the perm dentition. The eruption schedule was accelerated due probably to early loss of primary dentition. It is expected that alveolar destruction around her perm teeth will occur with time. Episodes of recurrent aphthous ulcers seen in neutropenic patients do not have a clear association with the circulating neutrophil count. With better antibiotic therapy, children with hematologic disorders are surviving infections and are requiring dental tx. Although caries does not appear to be a major problem, patients with chronic idiopathic neutropenia, daily stannous fluoride gel has been recommended to reduce the possibility of tooth loss or dental infection due to carious lesion. Stannous fluoride application has also been shown to reduce plaque formation and be potentially useful in the control of early periodontitis. A strict hygiene program should be implemented for these patients. Also, maximum precautions including aseptic rinses and antibiotic coverage, prior to tissue manipulation is suggested to reduce chance of bacteremias and post op infection.

Summary of conclusions: Chronic idiopathic neutropenia is characterized by a severe decrease in the number of circulating neutrophils. Symptoms include oral ulceration, gingival inflammation, and rapid alveolar bone loss.

Assessment of article: This article presented a good background of neutropenia types and there classification. All aspects presented from this case, including neutropenic symptoms, tx, and dx would be helpful to a pediatric dentist when tx a similar patient. This article, however, may be a little outdated. It was written when the neutrophils exact role in protecting the periodontal apparatus was still unknown. At 28 yo, this article outdates some of our residents, unfortunately I am not one of them.