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The Oral Manifestations of Intestinal Lymphangiectasia

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The Oral Manifestations of Intestinal Lymphangiectasia Theoral manifestationsof intestinal lymphangiectasia:case report Patrick M. Ralph, DMDKenneth C. Troutman, DDS, MPH Abstract patients with primary IL in whomstandard in vitro 6 Intestinal lymphangiectasiais a rare autosomaldomi- tests of neutrophil function were abnormal. In addi- nant disorder or acquired condition that leads to lymph tion, patients with IL have been shown to have im- paired cellular immunity secondary to loss of lympho- obstruction, poor chyle transport, and concomitantprob- 14,1s lems of hypoproteinemia, lymphocytopenia, hypogam- cytes into the bowel lumen. Consequently, these maglobulinemia,and peripheral edema. Patients develop patients potentially have impaired neutrophil (PMN) diarrhea, steatorrhea, and hypocalcemiasecondary to fat- function. Patients with IL also have compromisedcal- soluble vitamin malabsorption.Treatment is a restrictive cium and vitamin D homeostasis leading to defects of diet of low fat, mediumchain triglycerides. Oral manifes- both hard and soft tissues in the oral cavity. tations are gingivitis due to poor PMNfunction and The most apparent oral complications associated enameldefects due to poor calciumabsorption. A case of a with patients diagnosed with IL are hypoplastic enamel 14-year-old boy with both gingival and enamel problems defects (HED)and to a lesser extent, but not insignifi- secondary to intestinal lymphangiectasia is reported. cant, gingivitis. Both can lead to prematuretooth loss. (Pediatr Dent 18:461-64, 1996) IL patients present a chronic disruptive disorder of enamel enabling one to determine the severity and duration of the morphogenic disturbance from the ntestinal lymphangiectasia(IL) is a rare disorder 16 the small intestine characterized by dilated lymph- HED. IL patients have poor calcium and vitamin D I atics. IL has no gender predilection, usually appears homeostasis, resulting in HED,which is often related in the first decade of life, and maybe primary or sec- to the degree of patient compliance with the severely ondary to lymph obstruction. 1 Primary IL occurs con- restricted diet. Hypoproteinemia, hypogamma- genitally whenlymphatics of the small intestine fail to globulinemia, and hypocalcemia also develop as a re- unite. 2 IL can also be present as an autosomal dominant sult of poor dietary compliance.These deficiencies lead disorder knownas Milroy’s disease. As a secondary dis- to a decreased quality and quantity of neutrophils and order, lymphobstruction occurs as a result of an inflam- immunoglobulins--the primary immunocytes in the matory or neoplastic condition. For example, it can be periodontium--and poor gingival status in IL patients. secondary to messenteric tuberculosis, sarcoidosis, ma- Management lignant lymphoma,chronic pancreatitis, Noonan’ssyn- drome,and congestive heart failure. 3 IL ultimately inter- Successful medical managementof IL is related di- feres with the transport of chyle via the mesenteric, rectly to the patient’s adherence to the severely re- retroperitoneal, or main thoracic lymphatics into the stricted dietary therapy. The major goal of management blood circulation, resulting in mucosaldilation. is to eliminate lymphatic obstruction. This is accom- IL4 was first described in 1961 by Waldmannet al. plished by placing patients on a low fat, mediumchain As the authors described, patients with IL develop triglyceride (MCT)diet, 17 which decreases the amount protein-losing enteropathy leading to hypoproteine- of inflammation in the lumen, thereby allowing proper mia, lymphocytopenia, hypogammaglobulinemia, lymphatic flow to occur. MCTsare unlike long chain and peripheral edema. In addition, these patients triglycerides in that they do not require lymphatic develop mild to moderate diarrhea, steatorrhea, and transport. WhenMCTs are metabolized, they are ab- hypocalcemia secondary to fat and fat-soluble vita- sorbed directly into the circulation without stimulat- min malabsorption. ing lymphflow. This decreases villi dilatation and re- Intestinal lymphangiectasia has been reported spo- stores lymphatic flow in order to limit enteric fat, radically in the literature. ~-13 Boltonet al. reportedthree protein, and lymphocyte losses in the bowel lumen. PediatricDentistry - 18:7,1996 AmericanAcademy of PediatricDentistry 461 Case report Medical history and chief complaint A 14-year-old white male with complaint of poor dental esthetics was brought to the Columbia-Presby- terian Medical Center (CPMC) pediatric dental clinic. The patient was a 5 lb, 14 oz product of a full-term uncomplicated pregnancy from a nonconsanguinous marriage. He had no neonatal complications and was discharged with his mother at 3 days of age. From birth to 7 weeks the patient had a history of frequent stools with some respiratory congestion. His physical exam revealed pale skin with periorbital, pedal, and scrotal edema. In addition, the patient had ascites, decreased abdominal girth, left inguinal hernia, and generalized Fig 1. A 14-year-old boy with intestinal lymphangiectasia. lymphadenopathy. The laboratory evaluation revealed Note the periorbital edema and poor esthetics. hypocalcemia, hypoalbumenemia, hypoproteinemia, and evidence of fat malabsorption (carotene = 22 mg). quadrants with recurrent decay around all existing res- Abdominal radiographic review showed distended torations. Radiographs confirmed the caries (Fig 5). loops of bowel with increased distance between bowel Immunologic function tests were not available, how- loops suggestive of bowel wall edema. A bowel biopsy ever, all serum values were indicative of poor immune revealed gross dilatation of the lymphatic vessels of the function: total protein 3.8 (range = 6.8-8.5), globulin 1.2 submucosa with normal mucosal architecture. Clinical (range = 1.5-4.5), albumin 2.6 (range = 3.5-5.5). presentation led to the diagnosis of IL at age 7 weeks. Discussion No other family members were affected. By age 14 weeks the patient was maintained on a combination of A thorough review of the literature was conducted Progestimil™ and Portagen™ (Mead Johnson Nutri- to examine the relationship of this patient with other tional, Evansville, IN) along with vitamin supplements. reported cases of IL. Of all cases reviewed, all reported At 14 weeks the left inguinal hernia was corrected sur- manifestations were related to the gastrointestinal tract, gically. From age 14 weeks to the present age of 14 but none to the oral cavity. No significant differences were years this young man has been followed at CPMC for found between the overall manifestations (e.g., lymphatic extensive problems related to his diagnosis. Due to lack obstruction, generalized lymphadenopathy, gross intes- of compliance with the restricted diet, the patient had tinal dilation) of this patient and others reported. a history of frequent hospitalizations secondary to epi- Review of the dental history revealed no familial sodes of poor calcium and vitamin D homeostasis, hy- history of Al or excessive exposure to fluoride, ruling poproteinemia, hypogammaglobulinemia, and hypoal- out these causes of enamel defects. The potential for buminemia. Due to his fluctuating nutritional status poor oral hygiene as the cause of the gingival problems secondary to poor dietary compliance, a short stature could not be ruled out, but the possibility of IL contrib- and deficient immune system resulted. uting to the gingival problem is likely due to its signifi- Clinical exam cant systemic effects. The extraoral examination revealed a pale complex- HED and gingivitis are well documented in the lit- ion, dry skin, chapped lips, and periorbital edema (Fig erature, however not in association with IL. The excess 1). The cervical and submandibular lymph nodes were loss of nutrients into the bowel lumen in patients with palpable and slightly tender bilaterally. IL plays a major role in its pathogenesis. Since the mal- Intraoral exam revealed slight adenoid hyperplasia absorption of proteins and fat-soluble vitamins can lead with a midline uvula. The patient had poor oral hy- to poor immune responses and enamel hypoplasia, it giene and moderate to severe marginal generalized gin- is reasonable to assume that IL contributed to the den- givitis. No other soft tissue abnormalities were noted. tal complications seen in this patient. Other hereditary All permanent teeth were present with the exception causes had been eliminated. of the mandibular right first molar and the maxillary Persons afflicted with IL have impaired cell-medi- and mandibular third molars. Teeth had hypoplastic ated immunity (CMI), which is attributed to the con- enamel defects (Figs 2 and 3)— both the maxillary and tinuous depletion of immunologic proteins into the the mandibular teeth were equally affected—present- bowel lumen. In spite of this protein loss, IL patients ing widespread pitted defects and brown discoloration maintain satisfactory health without an abundance of of the enamel from the occlusal to the cervical third. opportunistic infections. However, they may develop Direct bond laminates had been placed on the max- small bacterial infections of the integument, respiratory illary permanent central incisors and the right lateral surfaces, and urinary tract as a result of poor CMI or incisor (Fig 4). The mandibular left first permanent neutrophil activity. molar had been extracted. Caries affected teeth in all The defensive role of neutrophils in the oral cavity 462 American Academy of Pediatric Dentistry Pediatric Dentistry - 18:7, 1996 lymphocyte and immunoglobulin depletion, causing
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