Enlarged dental follicles, a follicular cyst, and enamel hypoplasia in a patient with Lowe syndrome

Enlarged dental follicles, a follicular cyst, and enamel hypoplasia in a patient with Lowe syndrome Michael W. Roberts, DDS, MSCD,~ George H. Blakey, DDSb John R. Jacoway, DDS, PhD,’ Stephanie C. Chen, DDSd and C. Richard Morris, MD,” Chapel Hill, N.C. SCHOOL OF DENTISTRY AND SCHOOL OF MEDICINE, UNIVERSITY OF NORTH CAROLINA AT CHAPEL HILL

eruption,

An 1195 -year-old boy with Lowe syndrome presented with various dental abnormalities including delayed enamel hypoplasia, and enlarged follicles surrounding unerupted teeth. (ORAL SURC ORAL MED ORM. PATHOL

1994;77:264-5)

Lowe syndrome, or oculocerebrorenal syndrome, was described first in 1952.’ The syndrome is characterized by aminoaciduria, albuminuria, rickets, congenital cataracts, glaucoma, frontal bossing, and mental retardation. Concommitant congenital nephrotic syndrome has been reported as we11.2Inheritance is associated with a x-linked pattern3 CASE REPORT

An 11%-year-old white boy diagnosed with Lowe syndrome was referred to the University of North Carolina Department of Pediatric Dentistry for examination and treatment. The patient had exhibited acute anxiety and disruptive behavior during previous dental appointments. Only preventive oral hygiene care had been accomplished, and no oral radiographs had been obtained in the past. Intraoral examination was difficult and limited by the patient’s anxiety. The eruption pattern of the permanent dentition was delayed. All four first permanent molars and the left maxillary central incisor demonstrated extremely hypoplastic enamel and dental caries. A panoramic radiograph revealed large pericoronal radiolucencies associated with many of the unerupted permanent teeth. The radiolucency associatedwith the unerupted right second mandibular molar was significantly larger than the others and appeared to have displaced the developing tooth distally (Fig. 1). An oral and maxillofacial surgery consultation was obtained, and a team approach plan of care developed.The

aAssociate Professor,Graduate Program Director, Department of Pediatric Dentistry, School of Dentistry. bAssistant Professor, Department of Oral and Maxillofacial Surgery, School of Dentistry. cProfessorand Head, Oral Pathology, Department of Diagnostic Sciences,School of Dentistry. dFellow, Department of Pediatric Dentistry, School of Dentistry. eAssociateProfessor,Chief, Division of Pediatric Nephrology, Department of Pediatrics, School of Medicine. Copyright @ 1994 by Mosby-Year Rook, Inc. 0030-4220/94/$3.00+ 0 7/14/52561

264

patient was taken to the operating room for restoration of the four first permanent molars with stainless steel crowns and the central incisor with bonded resin. The primary first molars, cuspids, and unerupted right maxillary and mandibular permanent second molars were extracted. The associatedsoft tissues were sent for biopsy. The services of the Division of Pediatric Nephrology were solicited to manage the preoperative, operative, and postoperative electrolyte hemostasisof the patient becauseof severerenal impairment. The clinical appearance of the tissues connected to the extracted unerupted right second permanent molars was consistent with follicular cysts. The microscopic examination of the soft tissue excised with the maxillary secondmolar revealed lightly collagenized fibrous connective tissue with a small collection of stratified squamous epithelium cells and a small bone fragment. The specimen was diagnosed as consistent with a dental follicle. Sections prepared from the submitted specimen of the right second mandibular molar showed fibrous connective tissue with mild chronic inflammation and multiple foci of calcification. A cyst-like structure was present and was lined by thin stratified squamous epithelium consistent with a follicular cyst. No evidence of odontogenic tumor or malignancy was observed (Fig. 2). DISCUSSION Swelling of the alveolar ridge associated with erupting primary teeth and consistent with eruption cysts in the Lowe syndrome was first described by Illig et a1.4 We report a case with enlarged dental follicles, a confirmed follicular cyst, and several hypoplastic permanent teeth. Rickets is commonly associated with Lowe syndrome. The patient’s stature, delayed eruption of the permanent teeth, an absence of lamina dura, hypoplastic enamel, and elongated pulp horns are consistent with renal dysfunction rickets.5 Treatment with sodium, phosphorus, and vitamin D supplements have been successful in reducing the rickets-like symptoms.

OR.AL SURGERY ORAL volume 77, Number 3

MEDICINE ORAL PATHOLOGY

Roberts et al.

Fig. 1. Radiographic appearanceof enlarged dental follicles, cyst-like lesion, and hypoplastic first permanent molars (arrows).

Fig. 2. View of cystic processlined by thin stratified squamous epithelium. The pericystic fibrous connective tissue is lightly collagenized and contains a mild chronic infiltrate and an island of odontogenic epithelium (errowl. (Hematoxylin and eosin stain; original magnification x200.) REF‘ERENCES Lowe CU, Terry M, MacLachlan EA. Organic aciduria, decreased renal ammonia production, hydrophthalmos, and mental retardation: a clinical entity. Am J Dis Child 1952; 83:164-84. 2. Nielsen KF, Steffensen GK. Congenital nephrotic syndrome associated with Lowe syndrome. Child Nephrol Urol 1990; 10:92-5. 3. Abbassi V, Lowe CU, Calgagno PL. Oculocerebrorenal syndrome: a review. Am J Dis Child 1968;115:145-68. 4. Illig VR, Dumermuth G, Prader A. Das oculocerebrorenale syndrome (Lowe). Klinische, metabolische und elektroenceph-

alographische Befunde bei 3 Fallen. Helv Paediatr Acta 1963;18:173-202. 5. Shafer WG, Hine MK, Levy BM, eds. A textbook of or al pathology. Philadelphia: WB Saunders, 1983:641-5. Reprint requests: Michael W. Roberts, DDS, MScD Department of Pediatric Dentistry School of Dentistry, CB 7450 University of North Carolina at Chapel Hill Chapel Hill, NC 27599-7450