Case Report Delayed Tooth Eruption in Congenital Hypertrkhosis Lanuginosa

Case Report Delayed tooth eruption in congenital hypertrkhosis lanuginosa Deborah L. Franklin, PhD, MDent Sci, BDS, FDSRCS (Eng), MRCD (C) Graham J. Roberts, MDS, PhD, FDSRCS (Eng), BDS, MPhil ypertrichosis in childhood is found in a vari- Dental anomalies such as neonatal teeth, hypodontia, ety of conditions and may be localized or the presence of supernumerary teeth, and "defects" in generalized.' Localized hypertrichosis may be the enamel have been reported in association with hy- H 3 5 7 related to trauma, nevi, or spina bifida occulta. Gen- pertrichosis lanuginosa. ' ' The present case illustrates eralized hypertrichosis can occur with a variety of delayed eruption of primary and permanent teeth re- metabolic, chromosomal, and congenital disorders; sulting in unusual root morphology of primary molar these include Gorlin syndrome, Cornelia de Lange syn- teeth, and also enamel hypoplasia. drome, Leprechaunism, the porphyrias and muco- polysaccharidoses, trisomy 18, gingival hyperplasia Case report with hypertrichosis, and the congenital hypertrichoses. A male child was born of unrelated parents follow- Pre- or postnatal drug exposure with drugs such as glu- ing a normal pregnancy and delivery. He was the first cocorticoids, cyclosporin, and maternal alcohol abuse born and has an unaffected younger brother. The in pregnancy may also result in hypertrichosis. In the mother had taken no medication or vitamin/mineral congenital hypertrichoses, excessive hair growth is the supplements during the pregnancy. The child was cov- primary disorder. The terminology of these disorders ered in dense blonde lanugo hair at birth which was has been confused in the past but they have been de- particularly dense around the base of the spine and scribed as congenital hypertrichosis universalis, external auditory canals. The palms of the hands and transient congenital hypertrichosis universalis, and soles of the feet were free from hair growth. The scalp, congenital hypertrichosis lanuginosa.1 eyebrow, and eyelash hair were slightly coarser and The present case is one of congenital hypertrichosis darker than that on the rest of the body. At birth, the lanuginosa in which the body is covered with dense right cornea was noted to be cloudy as a result of glau- lanugo (fetal) hair at birth, which persists and is con- coma; this was treated with a trabeculotomy.10 During stantly renewed throughout life. Congenital the first year of life much of the hair fell out, but that hypertrichosis lanuginosa is very rare; a review of the on the face and ears persisted; this is shaved regularly literature in 1969 found reports of only 32 cases.2 The (Fig 1). There is no known maternal or paternal fam- inheritance is believed to be autosomal dominant with ily history of congenital varying expressivity2"7 and there are several families in hypertrichosis lanugin- which the disorder has been described in more than one osa. However the parents generation,3'5'6 with a four-generation pedigree in one and younger brother are Burmese family.5 Others have reported excessive hairi- "quite hairy", and the ness in previous generations,7 suggesting the idea of mother reports that she varying expressivity. The disorder is primarily an aes- has relatives who have a thetic one; the lanugo hair is constantly renewed large amount of head throughout life, but in some cases does appear to di- hair. Low intelligence minish with age.7 The growth of the hair is dense along and growth retardation the back, particularly at the base of the spine, and can have been reported pre- block the external nose and external auditory canals, viously in congenital causing apparent deafness. The only parts of the body hypertrichosis lanugin- free from hair are the palms of the hands and soles of osa.3 The child in our the feet. In the past, individuals with extremes of hy- case appears to have nor- pertrichosis of all types have been widely exhibited in al nt 1an d h sical Fig 1. Full face illustrating ™ ™ * P y side-shows and described in terms such as "dog-faced congenital hypertrichosislanuginosa development. 5 8 9 boy", "the nondescript", and "human werewolf'. ' ' (the face is shaved regularly). At the age of 2- no 192 American Academy ofPediatric Dentistry Pediatric Dentistry - 20:3, 1998 Fig 2a. Mandibular teeth showing pitting and discoloration of the incisal edges. Fig 2b. Maxillary teeth showing pitting and discoloration of the incisal edges. teeth had erupted; the gum pads were normal in ap- Discussion pearance but the maxillary pad was noted to be A variety of dental findings have been described in relatively large. By the age of 3-and-a-half the mandibu- relation to congenital hypertrichosis lanuginosa. These lar primary central incisors (71, 81) had erupted (Fig. include delayed eruption of permanent teeth, 2a) and by 5 years the maxillary primary central inci- hypodontia, neonatal teeth, microdontia, and the pres- sors (51, 61) were present. The child is now 6 years ence of supernumerary teeth.3'5'7 As there have been old and the upper right primary lateral incisor (52) has several reports of hypodontia associated with congeni- also erupted (Fig 2b). The teeth are chalky white with tal hypertrichosis lanuginosa, it has been suggested that pitting and brown discoloration of the incisal edges, there may be a hypodontia/congenital hypertrichosis particularly in the maxilla (Fig 2b); this suggests the lanuginosa syndrome.5 It is possible that teeth were possibility of an enamel defect. No treatment has yet present in some of the reported cases but failed to erupt been carried out and none is planned in the near fu- and could have been seen radiographically. One ture. Although the child appears to have developed case of congenital hypertrichosis lanuginosa has normally, both physically and mentally, he is quite shy been described in which there were no apparent and reluctant to cope with dental examinations. dental anomalies.8 The radiographs of the present case confirm the The present case exhibits delayed eruption of both presence of all primary teeth; the permanent incisors, deciduous and permanent teeth. At the age of 6-and- canines, and first molars (Fig 3). The unerupted pri- a-half years only five deciduous teeth have erupted, and mary molars are still completely covered in bone. The no permanent teeth. This is the stage of dental devel- first permanent molars, particularly on the right side, opment expected in a child younger than 1 year of age. appear closer to eruption than any primary molars. The In the present case, most of the permanent and decidu- roots of the mandibular primary molars are very close ous teeth are actually present. The teeth, particularly to the lower border of the mandible and the mesial and the deciduous molars, have failed to undergo eruptive distal roots have become divergent and splayed (Fig 3). movement within the alveolar bone. During the The maxillary primary molar roots are unclear on the intraosseous stage of tooth eruption, bone resorption radiograph. There is evidence that the maxillary sec- occurs over the crown of the tooth with bone forma- ond permanent molars are showing signs of tion beneath the roots forming an eruption pathway. mineralization. Between the mandibular primary mo- The dental follicle produces growth factors and en- lars there are triangular areas of altered radiodensity, zymes, which initiate much of this tissue turnover.11 It some of which extend from the alveolar crest to the is possible that the triangular areas of altered lower border of the mandible. radiodensity extending from the alveolar crest to the lower border of the mandible seen in the radiograph of this case represent extensions of the dental follicle. This may be an attempt to form an eruption pathway, which the teeth have failed to follow. This has resulted in splaying of the tooth roots. There is at present no evidence of the developing premolars and lower second permanent molars. Calci- fication of the crowns of the premolars is usually completed between the ages of 5 and 7 years and the second permanent molar crowns are often not calci- fied until the age of 8; it is therefore possible that these teeth may yet develop. Delayed eruption of the permanent teeth has previously been described in a case Fig 3. Panoral radiograph showing unerupted teeth and splaying of of congenital hypertrichosis lanuginosa,2 and delayed deciduous molar roots. Patient cooperation has not yet been sufficient for intraoral radiographs and photographs. tooth eruption occurs in children with other disorders Pediatric Dentistry -20:3, 1998 American Academy of Pediatric Dentistry 193 such as Downsyndrome, cleidocranial dysplasia, hy- Dr. Franklin is senior registrar, Paediatric Dentistry, Eastman popituitarism, hypothyroidism,and hereditary gingival Dental Hospital and Great OrmondStreet Hospital for Children hyperplasia associated with hypertrichosis. In cleido- and Dr. Roberts is joint professor of Paediatric Dentistry, East- cranial dysplasia, the deciduous teeth usually erupt man Dental Institute and Great Ormond Street Hospital for normally. In the permanent dentition, the delayed Children, London. eruption is often related to the numeroussupernumer- ary teeth which may be present. There is no References radiographic evidence of developing supernumerary l. Baumeister FA, Schwarz HP, Stengel-Rutkowski S: Child- teeth in the present case that maybe preventing the hood hypertrichosis: diagnosis and management.Arch Dis eruption of someteeth, but cooperation has not yet Child 72:457-59, 1995. been goodenough for intraoral radiographs to be taken. 2. Felgenhauer WR:Hypertrichosis lanuginosa universalis. J Genet Hum17:1-44, 1969. The enamelof the erupted teeth of the present case 3. Freire-Maia N, Felizali J, de Figueiredo AC, Opitz JM, appears to be hypoplastic. Enamelhypoplasia can oc- Parreira M, Maia NA:Hypertrichosis lanuginosa in a mother cur for a variety of reasons,12 including disorders and son.

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