Full-Mouth Rehabilitation with Calvarium Bone Grafts and

Dental Implants for a Papillon-Lefèvre Syndrome Patient:

Case Report
Bassam M. Kinaia, DDS, MS1/Kristyn Hope, DDS2/Ahmed Zuhaili, MD, DDS3/Jean Francois Tulasne, MD3

Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder of keratinization associated with
palmoplantar keratoderma and severe periodontitis resulting in complete edentulism in late adolescence. The
pathognomonic dental features of PLS are pathologic migration, hypermobility, and exfoliation of the teeth
without any signs of root resorption. It has been suggested that an effective way to treat PLS patients presenting
early in the disease progression is extraction of the erupted primary dentition or hopeless permanent teeth
followed by antibiotic coverage with periodontal therapy for the remaining teeth. Unfortunately, studies have
shown that this regimen only temporarily delays the progression of periodontal disease and does not prevent
further tooth loss and bone destruction in the long term. Post–tooth loss, atrophic ridges make conventional
prosthodontic rehabilitation quite challenging, and more recently, implant-supported prostheses have been
considered as a viable alternative. In a PLS patient, implant placement is complicated by inadequate bone
volume; thus, bone augmentation techniques or the use of short implants is often considered. When large
volumes of bone are required, parietal calvarium bone can be used to predictably reconstruct severe defects. A
PLS patient aged 21 years presented a chief complaint of ill-fitting conventional complete dentures. The patient
had severely atrophic ridges, requiring significant bone augmentation for an implant-supported prosthesis. The
present case is the first example of bone augmentation using autogenous calvarium parietal graft followed
by endosseous implant placement and prosthetic restoration in a PLS patient. Int J Oral Maxillofac Implants
2017;32:e259–e264. doi: 10.11607/jomi.6282

Keywords: bone graft, case report, dental implants, Papillon-Lefèvre syndrome/rehabilitation

P apillon-Lefèvre syndrome (PLS) is a rare autosomal

recessive disorder of keratinization.1 Excessive kera-
tin deposition is observed most prominently on the
associated with PLS.2 With a prevalence of 1 to 4 cases
per million and no known sex or race predilection, this
condition does not present routinely in periodontal
palms of the hands and soles of the feet, also known practice; however, when it does, patients display sig-
as palmoplantar keratoderma. Genetic studies have nificant oral changes. Consanguinity of parents is as-
revealed that the loss of cathepsin-C function due to sociated with PLS incidence; however, there is rarely a
a gene mutation in chromosome 11q14.1 is strongly reported family history of disease.3 Upon eruption of
the primary dentition, the gingiva becomes inflamed,
and a rapid destruction of the periodontium follows,
1Director and Associate Professor, Department of resulting in premature loss of the deciduous teeth. The
Periodontology and Dental Hygiene, University of Detroit same process occurs during eruption of the permanent
Mercy School of Dentistry, Detroit, Michigan, USA; Private
dentition, resulting in complete edentulism in adoles-
Practice, Sterling Heights, Michigan, USA; French Dental
Clinic, Dubai Healthcare City, Dubai, UAE. cence.4,5 The pathognomonic dental features of PLS
2Periodontal Resident, Department of Periodontology and are pathologic migration, hypermobility, and exfolia-
Dental Hygiene, University of Detroit Mercy School of tion of the teeth without any signs of root resorption.4,5
Dentistry, Detroit, Michigan, USA. No difference has been reported in the oral bacte-
3Surgeon, Private Practice, French Dental Clinic, Dubai
rial flora of PLS-affected individuals compared with
Healthcare City, Dubai, UAE.
chronic periodontitis patients, with gram-negative
Correspondence to: Dr Bassam M. Kinaia, Department of cocci, rods, and spirochetes being common.6 Neutro-
Periodontology and Dental Hygiene, University of Detroit Mercy, phil phagocytosis, impaired bactericidal activity, and
2700 Martin Luther King Jr. Blvd, Detroit, MI 48208-2576, USA. decreased cell migration have been suggested as fac-
Email: kinaiaperio@gmail.com, kinaiabm@udmercy.edu
tors involved in the increased susceptibility to perio­
©2017 by Quintessence Publishing Co Inc. dontal disease and infection in PLS cases.7 The rapid

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Kinaia et al

periodontal destruction is not arrested by traditional MATERIALS AND METHODS

periodontal therapy alone, leading to significant
bone loss and severely atrophic arches.8 The literature Clinical Presentation
cites different management protocols for patients A male patient aged 21 years presented to the perio­
with PLS. Tinanoff et al reported effective therapy of dontal clinic at the Boston University Institute for
PLS in a primary dentition patient with recommen- Dental Research and Education (BUIDRE) with com-
dation of extractions of the erupted primary teeth plete edentulism. The patient presented with a con-
and antibiotic coverage. The 6- and 15-year follow- cave facial profile and complained of inability to
up showed no effect of PLS on the permanent denti- chew food properly and was interested in a dental
tion, with no detected polymorphonuclear leukocyte implant–supported prosthesis (Figs 1a and 1b). Medi-
(PMN) dysfunction.9,10 With regard to the PLS patient cal history revealed Papillon-Lefèvre Syndrome with
having permanent dentition, extractions of hopeless an otherwise unremarkable medical record. The pa-
teeth combined with antibiotic coverage, periodontal tient exhibited palmar and plantar hyperkeratosis on
therapy, and effective oral hygiene can slow the pro- the extremities and reported a difficult lifestyle (Figs
gression of periodontitis, but this is only temporary, 1c and 1d). Dental history revealed complete loss of
resulting in eventual tooth loss.11 teeth at age 13 years, with three sets of complete
Atrophic ridges make conventional prosthodontic maxillary and mandibular dentures over the years.
rehabilitation quite challenging, and more recently, Clinical examination revealed extremely atrophic
implant-supported prostheses have been considered ridges (Fig 1e). Radiographically, cone beam comput-
as a viable alternative.12 Implants can improve the sup- ed tomography (CBCT) showed primarily basal bone
port, stability, and retention of prostheses, but to date, that was inadequate for dental implant placement
there have been very few cases available in the litera- (Fig 1f ). The patient was referred to the oral surgeons
ture with successful application of implant therapy in at French Dental Clinic for cranium parietal bone
PLS patients.1,12–15 In a PLS patient, implant placement graft technique evaluation.18 Further radiographic
is complicated by inadequate bone volume. These evaluation using a medical computed tomography
patients generally have complete alveolar bone loss (CT) scan (Fig 2a) was conducted, revealing adequate
with only basal bone present. Thus, extensive bone bone skull density of the cortical external and internal
augmentation using extraoral sites is often employed. surfaces for cranium graft to reconstruct the atrophic
Extraoral sites may include tibia, ribs, iliac crest, or cal- maxilla and the mandible. Complete rehabilitation of
varium bone.16 Bone harvested from the calvarium the maxilla and mandible using a cranium graft for
contains thick cortical bone with minimal cancellous a dental implant–supported prosthesis was recom-
bone, resembling the bone characteristics of the max- mended, and the patient consented to treatment.
illofacial complex. The rigidity of the cortical bone cre-
ates excellent space maintenance to reconstruct the Cranium Parietal Bone Graft Surgery
deficient alveolar bone and can be supplemented with The cranium parietal bone graft surgery was per-
additional cancellous bone to fill all dead spaces.16 formed under general anesthesia with A.Z. and J.F.T.
Therefore, when large volumes of bone are required, The maxillary and mandibular recipient sites were
autogenous calvarium bone can be a predictable prepared, including bilateral maxillary sinus eleva-
source to reconstruct severe defects.16 Although an tion procedures. The patient cranium (donor site) had
extraoral surgical site and general anesthesia are re- adequate bone thickness for multiple cortical parietal
quired with calvarium grafting, major complications at bone grafts from the external surface (Fig 2b). The
the donor site are rare.17 The thickness of the cranial cranium mimics the bone characteristics in the maxil-
bones varies with age, exhibiting a thick pericranium lofacial area, and therefore, possesses similar resorp-
until age 3 years, becoming thinner and almost disap- tion rates compared with intraoral grafts. In addition,
pearing after age 40 years. Tessier reported in a case se- grafts harvested from the cranium result in less mor-
ries that some patients had extremely thin bone, while bidity such as pain, difficulty in walking, or potential
others had over 12 mm bone thickness, and up to 32 body deformity compared with grafts taken from the
mm in one case.16 The cranial bone thickness requires tibia or iliac crest.16 The parietal bone grafts were se-
preoperative assessment through three-dimensional cured to the maxillary and mandibular arches using
radiographic evaluation. The present case shows the surgical fixation screws (Fig 2c). Complete primary
first example of successful bone augmentation with closure was obtained for recipient and donor sites,
the use of autogenous calvarium parietal graft fol- and the patient was followed up in the hospital for 2
lowed by endosseous implant placement and pros- weeks before being released (Fig 2d). The area was al-
thetic restoration in a male PLS patient aged 21 years lowed to heal for an additional 3 months prior to re-
completed in faculty and private practice settings. entry for implant placement.

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 Kinaia et al

a b c

f
Fig 1  Initial patient presentation. (a) Extraoral facial profile front view. (b) Extraoral
facial profile lateral view. (c) Palmoplantar hyperkeratosis on hands. (d) Palmoplantar
hyperkeratosis on feet. (e) Clinical occlusal maxilla. (f) CBCT for maxilla.

Fig 2   (a) Medical CT scan of skull thick-

ness. (b) Donor site preparation of bone
strips. (c) Recipient site with graft strips
secured using fixation screws. (d) Two
weeks postsurgical maxilla.

a b

c d

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Kinaia et al

Fig 3  (a) CBCT 3 months after maxilla

graft. (b) Fixation screw removal. (c) Im-
plant placement. (d) Clinical view showing
closure after implant placement in the
maxilla.

b c d

Implant Surgery RESULTS

An additional CBCT using a radiographic guide was
taken, demonstrating adequate bone quantity and The implants were allowed to heal for an additional 3
quality for dental implant placement (Fig 3a). The months, and healing abutments were placed (Fig 4a).
3-month follow-up demonstrated excellent graft in- Thereafter, implants were torqued with good osseo-
tegration, and the patient was cleared for dental im- integration. Impressions were made, and an implant-
plant placement with B.M.K. On the day of implant supported prosthesis was fabricated and connected
surgery, the patient rinsed with a 0.12% chlorhexidine to the implants (Figs 4b and 4c). The 1-year follow-up
gluconate solution for 1 minute presurgically (Hi-Tech showed well-osseointegrated implants with good
Pharmacal Co). Local anesthesia was administered us- function with esthetic smile, and the patient was satis-
ing 2% lidocaine with 1:100,000 epinephrine (Empi). fied with the treatment outcomes (Fig 4d). The patient
Using a surgical guide, the sequential implant osteot- continued to be seen every 4 to 6 months for perio­
omy was initiated through the soft tissue without flap dontal and prosthesis maintenance. The results were
elevation to mark the implant sites. A midcrestal inci- satisfactory at the 12-month follow-up. This is the first
sion and full-thickness facial flap was reflected main- reported case of cranium grafting for endosseous im-
taining adequate keratinized gingiva around dental plant-supported full-arch prostheses in a PLS patient.
implants. The surgical fixation screws were removed,
and osteotomy preparation continued according to
the implant company surgical protocol (Figs 3b and DISCUSSION
3c). Nine implants (Legacy 3, Implant Direct) were
placed in the maxilla and six in the mandible, and Patients affected by PLS have to manage severe oral
flaps were approximated for closure using 4-0 resorb- disability from a very young age. This can negative-
able sutures (Vicryl, Medline Industries) (Fig 3d). ly affect growing children socially, esthetically, and

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 Kinaia et al

Fig 4   (a) Panoramic radiograph after im-

plant placement. (b) Intraoral facial view
of implant-supported prostheses. (c) Lat-
eral cephalogram of implant-supported
prostheses. (d) Esthetic smile at 1-year
follow-up.

b c d

physically. The present patient presented very frus- retain, or stabilize traditional removable prostheses. Au-
trated with the failure of three previous removable togenous bone harvested from the calvarium has been
prostheses and felt unable to function adequately in reported to provide adequate bone volume and contours
his day‑to‑day life. The reasons for severe rapidly pro- to allow for predictable vertical and horizontal bone aug-
gressing periodontitis in PLS patients have not been mentation.17 The use of cranium bone generally results in
fully elucidated; however, the possible mechanisms less morbidity compared with grafts taken from the tibia
have been classified into three domains: immunologic, or iliac crest.16 In a case series by Restoy-Lozano et al, a
microbiologic, and genetic.19 From the immunologic mean vertical bone gain of 5.04 ± 1.69 mm was obtained
perspective, neutrophils have displayed impaired che- in 10 patients.17 Provided that adequate bone volume is
motaxis, phagocytosis, and bactericidal activities.20,21 present or can be achieved, osseointegrated implants
Microbiologically, the presence of A actinomycetem- provide an alternative with major improvements in the
comitans has been noted in periodontal pockets of PLS long-term prognosis for oral rehabilitation.15,24 A grow-
patients, and could be considered as a triggering fac- ing body of evidence continues to suggest that PLS
tor.6,22 This finding has been expanded upon by Clere- patients should be considered candidates for full-arch
hugh et al, who identified a broader range of putative implant-supported prostheses.
perio­dontopathogens with presence in the oral cav-
ity of PLS-affected individuals, including F nucleatum
and P gingivalis.23 The investigation of genetic factors CONCLUSIONS
has led to the hypothesis that inactivation of the ca-
thepsin-C gene is primarily responsible for abnormali- Although the long-term stability of PLS cases treated
ties in the skin development and periodontal disease with implant-supported prostheses has not been es-
progression in PLS patients.4 This gene is also mutated tablished, the present case demonstrates that despite
in two related conditions: Haim-Munk syndrome and several failed attempts at prosthetic rehabilitation,
aggressive periodontitis. A clinical manifestation com- a potential treatment option for the restoration of a
mon to all of these disorders is early onset, severe, and severely atrophic arch consists of an interdisciplinary
rapidly progressing periodontal destruction. approach to treatment with autogenous calvarium
Implant dentistry has broadened the treatment op- grafts followed by endosseous implant placement and
tions for severely atrophic arches unable to support, implant-supported prosthesis fabrication. Treatment

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Kinaia et al

planning may appear daunting for cases complicated 8. Rateitschak-Plüss EM, Schroeder HE. History of periodontitis in a
child with Papillon-Lefèvre syndrome. A case report. J Periodontol
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Periodontol 1986;13:6–10.
treatment progression. This case was managed using 10. Tinanoff N, Tempro P, Maderazo EG. Dental treatment of
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11. Nickles K, Schacher B, Ratka-Krüger P, Krebs M, Eickholz P. Long-
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vre syndrome with dental implants. Saudi Med J 2013;34:424–427.
ACKNOWLEDGMENTS 13. Woo I, Brunner DP, Yamashita DD, Le BT. Dental implants in a
young patient with Papillon-Lefevre syndrome: A case report.
The authors declare no conflicts of interest. No author received Implant Dent 2003;12:140–144.
14. Senel FC, Altintas NY, Bagis B, et al. A 3-year follow-up of the reha-
monetary compensation for this manuscript.
bilitation of Papillon-Lefèvre syndrome by dental implants. J Oral
Maxillofac Surg 2012;70:163–167.
15. Ahmadian L, Monzavi A, Arbabi R, Hashemi HM. Full-mouth rehabili-
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