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Apert syndrome is a rare congenital disorder characterized by craniosynostosis, midface hypoplasia and symmetric syndactyly of hands and feet. Abnormalities associated with Apert syndrome include premature fusion of coronal sutures system (coronal sutures and less frequently lambdoid suture) resulting in brachiturricephalic dismorphism and impaired skull base growth. After this brief explanation it is clear that these anatomical abnormalities may have a negative impact on the ability to perform essential functions. Due to the complexity of the syndrome a multidisciplinary (respiratory, cerebral, maxillo-mandibular, dental, ophthalmic and orthopaedic) approach is necessary in treating the psychological, aesthetic and functional issues. The aim of this paper is to analyse the different functional issues and surgical methods trying to enhance results through a treatment plan which includes different specialities involved in Apert syndrome treatment. Reduced intellectual capacity is associated to the high number of general anaesthesia the small patients are subject to. Therefore the diagnostic and therapeutic treatment plan in these patients has established integrated and tailored surgical procedures based on the patients’ age in order to reduce the number of general anaesthesia, thus simplifying therapy for both Apert patients and their family members.
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Treatment timing and multidisciplinary approach in Apert syndrome
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                                                                                                              Case report



    Treatment timing and multidisciplinary approach
    in Apert syndrome




                                                                                                            i
                                                                                                          al
                                                                                                       on
    MariaTeresa Fadda, MD, DDS, PhD1                               anatomical abnormalities may have a negative im-
    Gaetano Ierardo, DDS, PhD2                                     pact on the ability to perform essential functions.
    Barbara Ladniak, DDS, PhD2                                     Due to the complexity of the syndrome a multidis-
    Gianni Di Giorgio, DDS, PhD2                                   ciplinary (respiratory, cerebral, maxillo-mandibu-
    Alessandro Caporlingua MD1                                     lar, dental, ophthalmic and orthopaedic) approach




                                                                                             zi
    Ingrid Raponi, MD1                                             is necessary in treating the psychological, aesthet-
    Alessandro Silvestri, MD, DDS3                                 ic and functional issues. The aim of this paper is to
                                                                   analyse the different functional issues and surgical




                                                                               na
    Group of Apert syndrome, Policlinico Umberto I, “Sa-           methods trying to enhance results through a treat-
    pienza” University of Rome, Italy                              ment plan which includes different specialities in-
                                                                   volved in Apert syndrome treatment. Reduced in-
    1   Department of Oral and Maxillofacial Sciences, Max-        tellectual capacity is associated to the high num-
                                                                   ber of general anaesthesia the small patients are




                                                                     er
        illofacial Surgery Unit, Policlinico Umberto I, “Sapien-
        za” University of Rome, Italy                              subject to. Therefore the diagnostic and therapeu-
    2   Department of Oral and Maxillofacial Sciences, Pe-         tic treatment plan in these patients has established
        diatric Unit, “Sapienza” University of Rome, Italy         integrated and tailored surgical procedures based



                                                             t
    3   Department of Oral and Maxillofacial Sciences, Or-         on the patients’ age in order to reduce the number
                                                          In
        thognathodontic Unit, “Sapienza” University of Rome,
        Italy
                                                                   of general anaesthesia, thus simplifying therapy
                                                                   for both Apert patients and their family members.

                                                                   Key words: Apert syndrome, multidisciplinary ap-
    Collaborators: A. Polimeni4, M. Bossù4, E. Barbato4,           proach, congenital disorders.
                                             ni
    G. Ierardo4, A. Silvestri4, A. Pizzuti4, A. De Luca4, V.
    Guida4, A. Giancotti4, C. Moretti4, P. Papoff4, A. Spa-
    lice4, F. Ursitti4, G. Gualdi4, C. Di Biasi4, C. Andreoli4,    Introduction
                                   io

    C. Colaiacono4, R. Delfini4, A. Santoro4, A. Caporlin-
    gua4, F. Caporlingua4, M. De Vincentis4, A. Greco4,            Apert syndrome is a rare congenital disorder charac-
    P. Cascone4, V. Valentini4, M.T. Fadda4, C. Ungari4,           terized by craniosynostosis, midface hypoplasia and
    M. Gharbiya4, M. Marenco4, B. Carlesimo4, A. Spa-              symmetric syndactyly of hands and feet. Apert syn-
                          iz



    gnoli4, V. Mazzone4, M. Amabili4, L. Silvestrini4              drome was first reported by Wheaton in 1894 and a
                                                                   French paediatrician, Eugene Apert, published a se-
                                                                   ries of nine cases in 1906.
           Ed




    Corresponding author:                                          Apert syndrome is estimated to affect 1 in 160,000 live
    Maria Teresa Fadda                                             births. It is most frequently caused by a de novo muta-
    Department of Oral and Maxillofacial Sciences,                 tion in the male gameter. Two missense mutations in
    Maxillofacial Surgery Unit, Policlinico Umberto I              the fibroblast growth factor receptor 2 (FGFR2) gene
    “Sapienza” University of Rome                                  on chromosome 10 (1), have been found to account for
    Via Caserta 6                                                  the disorder in approximately 98% of patients (1): the
IC




    00161 Rome, Italy                                              p.P253R mutation accounts for 33% of the cases and is
    E-mail: mariateresa.fadda@uniroma1.it                          associated with more severe syndactyly when com-
                                                                   pared to other mutations that cause Apert syndrome:
                                                                   p.S252W, which accounts for 66% of cases is strongly
C




    Summary                                                        associated with cleft palate (2). These mutations lead
                                                                   to loss of ligand specificity of receptor, causing abnor-
    Apert syndrome is a rare congenital disorder                   malities in extracellular matrix composition and prema-
    characterized by craniosynostosis, midface hy-                 ture calvarial ossification. The syndrome may be inher-
©




    poplasia and symmetric syndactyly of hands and                 ited in an autosomal dominant trait. In these cases, ad-
    feet. Abnormalities associated with Apert syn-                 vanced parental age is often associated with a higher
    drome include premature fusion of coronal su-                  risk in having an affected child (3). With the present
    tures system (coronal sutures and less frequently              work we emphasize the need of an integrated and mul-
    lambdoid suture) resulting in brachiturricephalic              tispecialistic approach to the syndrome. We want to de-
    dismorphism and impaired skull base growth.                    fine main therapeutic steps performed by our group on
    After this brief explanation it is clear that these            patients affected by Apert syndrome.

    58                                                                                Annali di Stomatologia 2015; VI (2): 58-63
    Treatment timing and multidisciplinary approach in Apert syndrome


    Clinical features                                                A                   B

    Abnormalities associated with Apert syndrome in-
    clude premature fusion of the coronal sutures system
    (coronal sutures and less frequently lambdoid suture)




                                                                                                              i
    resulting in brachiturricephalic dismorphism and im-




                                                                                                            al
    paired growth of the skull base. When the lambdoid
    suture is involved, the resulting underdevelopment of
    the posterior cranial fossa may evolve toward an




                                                                                                         on
    Arnold-Chiari malformation with chronic tonsillar her-        Figure 3. A and B Severe syndactyly of a hand with com-
    niation and increased risk of hydrocephalus (4). Hy-          plete fusion of all five digits at the level of the terminal and
    drocephalus is associated with raised intracranial            middle phalanges, the hand has a cup-like appearance.
    pressure leading to clinical manifestations such as
    somnolence, irritability, vomiting, sixth cranial nerve       In addition to midface hypoplasia, the pharynx is small




                                                                                               zi
    palsy and cephalalgia. Other typical findings are mal-        and the bony nasal cavity is narrowed. These abnor-
    formations of brain gyri and heterotopia of the gray          malities may cause severe respiratory distress, espe-
    matter, ventriculomegaly, the malformations of limbic         cially during the neonatal period when patients are




                                                                                na
    structures and an ipoplastic or even absent corpus            nasal breathers. Therefore most neonates require air-
    callosum (5), absence or cystric septum. Patients             way interventions such as tracheostomy. Older pa-
    may present or develop intervertebral fusion, mainly          tients should be screened with polisomnograms for ob-
    involving the cervical spine.                                 structive sleep apnea (8). Conductive hearing loss is




                                                                    er
    In the orbital region there are retrusion of the upper        common in Apert syndrome. This results from chronic
    or lower orbital margin, associated with hyper-               otitis media and occasionally from middle ear anom-
    telorism, eyelid antimongoloid, proptosis, strabismus         alies (9). The ears are set low and may be enlarged.
    and eyebrow cleft (Fig. 1). Orbital retrusion in Apert        Bilateral symmetrical complex syndactyly of hands
    syndrome may extend to the midface, causing maxil-


                                                           t      and feet always occur with this syndrome. The hands
                                                        In
    lary hypoplasia with a V-Shaped maxillary dental arch
    (6). An anterior open bite with Angle class III maloc-
                                                                  have the following four common features regardless
                                                                  of severity (Fig. 3):
    clusion is also common. The palate is high and nar-           1. Complex syndactyly exists between the index,
    row with lateral hypertrophic swellings (Fig. 2). Cleft-           middle and ring fingers
    ing of the soft palate or uvula occurs in 30% of pa-          2. The thumb is shortened, with the proximal or di-
                                                 ni
    tients. Delayed or ectopic tooth eruption, cross bite              stal segment deviated radially (clinodactyly)
    and dental crowding are common (7). The nose has a            3. The fourth interdigital space exhibits simple syn-
    flattened dorsum and a small bulbous tip.                          dactyly
                                    io

                                                                  4. The phalanges exhibit brachyphalagism.
                                                                  At birth the cuneiform bones are well segmented.
                                                                  Progressive fusions interest the tarsal fist, but during
                                                                  childhood it will progressively involve the metatarsal
                           iz



                                                                  shafts and eventually involve fusions between distal
                                                                  and proximal phalanges of toes.
                                                                  Most patients with Apert syndrome have neurologic
          Ed




                                                                  involvement. Slow intellectual development occur in
                                                                  approximately 50% of cases, however most of these
                                                                  patients have only mild intellectual deficiency.


                                                                  Treatment plan
IC




    Figure 1. Orbital dystopia, hypertelorism, eyelid antimon-    After this brief explanation it is clear that these anatomi-
    goloid and globous nose in a patient affected by Apert syn-   cal abnormalities may have a negative impact on the
    drome.                                                        ability to perform essential functions.
C




     A                                           B                                  Figure 2. Intraoral features in Apert syn-
                                                                                    drome. A: cleft palate, V-Shaped maxil-
                                                                                    lary dental arch, shovel-shaped incisors;
©




                                                                                    B: open bite, Angle class III malocclusion,
                                                                                    crossbite, dental crowding, eruption
                                                                                    anomalies.




    Annali di Stomatologia 2015; VI (2): 58-63                                                                                 59
    M.T. Fadda et al.


    Due to the complexity of the syndrome a multidiscipli-       of age, while posterior vault expansion is performed
    nary (respiratory, cerebral, maxillo-mandibular, den-        at 6-12 months. Monobloc advancement or Le Fort III
    tal, ophthalmic and orthopaedic) approach is neces-          osteotomy and facial bipartition (Fig. 5) are per-
    sary in treating the psychological, aesthetic and func-      formed at 6-7 years (10).
    tional issues.                                               Oberoi et al. advice to perform frontal-orbital advance-




                                                                                                             i
    We believe that the best functional and aesthetic results    ment at 6-12 months and midface Le Fort III advance-




                                                                                                           al
    can be achieved only through an integrated multidiscipli-    ment at 9-12 years (11). Correction of syndactyly
    nary approach on the bases of the experience gained at       treatment is recommended at 13 months (12) for sep-
    the centers of cranio-facial surgery. Other considera-       aration of the finger to gain manipulation movement.




                                                                                                        on
    tions deal with high number of operations that must be       Cleft palate repair should be performed in this period
    performed at an early age, during growth and at the end      to rehabilitate speech and swallow disorders.
    of development. It has been shown that the high number
    of general anaesthesia in small patients is to be corre-
    lated with intellectual limitations and with the offset of




                                                                                                 zi
    cognitive impairment. This data gives the chance to re-
    flect on both, the need to anticipate or postpone the
    planned measures and on the need to perform multiple




                                                                               na
    operations at the same time to reduce the number of
    general anaesthesia. The aim of the paper is to analyse
    the different functional problems and the different surgi-
    cal methods trying to optimize results according to a




                                                                   er
    timetable that provides the integration of different spe-
    cialities involved in the Apert syndrome treatment.
    We decided to divide treatment plan in three steps:
    - step 1 — from birth to age 2
    - step 2 — growth period


                                                           t
    - step 3 — adult age.
    The latter will be described for each age group with
                                                        In
    relative diagnostic and treatment options in order to        Figure 4. Fronto-orbital advancement.
    optimize the protocol.
                                           ni

    From birth to age 2
                                 io

    At birth and in the first weeks cerebral, respiratory
    and ocular bulb emergencies should be taken into ac-
    count, particularly in severe cases of Apert syndrome.
    For these patients immediate surgical treatment is re-
                          iz



    quired in order to prevent or correct papilledema,
    corneal ulcers, severe respiratory distress and inter-
    cranial hypertension. It is fundamental to start inte-
         Ed




    grated counselling with all medical team involved in
    the diagnosis and treatment protocol: genetists, neu-
    ropediatricians, pediatricians, maxillo-facial surgeons,
    neurosurgeons, hand surgeons, ophthalmologists.
    Working together they guarantee a valid and multidis-
    ciplinary support to Apert families.
IC




    Surgery in Apert cases is important with respect to
    patient’s age, the degree of case-specific craniofacial
    dysmorphism and neuro-anatomical deformity. In this
    period cranioplasty and/or posterior vault expansion
C




    associated with a fronto-orbital advancement (Fig. 4)
    should be performed in order to guarantee the physi-
    ological vault expansion.
    Surgical options and timing are still debated in litera-
©




    ture: Fearon and Podner prefer to post-pone cranial
    vault expansion to the 15th month in cases of non ad-
    verse clinical features and at 5-9 years for second
    cranial vault expansion with separate Le Fort III dis-
    traction, if necessary (5).
    Allam et al., at the Los Angeles medical center, prefer
    to perform frontal-orbital advancement at 4-6 months         Figure 5. Facial bipartition.

    60                                                                                 Annali di Stomatologia 2015; VI (2): 58-63
    Treatment timing and multidisciplinary approach in Apert syndrome


    Growth Period                                                   vancement than traditional advancement and may re-
                                                                    duce complications such as meningitis.
    During growth (up to age 12) both the transversal di-           For our patients treatment we always use an external
    mension of the orbital region (hypertelorism and anti-          distraction device (Fig. 6B-7). In the past internal dis-
    mongolodin slant of the palpebral fissure) and retru-           tractions were widely used (Fig. 6C), however from




                                                                                                              i
    sion of the middle third must be corrected. In the first        our experience and in accordance with literature (8,




                                                                                                            al
    case surgeons may perform an orbital mobilization. In           17), the use of an external halo device offers greater
    the second case a Le Fort III osteotomy with external           advancement and better vector control.
    distraction can be performed (Fig. 6). Facial biparti-          Between 2 and 7 years, it is essential to follow cogni-




                                                                                                         on
    tion by external distraction may be performed (13-15)           tive and language development especially after cleft
    with good results, hypertelorism and midface flatten-           repair. This is particularly beneficial for rehabilitation
    ing are associated (Fig. 7).                                    with lingual devices.
    Since growth of the midface (especially in Apert syn-           In addition during this period the following must be
    drome) is not predictable, sometimes distraction ad-            performed:




                                                                                                zi
    vancement should be repeated at a later age to catch            - eye surgery, correction of strabismus after the fi-
    up with mandibular growth. Thus we believe that mid-                nal positioning of the orbits
    face advancement should be delayed at a later age in            - hand and foot surgery preferably after 4-5 years




                                                                                  na
    order to obtain predictable results and to reduce the           - dental and orthodontic therapies to monitor teeth
    surgical load.                                                      eruptions, prevent caries, guide the eruption and
    If the patient has adequate projection of the superior or-          allow dental alignment.
    bital ridge, Le Fort III osteotomy is the chosen proce-         Hand, eye and dental surgery can be carried out si-




                                                                       er
    dure, whereas monobloc advancement, which advances              multaneously in order to reduce the number of gener-
    the forehead simultaneously with the midface, may pro-          al anaesthesia to which the patient must be exposed.
    duce better results if the forehead remains retruded (16).      Oral clinical features in Apert syndrome are: maxillary
    Le Fort III or monobloc distraction provide greater ad-         ipoplasia, bifid uvula, Byzantine-arch palate associat-



                                                            t
     A                                               B
                                                         In                                        C
                                                 ni
                                    io
                           iz
          Ed




    Figure 6. Middle third advancement A:Le Fort III osteotomy line; B: external distractor device applied in a patients after a
    Le Fort III procedure. C: internal distractor device applied in a patient after Le Fort III osteotomies.


     A                                           B                                    Figure 7. Third middle advancement in a
                                                                                      8 year-old child with external distraction.
                                                                                      A: Pre surgical, B: Post surgical outcome.
IC
C
©




    Annali di Stomatologia 2015; VI (2): 58-63                                                                                61
    M.T. Fadda et al.


    ed with lateral swellings of the palatine process, den-
    tal plaque, dental calculus, congestion and swelling
    of the gingival and periodontal pseudo pockets, max-
    illary dental crowding, shovel-shaped incisors, dental
    agenesis and early dental loss (18).




                                                                                                              i
    In literature despite big interest in genetics and treatment




                                                                                                            al
    of the syndrome, today no emphasis is given to preven-
    tion and endodontic-conservative treatment. Often be-
    cause of the patients’ hand malformations unsatisfactory




                                                                                                         on
    oral care and dental problems occur. Early dental treat-
    ment consists in instructions for both patient and parents.
    It is important in our opinion to communicate the impor-
    tance of avoiding dental plaque, carious process that
    may cause peridontitis, pulpitis and dental loss.




                                                                                               zi
    Dentists should evaluate the patients’ efficiency and          Figure 9. Orthodontic presurgical treatment. In the picture it
    autonomy in using oral care devices and suggest the            could be noticed the presence of a palate incisor.
    use of electric toothbrush, chlorhexidine mouthwash




                                                                                na
    twice a week. Regular check-ups and treatment with
    fluoride are also suggested (19). After having reached         cranio-facial complex are combined with bone dis-
    a good level of oral care, patients could initiate en-         traction method.
    dodontic-conservative treatment with materials and             At the end of growth our surgical planning provides




                                                                     er
    techniques compatible with the disease.                        ancillary procedures necessary to adjust soft tissues
                                                                   and new skeletal architecture (rhinoplasty, lipofilling,
                                                                   plastic eyebrow, revision of the songs, autologous
    End of growth/Adult age                                        grafts, implants Medpor).



                                                            t
    Other surgical procedures are planned at the end of
    growth and concern the lower third of the skeleton
                                                         In        Conclusions
    and the ancillary procedures necessary to adjust the
    new skeletal architecture.                                     In conclusion it can be said that diagnostic and thera-
    For surgical treatment of skeletal Class III accompa-          peutic planning in patients with Apert syndrome em-
                                            ni
    nied by open bite, the use of standard techniques              phasizes the need to integrate various specialities.
    such as Le Fort I osteotomy and sagittal split osteoto-        It is necessary to frame the patient’s clinical picture
    my of the mandible (Fig. 8) is needed. It is important         upon his arrival.
                                  io

    to note that the mandible is not particularly advanced         Very important are also a multi-disciplinary plan and
    and that both Class III and open bite are mainly due           clinical programmed check-ups in accordance to a ra-
    to maxilla malformation.                                       tional therapeutic clinical widely agreed upon and
    The Orthodontist in the pre and post surgical phase is         verified in its efficiency.
                         iz



    essential in this age group.                                   As noted in this paper establishing an integrated and
    Close monitoring of teeth eruption in Apert syndrome           tailored surgery timing, scheduling, combining and
    is necessary due to their need for individualized plan-        coordinating actions to be taken at different stages of
         Ed




    ning of dental element alignment (Fig. 9). Sometimes           the patient’s age reduces the number of general
    surgical interventions that provide individualizes os-         anaesthesia thus simplifying therapy for both Apert
    teotomies in order to normalize the architecture of the        patients and their families (Tab. 1).
IC




     A                                       B                                       Figure 8. Inferior Third surgery: A: Le Fort
                                                                                     I osteotomies and advancement; B: Bilat-
                                                                                     eral split osteotomy of mandible.
C
©




    62                                                                                  Annali di Stomatologia 2015; VI (2): 58-63
    Treatment timing and multidisciplinary approach in Apert syndrome


    Table 1. Surgical timing in Apert syndrome.                                    a case with emphasis on oral manifestations. J Dent. 2011
                                                                                   Spring;8(2):90-95.
    Life period                       Surgical treatment                      8.   Moore MH. Upper airway obstruction in the syndromal cran-
                                                                                   iosynostoses. Br J Plast Surg. 1993 Jul;46(5):355-362.
    Within two yrs                    Emergencies                             9.   de Jong T, Toll MS, de Gier HH, Mathijssen IM. Audiologi-
                                      Cranioplasty




                                                                                                                             i
                                                                                   cal profile of children and young adults with syndromic and
                                      Cleft Palate Correction




                                                                                                                           al
                                                                                   complex craniosynostosis. Arch. 2011 Aug;137(8):775-
                                      Hand Surgery                                 778.
    Growth period                     Middle Third Correction                10.   Allam KA, Wan DC, Khwanngern K, Kawamoto HK, Tanna
                                      Strabismus Correction                        N, Perry A, et al. Treatment of apert syndrome: a long-term




                                                                                                                        on
                                      Hand And Foot Surgery                        follow-up study. Plastic and Reconstructive Surgery.
                                                                                   2011;127:1601-1611.
                                      Orthodontic Treatment
                                                                             11.   Oberoi S, Hoffman WY, Vargervik K. Craniofacial team man-
    End of growth                     Inferior Third Correction                    agement in Apert. Am J Orthod Dentofacial Orthop. 2012;Vol.
                                      Individualized Surgery                       141:S82-7 .




                                                                                                             zi
                                      Ancillary Techniques                   12.   Mazzone V. Apert’s syndactyly: strategies in surgical treat-
                                                                                   ment. Riv Chir Mano. 2006;2:124-127.
                                                                             13.   Mulliken JB, Bruneteau RJ. Surgical correction of the cran-




                                                                                             na
                                                                                   iofacial anomalies in Apert syndrome. Clin Plast Surg.1991
                                                                                   Apr;18(2):277-289.
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                                                                               er
     2. Heuzé Y, Singh N, Basilico C, Jabs EW, Holmes G,                     15.   Mulliken JB, Kaban LB, Evans CA, Strand RD, Murray JE.
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                                                                     t             G. Simultaneous Le Fort I, II, and III osteotomies for correction
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        131:132-142.                                                               May;103(5):e38-4l.
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     7. Vadiati Saberi B, Shakoorpour A. Apert syndrome: report of                 Tehran. 2011;Vol. 8(2):90-5.
          Ed
IC
C
©




    Annali di Stomatologia 2015; VI (2): 58-63                                                                                                  63
