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Guideline for Care of Patients With the Diagnoses of Craniosynostosis: Working Group on Craniosynostosis


Basic Questions

  1. What treatment is most indicated for maxillary hypoplasia (sagittal, vertical and transversal), including exorbitism?

  2. At what developmental stage is this treatment preferably performed?

  3. At what moment and in which way should associated hypertelorism be treated?

The focus of this document is on maxillary hypoplasia in syndromic craniosynostosis patients; the exorbitism is a consequence of this hypoplasia and is therefore not separately investigated, Life Balance v3.2.5 crack serial keygen. Furthermore, (correction of) hypertelorism is evaluated as part of the monobloc operation.


The Apert, Crouzon, and Pfeiffer syndromes are associated with hypoplasia of the maxilla, exorbitism, and hypertelorism. Indications for correction vary from acute vision impairment or respiratory problems to a relatively esthetic/psychologic problem. Various techniques are available to correct these deformities, and their timing greatly influences the eventual outcome.

Summary Literature

Syndromic craniosynostosis can be associated with skeletal hypoplasia of the midface, notably in the Apert and Crouzon/Pfeiffer syndromes. This benign hypoplasia can be present in 3 dimensions (sagittal, vertical, Life Balance v3.2.5 crack serial keygen, and transversal) and may result in restricted airway at the level of the nasopharynx (possible resulting in OSAS); exorbitism; malocclusion; and esthetic/psychosocial problems.

Although aspects of OSAS will be discussed in the Aftercare section of this guideline, the next section deals with the choice of surgical technique to correct the hypoplastic midface as well as the timing of surgery.

Corrections Shadow Of The Tomb Raider CPY Crack Full Download Latest PC Game the sagittal and vertical dimensions are discussed first, Life Balance v3.2.5 crack serial keygen, followed by those of the transversal dimensions.


Timing of the Surgical Correction and (Postoperative) Growth

As investigated by Posnick inthe face grows in 2 distinct periods. Craniofacial growth in the first 6 or 7 years is determined by growth of the brain, eyes, and the nasal cartilages. The second period starts after the seventh year, in which growth consists of bony surface apposition and deposition, development of the processus alveolaris and enlargement of the nasal cavities and paranasal sinuses. Analysis of CT-scans of nonsyndromic healthy patients shows that growth of the orbits and the nasal bone is largest between the third and fourth years, so that at the age of 5 years already 93% of the eventual dimensions is reached. This observation is likely to be important for the timing of corrective surgery. Although some authors suggest that further growth could be restricted by surgery, others report (very) slow unchanged growth after surgery in syndromic craniosynostosis patients (see below).

Bachmayer () determined growth of the midface in 19 surgically treated syndromic craniosynostosis patients (Apert, Crouzon, and Pfeiffer syndrome) aged from 6 to 15 years. Postoperative growth of the maxilla in sagittal direction was less than mm per year (negligibly small). In vertical direction, the growth was mm per year and thus comparable with the growth in a healthy control group and schisis patients. Meazzini concluded that the sagittal growth in untreated syndromic craniosynostosis patients was negligible, and if minimally present would not be adversely influenced by an operation. Kreiborg analyzed 8 patients with Crouzon or Apert syndrome on preoperative growth, stability of the Le Fort III osteotomy, and postoperative growth. Some vertical growth was supposed to occur, irrespective of surgical intervention, and as a result of remodeling and appositional growth rather than sutural growth. During 10 years&#x; follow-up (after Le Fort III osteotomy), no vertical or sagittal growth of the maxilla was observable.

Fearon compared postoperative growth of the midface after conventional osteotomy (N = 10) with that after a distraction operation (N = 12). Neither group showed postoperative horizontal and vertical growth. Fearon states that the disturbed maxillary growth in the syndromic craniosynostosis patients is intrinsically associated with the syndrome, and that this would not so much be the effect of an operation.

Correction of the Hypoplastic Midface: the Le Fort III Osteotomy

In syndromic craniosynostosis patients, notably those with Crouzon/Pfeiffer syndrome or Apert syndrome, the hypoplasia of the midface consists of a hypoplasia of the maxilla, Life Balance v3.2.5 crack serial keygen, nasal bone, Life Balance v3.2.5 crack serial keygen, and zygomatic bone bilaterally including the bony orbits. The choice of treatment is primarily aimed at correction of these underdeveloped anatomic structures. The Le Fort III osteotomy, as originally described by Gillies and Harrison in but further developed by Tessier in (including the variations Tessier described for the lateral orbital margin) is designed to correct all these elements by a single forward advancement. Usually by means of an approach from cranial, the entire midface (including the maxilla, nose bone, and caudal part of the bony orbits bilaterally) is wholly detached from the skull base and moved forward, Life Balance v3.2.5 crack serial keygen. Hollier described for the Le Fort III osteotomy a minimally invasive approach via local incisions (intraorally, upper, Life Balance v3.2.5 crack serial keygen lower eyelid).

Furthermore, a few publications are available on monobloc distractions (frontal bone including the Le Fort III part) in young patients, in whom osteotomy is not performed at all. , After surgical placement of the bone-borne distraction device, distraction is started. Liu et al described 4 patients (aged between 6 and 12 years) in whom a Le Fort III sutural distraction was performed (distraction without osteotomy), resulting in a mean 8 mm advancement. Life Balance v3.2.5 crack serial keygen et al reported on 4 syndromic craniosynostosis patients (all younger than 24 months) in whom midface advancement was obtained by means of a transfacial pin (K-wire) through both zygomatic bones connected percutaneously with an internal temporally fixed distractor bilaterally. All distractions are reported to have been successful.

Variants of the Le Fort III Osteotomy

InObwegeser presented several variants, including the combination of a Le Fort III and Le Fort I osteotomy in conjunction, and the butterfly (Le Fort III osteotomy without the nasal bone). Inthese osteotomic variants were again described by Kobus, although with application of external frame distraction. Ueki et al () reported on a Crouzon patient (15 year of age) in whom distraction with external frame of the midface and surgically assisted rapid maxillary expansion (SARME) was performed in conjunction. The osteotomy of the midface can be performed according to the design of Kuffner, a high Le Fort I up to and including the infraorbital margin and up to the zygomatic arc bilaterally.

Le Fort III and (Mal-) Occlusion: Indication for Additional Orthognatic Surgery

The Le Fort III procedure is primarily aimed at correction of the hypoplasia of the nasal bone, orbits, and zygomatic bones. The occlusion is of secondary importance here. Also because open bite is frequently present, Life Balance v3.2.5 crack serial keygen, additional orthognatic surgery to correct the malocclusion is often required. This is naturally preceded by a thorough (cephalometric) analysis in consultation with the orthodontist (including Life Balance v3.2.5 crack serial keygen treatment) (see this section of the guideline). It is recommended to plan the surgical intervention aimed at correction of the malocclusion after age 18 years in all patients. Tag: zemana antilogger crack a Le Fort III osteotomy after age 18 years is indicated (and the distance to be bridged is limited), this can be combined with a Le Fort I osteotomy.

Additional Periorbital Surgery

Patients with syndromic craniosynostosis (notably those with Apert syndrome) often show down slanting of the palpabral fissures (in which the lateral portion of the eye slit is lower than the medial portion), which Life Balance v3.2.5 crack serial keygen in a characteristic syndromic appearance. Several operative techniques are available to move the position of the lateral canthus to cranial, thus normalizing the down slanting. These procedures can be performed at the time of the distraction, at the time of possible orthognatic surgery, or separately. Occasionally, patients are satisfied with their facial appearance, or this surgical step is not at all indicated, and then further cosmetic interventions to the face are withheld.

The ventral movement of the midface, including the peri-orbital bony margin, is aimed at correction of the exorbitism, among other things. Nevertheless, after correction of the periorbital bony skeleton a soft tissue shortage of the lower eyelid may persist. Although all kinds of methods have been applied, such as local skin flaps, free skin transplants, Life Balance v3.2.5 crack serial keygen, and commercially available fillers, the Coleman lipofilling technique is increasingly used., After harvesting fat in places where this is abundantly available (such as abdomen, trochanter, and knee) the fat is centrifuged and next injected in the lower eyelid. It may be necessary to repeat this procedure several times to eventually obtain a good result.

Conventional Osteotomy or Distraction?

Application of distraction in the Le Fort III advancement is repeatedly underpinned with the argument that it allows for moving the hypoplastic midface over a larger distance to ventral (and possibly also to caudal) than with conventional osteotomy. This would lower the chance of needing a second intervention at later age after a first intervention during the Life Balance v3.2.5 crack serial keygen growth phase. Furthermore, distraction could prevent a number of disadvantages of conventional osteotomy, such as intermaxillary fixation and the additional harvesting of autologous bone transplants with risk of comorbidity.

Tables 6 and 7 present data of the Le Fort III osteotomy and distraction, respectively, including numbers of patients, distance achieved, age at time of operation, and relapse (if mentioned in the publication).


Conventional Le Fort III Osteotomy

NAdvancementMean AgeLong Term
Ousterhout2117&#x;f/u 5Stable
Bachmayer9Relapse % horizontal
% vertic.
David32Horizontal Relapse?

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Le Fort III Distraction

NAdvancementMean Age
HolemsLife Balance v3.2.5 crack serial keygen rowspan="1" colspan="1">1811
Mu89not mentioned
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