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Atlas Oral Maxillofacial Surg Clin N Am 10 (2002) 101130

Management of syndromic craniosynostosis


Sittachai Tantipasawasin, DDSa, Douglas P. Sinn, DDSa,*, Ghali E. Ghali, DDS, MD, FACSb
Division of Oral and Maxillofacial Surgery, University of Texas, Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390-9109, USA b Division of Oral and Maxillofacial Surgery (Head and Neck Surgery), Louisiana State University Medical Center, 1501 Kings Highway, P.O. Box 33932, Shreveport, LA 71130-3932, USA
a

Syndromic craniosynostosis has several inherited causes. Crouzon, Apert, and Per syndromes are the most common, although individual patients have unique presentations. Although they are of the same syndrome, there are wide variations of cranial appearance and levels of malformation. For easier diagnosis and treatment purposes, Tessier divided the craniofacial framework into ve levels (Fig. 1) and classied craniofacial synostosistopographically and anatomicallyinto six groups [1] (Table 1). Because craniofacial surgery is high risk, for safety reasons and good outcomes patients are best managed by a craniofacial team that includes a neurosurgeon, a craniofacial-plastic surgeon, a maxillofacial surgeon, an anesthesiologist, an ophthalmologist, an otolaryngologist, and an orthodontist. Surgical management has multiple approaches for the same syndromes depending on the level of the existing malformation and its position on the craniofacial framework. Each level of the craniofacial framework has a group of surgical procedures that are specic to certain malformations. Surgical options for correction of the malformation at each level of the craniofacial framework are shown in Table 2. The major goals of treatment include 1. Preventing brain compression, optic nerve compression/cornea injury, and psychosocial problems. 2. Promoting normal development of craniofacial structures such as brain, skull, facial bones, and muscle. 3. Decreasing craniofacial malformities by establishing nearly normal appearance including facial symmetry, facial proportion, and facial balance. 4. Improving breathing via increased nasopharyngeal and oropharyngeal airway space. 5. Decreasing morbidity and mortality. Timing of treatment The timing of reconstruction is divided into three major steps that are referred to as staged reconstruction. 1. Primary cranioorbital decompression-cranial vault reshaping in infancy; supraorbital rim advancement (ORA), anterior cranial vault reconstruction (ACVR), and posterior cranial vault reconstruction at 6 to 12 months up to 2 years.

* Corresponding author. E-mail address: douglas.sinn@utsouthwestern.edu (D.P. Sin). 1061-3315/02/$ - see front matter 2002, Elsevier Science (USA). All rights reserved. PII: S 1 0 6 1 - 3 3 1 5 ( 0 1 ) 0 0 0 0 6 - 3

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Fig. 1. Five levels of Tessiers craniofacial framework. Level A, cranial vault. Level B, orbitofrontal unit. Level C, lower orbit with body of mixilla and zygomas. Level D, upper jaw. Level E, mandible.

Table 1 Tessiers topographic and anatomic classication of craniofacial synostosis Tessiers classication Class Class Class Class Class Class 1: 2: 3: 4: 5: 6: isolated cranial vault dysmorphism syndromic orbitocranial dysmorphism asymmetric orbitocranial dysmorphism Saethre-Chotzen group Crouzon group Apert group Levels of malformation level A level B levels B and C levels AC levels AD levles AE

2. Management of midface deformity in childhood (57 years). 3. Management of the jaw deformity and malocclusion in adolescence (1318 years). In the rst stage, primary cranioorbital decompression cranial vault reshaping, the objectives are correction or prevention of brain compression and correction of the craniofacial framework at levels A and B. This stage of treatment should be done before 2 years of age. For skull healing and reshaping reasons, the authors prefer to operate at 6 to 12 months of age. In severe cases that show signs of increased intracranial pressure, an earlier operation is necessary. Repeated craniotomy for additional cranial vault decompression and reshaping may be required if there are signs of increased intracranial pressure (Fig. 2) or if the forehead and superior orbit are not satisfactorily corrected in shape or position before the second stage of treatment. In the second stage, management of the midfacial deformity, the goals of treatment are to correct midface hypoplasia, including the malar area, normalize the infraorbital rim-globe

S. Tantipasawasin et al. / Atlas Oral Maxillofacial Surg Clin N Am 10 (2002) 101130 Table 2 Surgical treatments related to level of malformation Level of malformation A Surgical options

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D E

primary craniotomies 1. anterior cranial vault reconstruction 2. posterior cranial vault reconstruction 3. strip craniotomy orbitofrontal unit reconstruction 1. rotation for correction of frontonasal angle 2. anteroposterior correction 3. transverse correction: widening or narrowing midface hypoplasia and normal upper face 1. extracranial LeFort III osteotomy/distraction 2. LeFort II osteotomy/distraction 3. augmentation midface hypoplasia and abnormal upper face 1. monobloc procedure 2. one-stage procedure: ACVR/ORA and LeFort III osteotomy 3. two-stage procedure: ACVR/ORA and LeFort III osteotomy hypertelorism 1. bifacial partition procedure 2. intracranial four-wall osteotomy maxillary procedures [2,3] mandibular procedures [2,3]

Fig. 2. Radiographic evidence of increased intracranial pressure. (A) Finger printing or beaten copper in plain lm. (B) Moth eaten in three-dimensional.

relationship, and prevent globe and cornea injury. This stage of treatment is usually done at 5 to 7 years of age, regardless of the occlusion. After operation, patients usually have a remaining malocclusion, open bite, or an atypical jaw relationship. This problem is addressed in the nal stage. Management of jaw deformities and malocclusion in adolescence occurs in the nal stage. The aim of treatment is to establish a normal jaw relation and proper occlusion. Orthognathic surgery is used via maxillary or mandibular procedures or both. The authors prefer to complete this correction at age 13 to 18 years. Specic detail relating to jaw correction is beyond the scope of this article.

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This article focuses on the correction of normal or abnormal upper face (Tessier levels A and B) and malformations of the midface (Tessier level C). The details of correction of anterior cranial vault malformations or posterior cranial vault malformations are discussed in the article on management of nonsyndromic craniosynostosis. Most syndromic craniosynostosis patients present with midfacial hypoplasia, orbital hypoplasia, and mild to severe hypertelorism or orbital dystopia. Many surgical procedures address midfacial hypoplasia problems, including 1. One-stage procedure: ACVR/ORA and LeFort III osteotomy (Fig. 3) 2. Two-stage procedure: ACVR/ORA and LeFort III osteotomy (Fig. 4) 3. Monobloc procedure (Fig. 5)

Fig. 3. One-stage procedure: ACVR/ORA and LeFort III osteotomy. The authors prefer to use this technique for mild to moderate deformities in an untreated child. (A) Preoperative tracing. (B) Area of reconstruction. (C) New position of forehead and midface. Bone gaps are lled with bone grafts.

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4. Extracranial LeFort III osteotomy/distraction (Fig. 6) 5. LeFort II osteotomy/distraction It is important to remember that the goals of this step of treatment are to advance the midface and normalize the orbital globe and orbital rim relationship. Additional facial proportion and balance are established. The position of orbitofrontal unit (Level B) and shape of forehead (Level A) must be evaluated before choosing the surgical technique. If Tessier levels A and B and orbital globe-orbital rim relationship are normal, the LeFort II procedure is the operation of choice. If Tessier levels A and B are normal for a mild deformity

Fig. 4. Two-stage procedure: ACVR/ORA then LeFort III osteotomy. The rst stage is ACVR/ORA. The deformities at Level A and B of Tessiers craniofacial framework are normalized at 6 to 12 months, up to 2 years. (A) Preoperative. (B) Areas of reconstruction. (C) New positions of forehead (Levels A and B). The second stage is LeFort III osteotomy to correct Level C deformities in a 5- to 7-year-old child (D).

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Fig. 5. Monobloc procedure: Osteotomies establish a single moved bony segment, including orbitofrontal unit, midface, and maxilla. (A) Anteroposterior views. (B) Lateral view.

but orbital globe-orbital rim relationship is abnormal, Lefort III procedure is the operation of choice. When the magnitude of midface advancement is more than 10 mm, the authors prefer to move the midface segment via distraction osteogenesis. If Tessier levels A, B, and orbital globe orbital rim relationships are poor, a monobloc procedure, either as a one-stage or two-stage procedure with or without ACVR/ORA and LeFort III osteotomy is chosen. The authors prefer two-stage procedures to a one-stage or monobloc procedure.

Surgical approach for two-stage procedure: anterior cranial vault reconstruction orbital rim advancement then LeFort III osteotomy/distraction Basic surgical sequence for craniofacial surgery Step 1. Anesthesia Oroendotracheal intubation is preferred, and the tube is secured adjacent to the incisal edge of the mandibular central incisors with wire (Fig. 7A). Nasoendotracheal intubation is used if the jaw relationship or occlusion is addressed. In difcult cases, oral to nasal intubation is chosen. Hypotensive anesthesia is recommended with control of mean arterial blood pressure between 50 and 55 mm Hg. Two units of packed red blood cells are prepared for use if needed. Step 2. Cranial approach A coronal incision is preferred. The authors put a Z-incision (Fig. 7B) at the supraauricular area to decrease wound tension and create a less visible scar. In the anterior cranial vault operation or midface operation, the authors prefer to place the incision more anteriorly, which provides the best exposure to the orbit and lateral part of Level C. Lidocaine (0.5%) with epinephrine (1:200,000) is injected to facilitate hemostasis. Raney clips are applied. Additional approaches such as infraorbital rim approach, transconjunctival incision with or without lateral

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canthotomy, subciliary incision, or lower midlid incision, and intraoral approach via upper vestibular incision may be required. Step 3. Scalp reection The anterior scalp ap is elevated at a subpericranium level down to the lateral orbital rim and supraorbital rim. The supraorbital nerve is released from its bony canal with 2 mm osteotome. Subperiosteal dissection is continued to expose the zygomatic arches and dorsum of the nose. The temporalis muscles are elevated and the lateral orbital wall is exposed (Fig. 8). For LeFort III osteotomy, the dissection is continued along the lateral wall of the maxillary sinus until it reaches the pterygomaxillary junction. Using an infraorbital rim approach, exposure of the medial wall of orbit, oor, and lateral wall of orbit is completed.

Fig. 6. Subcranial osteotomy designs of LeFort III osteotomy: Anteroposterior and lateral views. (A,B) Tessier I. (C,D) Modied Tessier I (vertical zygomatic split). (E,F) Modied Tessier II.

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Fig. 6 (continued )

Anterior cranial vault reconstruction and orbital rim advancement Step 4. Outline drawing The forehead is divided into two separate units (See Fig. 11F ). Superior orbital rim-lateral orbital rim unit or orbital bandeau is outlined rst (Fig. 9). The height of the orbital bandeau is approximately 1 to 1.5 cm. The remainder is superior forehead component and includes the malformation area. Posteriorly, barrel stave lines are drawn. If superior cranial width needs

Fig. 7. (A) Oroendotracheal intubation is preferred. (B) Z-incision at supraauricular area.

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Fig. 8. Coronal incision exposes supraorbital rim, dorsum of nose, lateral orbital wall, roof of orbit. The temporalis muscle is elevated. (A) Anteroposterior view. (B) Lateral view.

expansion, a vertical bandeau is used between the superior forehead component and the barrel staves (Fig. 10). Step 5. Craniotomy Six bur holes are completed. The craniotome is used for the craniotomy of the skull. The superior forehead component is removed rst. The orbital bandeau is cut as shown in Fig. 11. The orbital bandeau includes the orbital roof 3 to 5 mm from the orbital rim. It is cut from medial to lateral via a transcranial approach. If the superior orbital rim-lateral orbital rim unit needs to move anteriorly or correct a transverse discrepancy, it is cut free from the anterior cranial attachment.

Fig. 9. Anterior cranial vault reconstruction and orbital rim advancement procedure. (A,B) Osteotomy design; lateral view and 45 view. (C) Orbital rim advancement and anterior cranial vault reconstruction. (D) Fixation with Lactosorb plate and screw system.

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Fig. 9 (continued )

Step 6. Bone graft harvest Bone grafts are harvested by splitting the cranial bone ap with saw or chisel (Fig. 12). Bone dust is collected during the craniotomy process. Step 7. Superior orbital rim-lateral orbital rim unit reconstruction Morphology and position of the superior orbital ridge-lateral orbital rim unit is the key element for reconstruction (Fig. 13) of the forehead and midface in the future. The ideal supraorbital rim in the adult is located 8 to 10 mm anterior to the cornea [4]. The angle between supraorbital ridge to nasal bone at the level of frontonasal suture is 90 to 110. From the birds eye view, the rim should arc posteriorly to achieve a gentle 90 at the temporal fossa. Lactosorb (Lorenz) plates and screws are used for xation.

Fig. 10. Osteotomy design for ACVR/ORA. (A) With vertical bandeau. (B) Without vertical bandeau.

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Fig. 11. Orbital bandeau osteotomy. (AC) The direction and angulation of reciprocating saw. (D) The hand piece is in 45 inferiorly and the saw blade is inserted just beyond the height of the greater wing of sphenoid bone. Malleable retractors are used to protect the brain visually. (E) The anterior cranial base, 3 to 5 mm behind supraorbital rim, is cut from medial to lateral via the transcranial approach. (F) Two units of forehead: superior forehead component (A) and orbital bandeau (B).

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Fig. 12. Bone graft harvest. (A) Splitting the cranial bone ap with saw and/or chisel. (B) Bone paste (left) and hydroxyapatite (right).

Step 8. Superior forehead component reconstruction Cranial bone aps are tried in dierent positions to create a curved smooth forehead. A bone bender is used to contour the bone ap (Fig. 14). Selective osteotomies with a reciprocating saw and selective grinding with a bur are used as needed. From the lateral view (Fig. 15A,B), the forehead curve is approximately 60 and levels out at the coronal suture. Frontal prominence is established approximately 25 to 30 mm above the supraorbital rim. Lactosorb plates and screws are used for xation. Step 9. Grafting The bone gaps are lled with autogenous split-thickening cavalium grafts and bone dust. If there is not enough bone grafting then demineralized bone is used. Hydroxyapatite is used for additional onlay contouring of the forehead (Fig. 15C,D). The authors prefer mixing hydroxyapatite with blood and Avitene (Davoc/Bard) to create a paste for easier manipulation. Step 10. Closure Temporalis muscles are repositioned with 4-0 nylon sutures (Fig. 15D). The scalp is closed in two layers. The skull is allowed to consolidate and remodel roughly for 1 year before moving the midface (extracranial LeFort III osteotomy/distraction) (Figs. 16 and 17). The authors prefer to use a vertical zygomatic split technique to maintain zygomatic stumps for xation or placement of a distractor. Extracranium LeFort III osteotomy Step 11. Planning The goal of this treatment is to advance the midface and normalize the orbital globe-orbital rim relation (Fig. 18). In the adult, the cornea is 12 mm anterior to lateral orbital wall and 0 to 3 mm behind the infraorbital rim. The vector of movement is parallel to the Frankfort horizontal plane (Fig. 19). 20% overcorrection is recommended. Step 12. Splint preparation A splint is made to cover the palatal and occlusal one third of teeth. It is used during mobilization of the midface segment with the disimfaction forcep, which prevents a palatal soft tissue injury, dental injury, and accidental palatal fracture.

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Fig. 13. Superior orbital rim-lateral orbital unit is the key for reconstruction of forehead and midface. Supraorbital rim is located 8 to 10 mm anterior to the cornea, and supraorbital ridge-nasal bone angle is 90 to 110. (A,B) Rib bender is used for supraorbital ridge-nasal bone angle correction when orbital bandeau attaches to base of skull. (C,D) Orbital bandeau is detached from base of skull for establishing ideal position and angulation of superior orbital rim-lateral orbital unit. (E,F) Orbital bandau is sagittal split for correction anterior cranial width, widening or narrowing.

Basic steps for craniofacial surgery Anesthesia, surgical approaches, and ap reection are the same as in steps 1 to 4. Step 14. LeFort III osteotomy: vertical zygomatic split technique A vertical cut at the body of zygoma is completed by reciprocating saw. The cut divides the frontal process of zygoma into an anterior part and posterior part equally. These cuts on the right side and left side must be parallel and perpendicular to the vector of movement (Frankfort horizontal plane) (Fig. 20). The horizontal cut is made 5 mm below the frontozygomatic suture by reciprocating saw. The direction is parallel to vector of movement as it moves toward orbital

Fig. 14. Selective osteotomies and contouring of bone ap with rib bender.

Fig. 15. Superior forehead component reconstruction. (A,B) Anteroposterior view and lateral view of anterior cranial vault reconstruction and orbital rim advancement with Lactosorb plates and screw xation. (C,D) Hydroxyapatite with blood and avitene mixture is used to contour the forehead and establish the frontal prominence as needed.

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Fig. 16. Dressing for 24 hours.

cavity. A cut is completed behind the lateral orbital rim 8 to 10 mm, and the direction of osteotome is toward inferior orbital ssure. This cut is made by reciprocating saw via supratemporal fossa with careful globe protection. From inferior orbital ssure, the posterior wall of maxillary sinus is cut posteriorly and inferiorly toward the inferior one third of pterygomaxillary junction by an osteotome. The horizontal cuts at nasal bone are completed 5 mm below nasofrontal suture by a reciprocating saw. The direction is posterior inferior above medical canthal ligament and nasolacriminal apparatus. After nishing the nasal bone cut behind the posterior lacrimal crest, the direction of osteotomy is inferior and parallel to posterior lacrimal crest and it moves toward inferior orbital ssure. This step is completed by an osteotome (Fig. 21). The inferior one third of pterygomaxillary junction is separated by curve osteotome bilaterally. This step can be approached via temporal fossa (Fig. 22A) but usually is easier via the intraoral approach (vestibular incision). The nasal septal osteotome is used to separate nasal septum from cranial base. The direction of osteotome is parallel to the base of the skull and directed toward the posterior nasal spine. Step 15. Midface mobilization A splint prepared in the laboratory is inserted to protect the palate and injury to the dentition. Maxillary disimpaction forceps are applied to mobilize the midfacial segment until the planned distance is achieved without tension (Fig. 22B). Step 16: Fixation or distractor placement When the magnitude of midface advancement is more than 10 mm, the authors prefer to move the midface segment via distraction osteogenesis (Figs. 23 and 24). Distractors are placed

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Fig. 17. A 5-month-old patient with Apert syndrome. (A,B) Preoperation. (C,D) Four years s/p ACVR and ORA procedure.

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Fig. 18. Extracranial LeFort III osteotomy/distraction. (A) Preoperative drawing. (B) Modied Tessier I (vertical zygomatic split) technique. (C) LeFort III osteotomy (<10 mm)/LeFort III distraction (>10 mm).

Fig. 19. Vector of movement is parallel to the Frankfort horizontal plane. (A) Diagnostic tracing. (B) Prediction tracing.

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Fig. 20. LeFort III osteotomy line: vertical zygomatic split technique. (A,B) Horizontal nasal cut: 5 mm below nasofrontal suture, above medial canthus ligament and nasolacrimal groove, stop just posterior to posterior lacrimal crest. (B,C) Medial wall, oor, and lateral wall of orbit cut: inferior orbital ssure is the key landmark. Connect the cut from posterior lacrimal crest, move toward the anterior end of the inferior orbital ssure, move laterally to join with horizontal cut at frontal process of zygoma: 5 mm below the frontozygomatic suture and 8 to 10 mm behind the lateral orbital rim. (C) Vertical cut of the body of zygoma is perpendicular with Frankfort horizontal plane and divides the frontal process of zygoma into anterior part and posterior part. (D) Lateral wall of maxillary sinus cut: the osteotomy line connects between the anterior part of inferior orbital ssure and pterygomaxillary junction 10 to 15 mm cephalically. Pterygomaxillary junction separation.

at the zygomatic stump and body of malar bone (Fig. 23C,D). If LeFort III osteotomy is planned, oral to nasal reintubation is performed. A splint is used to set the position of the midfacial segment. After the midfacial segment is set in position, 2-0 plates and screws are used to stabilize at frontozygomatic processes, zygomatic processes, and nasal bones.

Step 17. Bone grafting Bone defects at the zygomatic processes, frontozygomatic processes, nasal bones, and oor of orbits are grafted with locally harvested cranial bone or ribs.

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Fig. 21. LeFort III osteotomy: Tessier I procedure. (A) Superiorlateral orbital rim osteotomy by reciprocating saw. (B) Lateral orbital wall osteotomy by saw and osteotomes. (C) Body of zygoma osteotomy by reciprocating saw. (D) Orbital oor osteotomy by small osteotome.

Fig. 22. (A) Pterygomaxillary junction separated by curve osteotome via temporal fossa approach. (B) Midface mobilization by maxillary disimpaction forceps.

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Hypertelorism Hypertelorism is a condition that is characterized by an abnormal increase in interorbital and interpupillary width. This distance varies depending on race. Normally, the soft tissue interorbital distance ranges from 30 to 34 mm (bony interorbital distance is approximately 28 mm). Any measurement that is more than 40 mm (bony interorbital distance more than 35 mm) is considered abnormal. Correcting this problem is planned before the patient is of school age, approximately 5 to 7 years old. Intracranial medial four-wall translocation (Fig. 25) is the standard technique that the authors use for hypertelorism correction (Fig. 31). In cases in which the deformity from hypertelorism is less severe, the extracranial approach for orbital rearrangement is used.

Fig. 23. A 9-year-old patient with Crouzon syndrome was treated with LeFort III distraction osteogenesis. (A,B) Preoperative appearance (front, side). (C,D) Distractor placement. (E to H) s/p 19 mm LeFort III distraction osteogenesis (front, side, top, bottom).

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Fig. 23. (continued )

Surgical method for hypertelorism correction with intracranial four-wall osteotomy Step 1. Anesthesia Anesthesia preparation is the same as for craniofacial surgery.

Step 2. Nasolacrimal apparatus prophylaxis Crawford nasolacrimal tubes are inserted (Fig. 26). The tubes are maintained for 10 to 12 weeks or until swelling is reduced and nasolacrimal systems are patent.

Step 3. Medial canthal tendon identication The 5-mm skin incision along the epicanthal fold is performed 7 mm medial to medial palpebral ssure. Blunt dissection techniques is used to localize medial canthus and, with 4-0 clear nylon, they are marked for later use (Fig. 27).

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Step 4. Surgical approach The coronal incision and transconjunctival incisions with lateral canthotomies are completed. Periorbita (2530 mm from orbital rim) are reected to free orbital contents from orbital bony wall. Vestibular incisions are used to expose the anterior wall of maxillary sinus and piriform rim up to the infraorbital nerve.

Fig. 24. A 7-year-old patient with Crouzon syndrome (s/p ACVR/ORA) was treated with LeFort III distraction osteogenesis. (A,B) Preoperation (front, side). (CH) s/p 15 mm Lefort III distraction osteogenesis (front, side, top, bottom). (G) Preoperative radiograph. (H) Postoperative radiograph.

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Fig. 24 (continued )

Step 5. Outline drawing Step 6. Osteotomy Frontal craniotomy is completed for access to anterior cranial base (Fig. 28). A horizontal cut 10 mm above the supraorbital rim is completed. At the superior-lateral rim, the osteotomy line follows the curve of the lateral wall of orbit and splits the frontal process of zygoma as it reaches the body of malar bone at the level of infraorbital nerve (Fig. 29A,B). The excess bone from the middle of the nasal area is removed, which leaves 9 to 10 mm of frontal processes of maxilla from the anterior lacrimal crest to the medial wall of the orbit (Fig. 29C). Bilateral complete ethmoidectomies are completed, keeping the cribiform plate intact (Fig. 29D). The roofs and medial wall of orbit are cut by reciprocating saw approximately 25 mm behind orbital rim via cranial approach. Osteotomies of the lateral walls of orbit are completed via temporal fossa approach. The oor of the orbits is cut by osteotome via

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Fig. 25. Hypertelorism correction: intracranial four-wall osteotomy. (A) Preoperative appearance. (B) Bifrontal craniotomies are performed. A 1-cm frontal bar is established. The central block of bone is removed. Ethmoidectomy is addressed. Orbital four-wall osteotomies are completed. (C) Medial repositioning of the orbital segments and xation. (D) Bone grafts and nasal reconstruction.

the transconjunctival approach 25 mm behind orbital rim at the level of inferior orbital ssure. All osteotomy lines are connected to one another. The horizontal cut at the anterior wall of maxillary sinus connects to the osteotomy from zygomatic body to piriform rim at the level below the infraorbital nerve and inferior turbinate. From the piriform rim, the lateral nasal wall osteotomy is completed with a nasal osteotome. The four walls of orbit are mobilized from associated bone.

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Fig. 26. Nasolacrimal apparatus prophylaxis. (A) Crawford lacrimal intubation set. (B) Nasolacrimal punctum is dilated by nasolacrimal dilator. (C) Guide wise is passed from superior punctum and inferior punctum to nasolacrimal duct. (D) Artery forceps are used to receive the guide wire.

Step 7. Medial translocation of orbit The orbital segments are mobilized and repositioned medially. Interorbital bony distances are measured. The orbits are placed level and symmetrical. Overcorrection is recommended to the interorbital bony distance of 18 to 20 mm (Fig. 29E,F).

Step 8. Rigid xation 2-0 titanium plates and screw system is used to stabilize the orbital segment, as shown in Figure 29G.

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Fig. 27. Medial canthal tendon identication. (A) 3- to 5-mm skin incision along the epicanthal fold, 5 mm medial to palpebral ssure, is performed. The medial canthal ligament is identied by blunt dissection. (B) 4-0 clear nylon is used for localization.

Fig. 28. Intracranial four-wall osteotomy lines. (A,B) Supraorbital cut is 10 mm above supraorbital rim. Infraorbital cut is just below the infraorbital nerve toward piriform at inferior meatus level. Vertical nasal cut is 9 mm medial to anterior lacrimal crest from subraorbital cut to piriform rim. Orbital cut is 25 mm behind orbital rim. (C) Inferior orbital ssure is a key landmark.

Step 9. Frontal sinus ablation The remaining frontal sinus soft tissue is removed. Pericranial aps with brin adhesive are used to separate anterior cranial fossa from nasal cavity (Fig. 29HJ). The frontal craniotomy ap is returned to position shaped and xed with resorbable Lactosorb plates and screws (Fig. 29K).

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Step 10. Medial canthopexy Each medial canthus is tied with 28 gauge stainless steel wire (Fig. 30). The wire is passed to opposite medial walls of the orbit at the posterosuperior point of the posterior lacrimal crest and secured. Soft tissue interorbital distance is measured and the medial canthal ligament nylon sutures are (from step 2) pexed into the underlying soft tissue for additional support.

Fig. 29. Intracranial four-wall osteotomy procedure. (A,B) Supraorbital cut by reciprocating saw with malleable retractor protection, (C,D) Excess intercanthal bone is removed and ethmoidectomy is completed. (E,F) The orbital blocks are mobilized and medially repositioned. Bony intercanthal distance is assessed (1820 mm). (G) 2-mm titanium plates and screws system is used for xation. (HJ), Pericranial ap and brin adhesive are used to disconnect cranionasal communication. (K) Bone aps are returned.

Fig. 29 (continued )

Fig. 30. Medial canthopexy. (A) 28 grade wire is used to secure medial tendons. (B) Medial canthopexy is performed with two separate wires for independent control of respective canthal tendons.

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Fig. 31. A 15-year-old patient with frontonasal syndrome was treated with intracranial four-wall osteotomy and nasal reconstruction. (A) Preoperative view. (BD) 1 week after operation from three views (front, side, top).

Step 11. Nasal bone reconstruction Most hypertelorism patients require nasal bone reconstruction. The details are in the bibliography of recommended readings. Summary Normalization of craniofacial malformation in syndromic craniosynostosis patients is an extended process. The care starts at birth and ends in adolescence. Multiple surgeries may be

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required for each deformity. The relationship among patient, family, and doctor team is important because they must understand the complete processes of management. Psychologists provide an important support mechanism.

Acknowledgments The authors would like to acknowledge two people who stimulated their thinking over the years: Craig P. Hobar (craniofacial plastic surgeon, University of Texas, Southwestern Medical Center) and Frederick Sklar (paediatric neurosurgeon, Childrens Hospital, Dallas, TX). With their talents, skills, great experiences, and dedication, the care and management of syndromic craniofacial synostosis patients have been possible at our center.

References
[1] Cohen Jr. MM, Maclean RE. Craniosynostosis: diagnosis, evaluation, and management. 2nd edition. New York: Oxford University Press; 2000. [2] Bell WH. Modern practice in orthognathic and reconstructive surgery. Philadelphia: WB Saunders Company; 1992. [3] Epker BN, Stella JP, Fish LC. Dentofacial deformities: integrated orthodontic and surgical correction. 2nd ed. St. Louis: MosbyYear book; 1995. [4] Salyer KE, Bardach J. Salyer and Bardachs atlas of craniofacial & cleft surgery. Philadelphia: Lippincott-Raven Publishers; 1999.