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Multiple Suture Nonsyndromic Craniosynostosis


Introduction

As previously discussed in other chapters, single suture nonsyndromic craniosynostosis has the highest prevalence of the craniosynostosis, with sagittal synostosis being the most common followed by metopic, coronal, and lastly, lambdoid stenosis. In the multiple suture synostosis group, bilateral coronal synostosis (typically associated with a craniofacial syndrome) is the most common type. There is yet another subset of multiple suture synostosis that does not appear to be associated with a known syndrome and may include two or three sutures. All of the patients present at or soon after birth and develop rapidly progressive cranial and facial deformities. However, these patients grow to develop normally and do not appear to have association with a well-known craniofacial syndrome. Prior to our introduction of minimally invasive endoscopic procedures to treat this subgroup of patients, conventional wisdom dictated that the patient be allowed to reach the age of 10 to 12 months before undergoing treatment. Unfortunately, the rapid growth phase that occurs during this waiting period, often leads to severe cranial and facial deformities which makes successful treatment more difficult to obtain. Often, the complex nature of the untreated multisuture deformity leads surgical teams to recommend staged surgical procedures to treat the complex abnormalities. Our success with treating single suture synostosis with endoscopic techniques led us to apply these procedures to the treatment of a combination of stenosed sutures in young patients. Other authors have followed with similar results. At first the goal was simply to perform a quick, minimally invasive procedure to release the closed sutures with the goal of halting the ongoing deformation and then proceed with a standard calvarial vault reconstruction at a later date. However, we became extremely surprised when we noticed that not only was the deformity progression being halted but that actual correction was taking place without the ultimate need for a calvarial vault remodeling. In this chapter we present our surgical approach and our short- and long-term results treating this cohort of patients utilizing endoscopic techniques for the treatment of multisuture synostosis. The group can be divided into seven categories: (1) sagittal and coronal synostosis; (2) sagittal and metopic synostosis; (3) sagittal and bilambdoid synostosis; (4) bilateral lambdoid synostosis; (5) coronal and metopic synostosis; (6) sagittal, coronal, and lambdoid synostosis; and (7) sagittal and bicoronal synostosis. Clinical presentation and radiographic findings will be discussed.

Sagittal and Coronal Synostosis

As would be expected, a combination of sutural stenosis which involves the sagittal and a coronal suture will lead to a recognizable phenotypic presentation with combination of both pathologies. The clinical features include: (A) vertical dystopia; (B) nasal deviation to contralateral side of coronal stenosis ( Fig. 14.1 ); (C) sagittal plane imbalance/scoliosis ( Fig. 14.2 ); (D) recession of forehead and orbital rim ipsilateral to affected coronal suture ( Fig. 14.3 ); (E) severe frontal bossing contralateral to coronal stenosis; and (F) scaphocephaly ( Fig. 14.4 ). Radiographic features are seen in Figs. 14.5–14.7 .

Fig. 14.1, One-month-old male with right coronal and sagittal synostosis. The right orbit is elevated producing vertical dystopia. Significant compensatory left frontal bossing is evident. The nasal tip deviates to the left.

Fig. 14.2, Severe depression of the right pterion, right supraorbital rim recession, and scaphocephaly are seen.

Fig. 14.3, Anteroposterior view of same infant shows significant contralateral frontal compensatory bossing as well as right orbit elevation and dystopia.

Fig. 14.4, Top view of 3-month-old male with right coronal and sagittal synostosis. Evident are the following findings: scaphocephaly, right frontal plagiocephaly, nasal tip deviation to the right, skull base angulation at anterior fontanelle as seen by the divergence of the red and green lines.

Fig. 14.5, Three-dimensional computed tomography scan of a 2-month-old female with sagittal and right coronal craniosynostosis. Submental-vertex view depicts the classic right frontal plagiocephaly and scaphocephaly. Also noted is the side deviation from midsagittal plane towards the left side.

Fig. 14.6, Axial computed tomography of a sagittal/coronal synostosis patient demonstrates dolichocephaly and frontal plagiocephaly.

Fig. 14.7, Axial computed tomography scan of 4-month-old male with sagittal and right coronal craniosynostosis demonstrates normal brain architecture. Noted is the scaphocephaly associated with deviation from the midsagittal plane towards the side contralateral to the stenosed coronal suture.

Positioning

In order to have complete access to the front and back of the head, the patient is placed in the modified prone (sphinx) position. The overall set up is the same as described in detail in the sagittal synostosis chapter ( Chapter 8 ).

Incisions

The incisions used to treat this group also resemble the ones made in the sagittal synostosis group. The only difference is that the anterior incision is made at the level of the anterior fontanelle ( Fig. 14.8A ). Also, the anterior is made a bit longer and extends more towards the affected coronal sutures. Subgaleal dissection is made as described in the sagittal chapter. However more dissection is done over the affected suture and should reach all the way to the pterion. If the anterior incision is not sufficient to reach the ipsilateral squamosal suture, a second incision on the side can be made as seen in Fig. 14.8B .

Fig. 14.8, (A) Location of the incisions for removal of the vertex and release of the stenosed right coronal suture. (B) If access to the ipsilateral pterion and squamosal suture cannot be easily achieved, a third incision can be added halfway between the fontanelle and the pterion.

Osteotomies

The amount of bone removed to treat the sagittal component is similar as to that described in the sagittal chapter ( Chapter 8 ). The amount removed is inversely proportional to the age of the patient. The vertex craniectomy is performed and the barrel stave osteotomies of the lambdoid sutures and the nonaffected coronal suture are performed as previously described. The primary difference is that the ipsilateral frontal wedge osteotomy that is placed over the stenosed coronal suture is extended all the way down to the pterion and should reach the squamosal suture, as described in the coronal chapter ( Chapter 9 ) ( Fig. 14.9 ). A skull radiograph obtained after surgery shows the amount and extent of bone removal ( Fig. 14.10 ). As with all other surgeries, the patients are placed in custom-made cranial orthoses which keep the anterior/posterior dimension constant during first 2 months while allowing the depressed temporal area and recessed forehead and orbital rim to expand and move into a normal position.

Fig. 14.9, The green shaded area illustrates the amount of bone removed for treatment of sagittal/coronal synostosis. The osteotomies are very similar to the sagittal synostosis surgery except that the affected coronal suture osteotomy is taken all the way down to the ipsilateral squamosal suture, as depicted.

Fig. 14.10, Lateral skull radiograph after release of the sagittal and coronal sutures. The coronal osteotomy on the affected side is marked with red dots (A) and extends all the way down to the pterion/squamosal suture. The contralateral coronal osteotomy is labeled with (B), whereas (C) and (D) show the lambdoid osteotomies.

Results

There were seven patients, five males and two females. The mean age at time of surgery was 2.6 months and the median was 2.2 months. The mean estimated blood loss was 25 mL. There were no intraoperative or postoperative blood transfusions. The mean surgical time was 88 minutes. All patients were discharged on postoperative day (POD) 1. There were no complications. The preoperative cranial deformities underwent early and significant correction which has persisted long-term as evidenced by the patient in Clinical Case 1 ( Figs. 14.11–14.15 ). The other patients have achieved similar results ( Figs. 14.16–14.18 ).

Fig. 14.11, (A) Two-month-old male presented with right coronal and sagittal craniosynostosis. This preoperative frontal photograph shows the classic findings associated with this sutural stenosis combination which includes vertical dystopia and nasal tip deviation to the contralateral side. (B) Top view demonstrates scaphocephaly and occipital elongation. (C) Left lateral view shows significant left frontal bossing and prominence. (D) Right lateral view demonstrates orbital complex retraction and horizontal dystopia with recession of right supraorbital rim and forehead.

Fig. 14.12, The Patient is Positioned in the Sphynx Position.

Fig. 14.13, (A) Six-and-a-half-month postoperative frontal view. (B) Six-and-a-half-month postoperative top view. (C) Six-and-a-half-month postoperative left lateral view. (D) Six-and-a-half-month postoperative right lateral view.

Fig. 14.14, (A) Four-year postoperative frontal view. Continued correction and improvement of vertical dystopia is noted. (B) Four-year postoperative top view shows marked correction of frontal plagiocephaly and asymmetry.

Fig. 14.15, (A) Seventeen-year postoperative frontal view. The facial asymmetry has rebalanced with almost complete resolution of vertical dystopia. Patient does not report dystopia or visual disturbances. (B0 Seventeen-year postoperative top view. Complete correction of frontal and forehead deformity has been accomplished. Both supraorbital rims are aligned and symmetric. (C) Seventeen-year postoperative left lateral view. Complete normalization of the profiled has been achieved. Normal frontal sinus development is noted. (D) Seventeen-year postoperative right lateral view. Right forehead and supraorbital rim have moved forward and align with the left side.

Fig. 14.16, (A) Preoperative frontal view of a 4-week-old male with right coronal and sagittal synostosis. (B) Preoperative right lateral view. Scaphocephaly and frontal bossing is seen. (C) Preoperative top view. (D) Preoperative submental view shows typical deformity consisting of vertical dystopia, nasal deviation, contralateral frontal bossing, and depressed right pterion.

Fig. 14.17, (A) Five-year postoperative frontal view demonstrates completed correction of cranial and facial deformities. (B) Five-year postoperative right lateral view. (C) Five-year postoperative left lateral view shows a normal side profile. (D) Five-year postoperative top view depicts forehead normalization and scaphocephaly correction.

Fig. 14.18, Six-year postoperative 3-dimensional computed tomography scan. Osseous features have fully normalized. Nose is midline. Orbits are aligned at mid-horizontal axis. Forehead is symmetric. Patient developed hydrocephalus and had a right frontal up shunt placed. A small cranial defect surrounding the shunt entrance required a custom cranioplasty. The patient is developmentally normal.

Coronal and Metopic

This sutural stenosis combination causes deformational changes that affect the face, forehead, and cranium. The following clinical features are seen: (A) curved midline-forehead ridge ( Fig. 14.19 ); (B) vertical dystopia on the side of the coronal synostosis; (C) deviation of the forehead ridge toward the affected coronal suture ( Fig. 14.20 ); (D) nasal deviation away from coronal suture ( Fig. 14.21 ); (E) sagittal imbalance pivoted at the anterior fontanelle ( Fig. 14.22 ); and (F) asymmetric trigonocephaly with associated plagiocephaly of the affected coronal suture ( Fig. 14.23 ). The patients are typically irritable and uncomfortable most likely due to brain pressure from the multiple suture synostosis. Skull radiographs will show harlequin’s eye and closed metopic suture on the same patient ( Fig. 14.24 ). Computed tomography (CT) scans show marked skull base deviation of the anterior cranial base towards the affected coronal ( Figs. 14.25–14.29 ).

Fig. 14.19, Frontal view of a 2-month-old female with metopic and right coronal synostosis. The dotted forehead markings denote a curved prominent forehead ridge. Note nasal deviation to the left side and vertical dystopia.

Fig. 14.20, Three-month-old female with metopic and right coronal synostosis shows similar deformational pattern as patient in Fig. 14.19 .

Fig. 14.21, Top view shows right coronal stenosis with forward movement of the stenosed suture. Nasal deviation and right forehead recession are noted as well as right sided proptosis.

Fig. 14.22, Three-dimensional computed tomography scan shows significant sagittal imbalance centered at the anterior fontanelle as shown by the red and green arrows.

Fig. 14.23, Top view of a patient with coronal and metopic synostosis. An imbalanced trigonocephalic forehead is seen prior to surgery.

Fig. 14.24, Anteroposterior skull radiograph shows right harlequin sign and right-sided cranial deviation.

Fig. 14.25, Three-dimensional computed tomography scan showing marked changes of the skull base in a patient with metopic and left coronal synostosis. There is significant deformation of the left orbit volume and shape.

Fig. 14.26, Three-dimensional computed tomography scan shows orbital and forehead changes.

Fig. 14.27, Three-dimensional computed tomography scan demonstrates compensatory widening and separation of the contralateral coronal and sagittal sutures.

Fig. 14.28, Closure of these sutures leads to major changes in the skull base as noted on this computed tomography scan. The clival-foramen magnum axis is significantly misaligned from the clival-nasion axis, which should be all in a single straight line (red and blue arrows).

Fig. 14.29, The skull base changes effects on the globes and orbital contents are clearly seen in this computed tomography scan axial slice.

Incisions

A single midline incision is placed transversely over the anterior fontanelle and a second incision is made half way between the anterior fontanelle and the pterion on the side of the closed coronal suture ( Fig. 14.30 ). This combination of incisions allows full exposure of the anterior fontanelle, the metopic suture to the nasion, and the affected coronal suture to the pterion and squamosal suture.

Fig. 14.30, Artist’s representation of the location of the two incisions used to release both stenosed sutures.

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