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Year : 2022  |  Volume : 17  |  Issue : 5  |  Page : 29-43

Multisuture and syndromic craniosynostoses: Simplifying the complex

1 Division of Paediatric Neurosurgery and Craniofacial Surgery, Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India
2 Division of Craniomaxillofacial Surgery, Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India

Date of Submission06-Feb-2022
Date of Acceptance12-Mar-2022
Date of Web Publication19-Sep-2022

Correspondence Address:
Prof. Suhas Udayakumaran
Division of Paediatric Neurosurgery and Craniofacial Surgery, Department of Neurosurgery, Amrita Institute of Medical Sciences and Research Centre, Kochi, Kerala 682041
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_26_22

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Most complex craniosynostoses are managed the same way as syndromic craniosynostoses (SCs), as these patients often experience similar problems regarding cognition and increased intracranial pressure (ICP). The evaluation and treatment plan for craniosynostoses is complex, and this, additionally, is complicated by the age at presentation. In this article, the authors review the complexity of SCs in the presentation and management. An algorithm is necessary for such multifaceted and multidimensional pathology as craniosynostoses. In most algorithms, posterior calvarial distraction is a consistent early option for complex craniosynostoses presenting early with raised ICP. Addressing the airway early is critical when significant airway issues are there. All other surgical interventions are tailored on the basis of presentation and age.

Keywords: Algorithm, complex, craniosynostoses, management, multisutural, syndromic

How to cite this article:
Udayakumaran S, Krishnadas A, Subash P. Multisuture and syndromic craniosynostoses: Simplifying the complex. J Pediatr Neurosci 2022;17, Suppl S1:29-43

How to cite this URL:
Udayakumaran S, Krishnadas A, Subash P. Multisuture and syndromic craniosynostoses: Simplifying the complex. J Pediatr Neurosci [serial online] 2022 [cited 2022 Oct 2];17, Suppl S1:29-43. Available from: https://www.pediatricneurosciences.com/text.asp?2022/17/5/29/356362

   Introduction Top

Syndromic craniosynostosis (SC) usually involves multiple sutures combined with malformations of other organs. Syndromes most frequently associated with craniosynostosis are Apert, Crouzon, Pfeiffer, and Saethre–Chotzen syndromes. Of these, Apert syndrome is the most common, with a prevalence of 1:100,000.[1]

Complex varieties of craniosynostoses (clinical suspicion of SC without proven genetic abnormality, often with multiple sutural synostoses) have raised intracranial pressure (ICP), airway, and aesthetic issues. In SCs, the skull deformity is associated with additional clinical problems, including hand and feet malformations, skeletal and cardiac defects, developmental delay, and others. The distinction between multisuture craniosynostosis (MC) and SC is made based on phenotype. MC can occur in all variations of two or more affected cranial sutures. In this group, new genetic causes for craniosynostosis are still identified, such as the genes TCF12, ERF, and IL11RA.[2] Additionally, this may involve metabolic causes.[3] Systemic diseases possibly associated with SC include skeletal dysplasias such as acrodysostosis [OMIM: # 101800], hypophosphatasia [OMIM: # 146300], hypophosphatemic rickets [OMIM: # 307800]; inherited metabolic diseases with skeletal involvement such as mucopolysaccharidoses; and ciliopathies such as Sensenbrenner syndrome [OMIM: # 218330].

In SC, additional congenital disabilities and dysmorphisms are present. The four most common forms of SC are Apert, Crouzon (including Pfeiffer syndrome), Saethre–Chotzen, and Muenke syndrome.

Most complex craniosynostoses are managed the same way as SCs, as these patients often experience similar problems regarding cognition and increased ICP.[4] The treatment plan for craniosynostoses is complex, and this, additionally, is complicated by the presentation and age at presentation.

In this article, the authors review syndromic and multisutural craniosynostoses and the challenges involved in managing them.

   Materials and Methods Top

Articles were searched with literature until January 1, 2022, using search engines PubMed, Embase, and Google Scholar, using MeSH terms “syndromic craniosynostoses,” “multisutural craniosynostoses,” and “complex craniosynostoses.”

   Discussion Top


In 1993, Jabs et al.[5] were the first to describe the genetic origin of SC. They identified a mutation located in the MSX2 gene in a patient with Boston-type craniosynostosis. A genetic cause has been found in a much higher proportion of SCs [Table 1].[6]
Table 1: Nosology and etiology[8]

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Progress in identifying new genes involved in the pathogenesis of inherited disorders is possible with the application of high-throughput molecular methods such as Genome Wide Association Studies (GWAS), Next-Generation Sequencing (NGS), or Array Comparative Genomic Hybridization (aCGH) techniques.

Genetic counseling

Clinical differentiation between isolated synostosis and SCs is essential for proper genetic counseling to estimate the recurrence risk in families. Various experts should carefully examine each case to put forward the most reliable clinical diagnosis and perform appropriate molecular testing. The majority of SCs are inherited autosomal dominant, although, in many cases, patients present with de novo mutation that is not inherited from the parents. In many instances, somatic mosaicism for the causative mutation was described; therefore, patients’ parents should constantly be tested for mutation’s presence. The recurrence risk in such families is higher than in the general population. In the case of craniosynostoses inherited in an autosomal recessive manner, with confirmed carrier status in both parents, the recurrence risk is high, at 25% (1/4). According to Johnson and Wilkie,[7] when the family history is negative for craniosynostoses and no genetic or[7] cytogenetic alternations were found, the recurrence risk to offspring is ~ 5% in the case of nonsyndromic sagittal, metopic, and unicoronal synostosis and 30–50% in bicoronal or multisuture synostosis. In craniosynostoses caused by a single gene defect, when the mutation was not identified in both parents, the recurrence risk is shallow (< 1%), such as in the case of fibroblast growth factor receptor (FGFR)-related disorders. If no instances of mosaicism have been described, like in the case of rare TWIST1 mutations, the recurrence risk should be estimated at 2%. In the case of craniofrontonasal syndrome caused by EFNB1mutations, the somatic mosaicism is quite frequent (18.5% of parents) and, therefore, the recurrence risk should be calculated at 10%.[7]

Antenatal diagnosis

SCs can be diagnosed in the second trimester. A fetal magnetic resonance imaging (MRI) is indicated. However, a precise prenatal prognostication regarding the outcome is impossible.[9] There is no absolute indication for a cesarean section with a cephalic position, and the head circumference is not raised excessively. The patients should be informed about the higher risk of arrested labor and emergency cesarean section in significant skull deformities. The delivery should be planned of a perinatal center to ensure the newborn’s excellent postnatal care, especially regarding airway management.[10] Prenatally, a pediatric neurosurgeon should be consulted and inform the parents about possible operative procedures.[9],[11],[12]

   Screening Tools Top

The following tools are a part of the screening and the presentation for decision making.

  • (1) Clinical evaluation, including symptomatology, signs, and head circumference;

  • (2) Eye and visual assessment;

  • (3) A sleep study or polysomnography (PSG);

  • (4) Imaging: Skull radiographs, multidimensional computer tomography (MDCT), and MRI;

  • (5) Nasal endoscopy if indicated.

Clinical evaluation including head circumference

Clinical evaluation is done to understand the general well-being and to identify symptoms and signs of raised ICP and airway compromise. Documentation of the trend of head circumference, especially if some form of decompression has been done, must be done. The abnormal trend may be the only early indicator of progressive hydrocephalus, especially when ventriculomegaly is already present. At the same time, a lack of head circumference change may sometimes be deceptive.

Ophthalmological evaluation

All children with SCs undergo ophthalmologic examination. The examination essentially is to

  • (1) Evaluate for signs of acute and chronic raised ICP;

  • (2) The severity of visual proptosis, its sequelae, and initiate protective measures;

  • (3) Other issues strabismus, hypertelorism.

The presence of papilledema indicates raised ICP and hence requires early timing of vault surgery. Severe proptosis leading to exposure keratitis orbital subluxation may indicate early midface surgery.[13],[14],[15]

Sleep study or polysomnography

A sleep study is essential to understand the significance of any clinical apnea, especially if the child has midface hypoplasia. The sleep study is an efficient tool to understand and gage airway physiology and the effect of treatment.[16] It may contribute to the decision-making. Still, the question remains how much it contributes to the final decision-making when glaring symptoms of midface hypoplasia are present.[16] Usually, the syndromic children undergo posterior calvarial vault distraction (PCVD)/posterior vault distraction osteogenesis especially if hydrocephalus or Chiari malformation is present or unless older than 2 years with none of the previously mentioned indications. If obstructive airway symptoms or signs are present, they undergo early midface advancement in our protocol [Figure 1] and [Figure 2].
Figure 1: Algorithm for less than 2-year-old (age at presentation). PVDO (PCVD) is usually the first option. This is usually followed by FOAR. Other options are as suggested in the algorithm

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Figure 2: Algorithm for more than 2-year-old (age at presentation). PVDO (PCVD) is not usually the first option and is done only if the Chiari and hydrocephalus are a prominent presentation or if extreme turricephaly needs to be addressed. FOAR may be the initial option in this age category. Other options are as suggested in the algorithm. The algorithm is tailored depending on the patient presentation

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Imaging in complex craniosynostoses

Although the preliminary diagnosis of craniosynostosis is primarily the result of physical examination and craniometrics, the radiological assessment currently plays a vital role in understanding and confirming the diagnosis, the surgical planning, and even the post-operative follow-up, especially in SCs.[17] On the contrary, in infants, the use of radiation or the need for sedation/anesthesia raises the problem to reduce them to a minimum to preserve such a delicate category of the patient from their adverse effects. Also, the radiological examinations may be helpful to assess the timing/pattern of premature closure of the sutures since all or most of them progressively close but with different patterns according to the different syndromes.[18]

Multidimensional CT

  • (1) Diagnosis and evaluation: The CT scan demonstrates the sutures involved. Moreover, intracranial 3D images are a valuable tool in evaluating skull base hypoplasia and minor suture synostoses.[19] Because of the artifact related to the threshold, the classic 2D images should always be considered for an appropriate evaluation of the suture patency [Table 2].[17]

  • (2) Venogram: The venogram demonstrates the venous architecture, including the calvarial veins, which may be important for surgical strategy.

  • (3) Post-operatively and follow-up: CT may be used post-operatively for possible complications. It may demonstrate the persistent bone gaps, which may require repair in some instances.
Table 2: Various named clinical syndromes and salient clinical features[8]

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MRI brain

Imaging adds or modifies decision-making in craniosynostoses.[17] The indication for MRI in craniosynostoses in our protocol is as follows:

  • (a) Complete the evaluation, especially if atypical features are present in a clinical diagnosis of single suture synostoses.[17]

  • (b) The presence of hydrocephalus and Chiari will influence management.[20],[21] We additionally look for the subarachnoid space to understand the areas of calvarial volume compromise, although this is presently a subjective assessment.

  • (c) Understanding the venous anatomy affects the surgical strategy.[17],[22],[23]

Using Black bone MRI,[24] to study the bone and sutures is a newer advancement gaining momentum and promising.

   Clinical Issues in Complex Craniosynostoses: Goals of Management and Algorithm Top

Initial evaluation during the presentation is focussed on addressing acute issues, viz., severe raised ICP, airway (nasopharyngeal constriction from a retruded maxilla, oropharyngeal constriction from retro/micrognathia), eyes (exorbitism with corneal exposure), or cerebrum (severe cranial constriction).

   Timing of Surgical Intervention: When and What? Top

Raised ICP and management

The risk of elevated ICP increases exponentially with the number of affected sutures. In SCs, the risk of ICP can be further elevated due to intracranial venous congestion, hydrocephalus, and upper airway obstruction.[25],[26],[27]

Several reports have identified Crouzon syndrome as carrying a higher risk of elevated ICP than other forms of SC. One study demonstrates a 65% elevated ICP and the remainder borderline elevated.[25],[26],[27]

In the formulation of the management of multisuture and SC, there is no spontaneous improvement in cranial deformity. There is a significant syndrome-specific risk of increased ICP, leading to neurocognition and vision impairment in the patient. If protocol-based surgery is not performed in the first year of life, frequent monitoring for signs of elevated ICP is necessary.[15] Most workers advocate managing raised ICP when signs of increased pressure appear. Although most syndromic and mutisutural craniosynostoses can have elevated ICP, signs may be present in a limited percentage. In a significant percentage, it may remain unnoticed because none of the screening methods is 100% sensitive. Additionally, it is unclear whether cranial expansion before the development of raised ICP is beneficial.

Timing of cranial expansion

Patients with SC in whom the cranial expansion is performed within the first year of life may have a better cognitive outcome: 17% of Apert children and 81% of Crouzon children have a normal IQ at the surgery before the age of 1 vs. 0% and 56%, respectively, at surgery after the age of 1. For bicoronal synostosis, the difference was an IQ of 99 at the surgery before the age of 1 year vs. of 89 at the surgery after the age of 1 year.[28],[29]

Timing of treatment of patients with Muenke syndrome from the age of 6 to 9 months may have a favorable effect on the aesthetic outcome compared with earlier intervention and is justified given the low prevalence of elevated ICP.

Timing of treatment of patients with Apert, Crouzon, or Saethre–Chotzen syndrome between the ages of 6 and 9 months may have a favorable effect on the aesthetic outcome compared with an earlier or later operation and seems appropriate given the high prevalence of elevated ICP.[30],[31],[32]

Surgery for cranial expansion

Primary tenets of timing and technique

  1. Apert and Crouzon require expansion in the form of PCVD and fronto-orbital advancement and remodeling (FOAR);

  2. Muenke and Saethre–Chotzen in the form of FOAR;

  3. Technique in other multisutural craniosynostoses depending on the deformity;

  4. Early surgery 6–12 months is preferable;

  5. Infrequently calvariectomy might be required for raised ICP at birth or in the neonatal period.

Early PCVD and why PCVD

Cerebellar tonsillar descent, or Chiari malformation type I, is often associated with craniosynostosis, especially in syndromic cases, and multisutural and lambdoid synostosis.[33],[34],[35] PCVD is the standard initial step in most described algorithms when raised ICP is the presentation.[36],[37] Most units use PCVD to postpone FOAR to a later date.[38] ICP being the earliest issue in SCs,[23],[39-44] PCVD is a constant option. Iida et al.[45] instead proposed that cranial expansion should be performed at an early age to prevent turribrachycephaly and neurocognitive complications. Still, some authors opine that it is better to wait to perform posterior distraction to prevent gull-wing deformity.

Even for the asymptomatic patients, the primary aim was disease stabilization and prevention of symptoms, and the secondary purpose was to reduce the Chiari malformation. The decision for surgery on asymptomatic patients was based on the current knowledge of the condition’s natural history.[37] Our protocol only differed in older children (arbitrarily over 2 years) unless Chiari malformation is a presenting feature.

The benefits of a primary frontal expansion for a young child are short-lasting,[46] and it would have only a minimal effect on the remainder of the facial deformity, and the subsequent intervention for a facial correction (Le Fort III, monobloc, or facial bipartition) would therefore be unnecessarily compromised.[47],[48]

Fronto-orbital advancement and remodeling

FOAR is usually selected when aesthetics and exorbitism are the presentation.[36] It is performed at 6–9 months of age.[46],[49] Calvarial volumes in the SC patients were smaller shortly after birth but tended to reach average values at 6–8 months and had normalized entirely at 13 months of age.[50] This would imply that surgical intervention should be delayed until 6–8 months, thus maximizing the effects of accelerated normal orbital growth.[51]

FOAR is also our choice of surgery in older children with raised ICP. This is considering the logic that the requirement of cranial expansion may not be much at this age, and later timing of the frontal procedure would often remove the need for a frontal re-operation. The exception is severe exorbitism posing a threat to vision, for which FOAR should indeed be the early or even the first procedure.

Interval Between PCVD and FOAR

We tend to give at least 6 months between PCVD and FOAR. This interval is to exhaust the utility of the space created by PCVD and stagger the response to pathology. Nowhere in the literature is the interval between the two procedures mentioned. The authors have mentioned that the use of PCVD may negate the need or delay FOAR.[37],[38]

If the airway needs to be addressed due to a significant obstructive element, the midface is addressed prior to FOAR. Until this stage of evolution of our algorithm [Figure 1] and [Figure 2], we have not seen a reason to negate FOAR as an aesthetic issue is common in all these children. An early addressal, like in any scenario in which FOAR is used, improves aesthetic results and allows normal psychosocial development of these kids.[15]

Spruijt et al.[52] reported a high 21% baseline incidence of elevated ICP in children with SC. Although the prevalence decreased to 8% within 1 year of vault surgery, it returned to 21% thereafter.[52]

The type and timing of surgical treatment for syndromic synostosis are critical for alleviating elevated ICP not just immediately but also longitudinally.[38]

Calvarial distraction for SCs

PCVD, as described earlier, has become a pivotal adjunct to distraction techniques used in SCs.[53] Other distraction techniques such as monobloc and frontal distraction have also been used for the management of SCs.[54],[55]

Endoscopy in syndromic craniosynostoses

Endoscopy has established itself to a reasonable extent in managing single suture craniosynostoses. For multisuture and SCs, there is no clear consensus among care teams regarding the indications, advantages, and disadvantages of endoscopic treatment vs. open surgery for craniosynostosis.

Some workers have supported the use of endoscopic strip craniectomy with helmets as a reasonable, safe, and efficacious option. It provides low rates of transfusion, complications, and ICU admission, as well as a short length of anesthesia, surgery, and hospital stay, along with normalized head growth.[56] For gaining these distinct advantages for the young infant with craniosynostosis, an early and expeditious referral of infants with this condition by their primary care physician is crucial.[56]

The role of calvariectomy and morcellation

With the introduction of PCVD, the utilization of early calvariectomy has been infrequent. It still may be employed very early after birth in a neonate or when the highly lacunar skull may not permit PCVD in which instances can be lifesaving. The infant may be considered for routine protocol later during infancy when physiologically more suitable.

   Midface, Airway, and Management of Airway Top

Syndromic cases, such as the FGFR2-related syndromes (Apert, Crouzon, and Pfeiffer), account for 15–30% of all craniosynostoses and involve several extracranial manifestations. These syndromes, apart from the calvarial abnormalities, are notable for their prominent facial deformities causing functional, psychosocial, and aesthetic impairment. Midfacial dysmorphism is the most ubiquitous, involving a retrusive and deformed maxilla that can cause airway compromise. Obstructive sleep apnea (OSA) is present in up to 68% of SCs.[57],[58] The pathogenesis of OSA in this patient population varies between syndromes but is mainly due to midface hypoplasia resulting in a reduction of the nasopharyngeal and oropharyngeal airway space.[58],[59] Other factors such as mandibular hypoplasia, adenotonsillar hypertrophy, and pharyngeal collapse also play a role in the occurrence of OSA. It is important to diagnose and to treat OSA as early as possible, as OSA can induce intracranial hypertension and have neurocognitive, cardiovascular, and metabolic consequences. The clinical suspicion of OSA is most often raised when symptoms such as snoring, hyperactivity, inattention, breathing stops, and failure to thrive are present. The gold standard for diagnosing OSA is overnight PSG in a hospital setting, although ambulatory PSGs may also be an alternative diagnostic tool.

Severe airway issues early in life cannot be neglected as they have functional repercussions on physical and mental development. Aggressive surgical intervention to enlarge the nasopharyngeal space can reduce OSA severity and, therefore, avoid the need for tracheostomy. Surgical approaches include adenotonsillectomy, uvulopalatopharyngoplasty, and midface advancement.[60]

Surgery of the midfacial hypoplasia is usually corrected later in childhood (after 8 years of age) with Le Fort III distraction osteogenesis. Le Fort III osteotomy was first described by Gillies and Harrison[61] in 1950 to treat children affected by craniofacial malformation; later, Tessier[62] refined the midface osteotomy. Ortiz-Monasterio et al.[63] developed the monobloc advancement; the fronto-orbital segment was advanced simultaneously with the midface.

Early monobloc frontofacial advancement was proposed by Marchac and Renier.[64] This corrects the exorbitism satisfactorily, but the airways improvement might be limited, although our experience has been different. We have found considerable functional improvement in these children when the airway is addressed very early, with timing being governed by the severity at presentation and other clinical issues.

Timing of airway management

Surgery of the midfacial hypoplasia is usually corrected later in childhood (after 8 years of age). Apart from the question of the extent of functional improvement, the advancement is only horizontal and will not correct the face’s associated enlargement, unless combined with facial bipartition, as proposed by Ortiz-Monasterio et al.[65] When this operation is performed in infancy, there is frequently some deterioration at the maxillary level over time, leading to re-operations.[28] As there is no maxillary growth after an early monobloc, a second monobloc (or Le Fort III) operation after 18 may be necessary. Considering the functional advantage of early addressal, we have preferred this strategy, allowing us to avoid tracheostomy altogether. Presently, the long-term outcome of this strategy is not clear; hence, this is the severe limitation of our strategy.[66]

Protecting the eye

Early tarsorrhaphy may be merited in extreme exorbitism. FOAR and early midface advancement are usually definitive. Occasionally, in the presence of midface hypoplasia, episodes of globe subluxation can create an ocular crisis, including the risk of loss of vision. We consider this an early indication of midface distraction.

   Managing Hydrocephalus Top

The prevalence of hydrocephalus is possible at 6–26% in Crouzon and Pfeiffer, 0–6% in Apert, 5–12% in MC, and negligible in Saethre–Chotzen and Muenke syndrome.[67],[68],[69] In these children, ventriculomegaly is monitored using MRI scanning and 6-monthly fundoscopy. Progressively increasing ventricle width or papilledema is managed according to a treatment plan. Progressive change in head circumference trend can be another indicator of unresolved hydrocephalus in spite of expansion surgeries.

Shunting is counterproductive to cranial vault expansion. In the presence of hydrocephalus, all efforts should be directed at treating increased ICP utilizing cranial vault expansion.

The order and timing of treatment modalities have never been systematically investigated. Renier et al.[28] recommend performing cranial vault expansion first and then proceeding to shunt placement, provided that this is clinically feasible. After expansion procedures (PCVD, FOAR, craniectomy), a shunt has to be inserted if the progression of head circumference occurs or if any other signs of raised ICP persist for a prolonged time.[51] Ventriculoperitoneal shunt (VPS) if used should preferably be a programmable device with an antisiphon mechanism to reduce the secondary craniosynostoses.

Cerebrospinal fluid (CSF) diversion in the form of endoscopic third ventriculostomy (ETV)[70] or CSF shunt may precede expansion procedures, specifically early presentation (at birth) of hydrocephalus with poor general condition and occasionally in poor bone condition with extreme lacunae.

Timing of Other Issues

To optimize the management of complex craniosynostoses and attain the utmost quality in these children, many other issues need to be handled with contribution from other subspecialties. The following are the other issues that need to be addressed.

Management of hand

Management of the hand is scheduled after the cranium and if the airway needs to be addressed early.[71] This is usually prioritized, enabling children to pinch and grasp objects at a younger age and also gain manual dexterity.[72] This can be done beyond 9 months, preferably earlier by 2 years.

Visual deficit, exposure keratitis, and hypertelorism

Hypertelorism and exorbitism can cause visual deficits, keratitis, or globe herniation.

Orbital hypertelorism may be treated with orbital box osteotomy. This procedure cannot be performed until the teeth have erupted permanently, usually from approximately 14 years. Orbital box osteotomy is well suited to be combined with an orthognathic procedure. We prefer facial bipartition especially if the occlusal surface is misaligned.

Orthognathic issues

The abnormal patterns of facial growth in children with craniosynostosis syndromes often result in significant dentofacial deformities. Class III malocclusion, secondary to mid-face hypoplasia, is the most commonly seen deformity and often develops despite appropriate midface surgical treatment. Management of these jaw abnormalities involves an orthodontist, a dentist, and a craniofacial surgeon. With the completion of the growth of both the maxilla and the mandible, surgery may be indicated. Orthodontic therapy to optimize the bite is the initial step. Surgery is customized to the individual jaw discrepancy, but most commonly involves osteotomy at the Le Fort I level with a sliding genioplasty. These surgical procedures are usually performed between the ages of 14 and 18 years when the facial skeleton is mature.

However, there is a paucity of guidelines, especially regarding the timing and the management of residual hypoplasia of the maxilla.[73],[74],[75]

Integration of genetic evaluation into the algorithm: The next frontier

Genetics of craniosynostoses are still incomplete in understanding.[8] Many patient presentations are left without a molecular diagnosis, and other disease-causing mechanisms are suspected in these cases. Whether the knowledge of genetic makeup can improve our algorithm and its timeline is presently a matter of conjecture only.[8],[76]

Revision surgeries and final facial contouring

Many of these children require revision of the FOAR and some minor corrections, depending on the indication and presentation. These can be integrated into the algorithm pro re nata. At the completion of facial growth and all major osteotomies, contour irregularities of the facial skeleton may still remain. Final contouring procedures are often performed at this time. They include smoothing down irregularities, adding bone grafts or bone substitutes to different areas (e.g., calcium carbonate cement), and resuspending soft tissues such as the midface or canthus.[77]

AIMS algorithm for management [Figure 1][Figure 2][Figure 3][Figure 4][Figure 5][Figure 6][Figure 7][Figure 8][Figure 9]
Figure 3: Illustrative case 1. A and B: An 11-month-old infant presented with hydrocephalus and signs of raised ICP. He had PCVD (illustrated in [Figure 4]). Additionally, the child had severe airway issues with recurrent episodes of URTI. C: Subsequently, he had robotic-assisted frontofacial advancement (during the removal of posterior calvarial distractors). D: Robot-assisted insertion of transfacial pin for MD. E: MDCT showing the trajectory of the transfacial pin planned to get adequate purchase keeping avoidance to the injury globe and tooth buds in perspective

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Figure 4: Illustrative case 2. An 11-month-old infant undergoing PCVD. A: Pre-operative MR showing tight posterior fossa and tonsillar herniation. B: Surgical planning with the patient-prone position. The incision is planned, keeping the distractor position in perspective. C: Intraoperative photograph showing the craniotomy with distractor in situ. D: Post-operative MRI improved CSF spaces in the posterior fossa and the posterior calvarium in general. E: Pre-operative MDCT showing flattened occiput, multisutural involvement, and the digital impression suggesting raised ICP. F: Post-operative MDCT showing the craniotomy with distractor in situ

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Figure 5: Illustrative case 3. A child with Crouzons. He presented as an infant with hydrocephalus. At 3 months, he had an endoscopic third ventriculostomy. Subsequently, he had PCVD. Upper row: Post PCVD. He continued to have severe airway issues with repeated episodes of upper respiratory infections. He underwent frontofacial distraction using internal distractors. Middle row: Post frontofacial distraction. Lower row: Follow-up at 6 years. At follow-up, his aesthetic outcome seemed suboptimal, which is one of the disadvantages of early midface correction

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Figure 6: Illustrative case 4. A: A 17-year-old, young boy with Apert syndrome presenting in a delayed manner. He was scholastically poor but his concern was aesthetics only. B: He underwent frontofacial distraction using an external distractor (RED) device. C: Post distraction, the result seemed satisfactory

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Figure 7: Illustrative case 5. This was a 5-year-old child who presented to us with hypertelorism. He underwent frontal remodeling with box craniotomy (extreme left)

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Figure 8: Illustrative case 6. A 5-year-old child with Apert syndrome who had an FOAR at 14 months now presented with signs of raised ICP. He would require an MD later. Presently, he has no airway issues

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Figure 9: Illustrative case 7. A child with multisutural craniosynostoses. He presented at 2 years with signs of raised ICP. MDCT showed multiple sutural stenoses

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We follow the workflow based on the symptom combinations.

Below 2 years [Figure 1]:

  • The presence of calvarial aesthetic issues only;

  • Presence of raised ICP, airway, aesthetic issues [Figure 3][Figure 4][Figure 5];

  • Presence of predominantly airway issues and aesthetic issues;

  • ICP and aesthetic issues.

  • Above 2 years [Figure 2]:

    • • The presence of calvarial aesthetic issues only;

    • • Presence of raised ICP, airway, aesthetic issues + other issues;

    • • Presence of predominantly airway issues and aesthetic + other issues;

    • • ICP and aesthetic issues;

    • • Delayed presentation [Figure 6].{Figure 6}
      • (1) Indications for FOAR:
        • i. Older children with raised ICP without Chiari malformation hydrocephalus or lambdoid synostoses;

        • ii. Forehead advancement with/without exorbitism (all age groups) [Figure 3] and [Figure 4].

      • (2) Managing hydrocephalus:

        • i. We prefer managing hydrocephalus with PCVD initially;

        • ii. If the hydrocephalus persists, a VPS is done [Figure 5];

        • iii. Alternatively, if the infant has a poor general condition or failure to thrive, a primary CSF diversion in the form of ETV or VP shunt is preferred.

      • (3) Institutional protocol for assessing the significance of midface retrusion in craniofacial syndromes:

        • i. Recurrent upper respiratory tract infection (URTI)/pneumonia and hospital admissions with/without features of failure to thrive [Figure 3] and [Figure 4];

        • ii. Poor sleep quality with respiratory distress and frequent awakenings.

        • iii. PSG with obstructive findings (not a strict inclusion criterion when abnormal functional status has been convincing. A screening PSG is acceptable when a complete study is not feasible).

        • iv. Significant exorbitism with incomplete eyelid closure (exposure keratitis, corneal opacity, globe subluxation, or imminent globe subluxation).

    If signs and symptoms are apparent, we do not rely on PSG for further decision-making.

    • (4) Timing of other surgeries

    Hand surgery, preferably late infancy and beyond, as the other functional issues would allow.


    • (1) Although we have created an algorithm, a rigid application of the same is impossible and may be tailored to the presentation and logistics. Hence, the algorithm flow directions are not watertight in their constitution.

    • (2) The algorithm does not incorporate etiology, viz., genetics into the workflow. Various genetic expressions may have a variable outcome. Identifying the genetic defect seems very important for disease prognosis and treatment decisions (e.g., in the case of early surgical intervention) and also allows for identifying the persons’ genetic risks and giving proper genetic counseling to the patients’ families.[8]

       Conclusion Top

    An exact algorithm is necessary for such multifaceted and multidimensional pathology as SCs. PCVD is a consistent early option for complex craniosynostoses presenting early in most algorithms, and addressing airway early is a critical difference in our algorithm. All other surgical interventions are tailored based on presentation and age.

    Financial support and sponsorship


    Conflicts of interest

    The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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      [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]

      [Table 1], [Table 2]


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