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PubMed This is a summary of 20 peer-reviewed journal articles Updated
Craniofacial Surgery

The Surgical Journey: A Roadmap for Care

At a Glance

The surgical roadmap for Apert syndrome involves cranial surgery in infancy to protect brain development, staged hand and foot reconstruction in toddlerhood to improve function, and midface advancement in childhood or adolescence to address breathing and facial alignment.

Managing Apert syndrome involves a carefully coordinated series of surgeries. While every child’s path is unique, specialists follow a standardized roadmap designed to protect brain development, ensure a clear airway, and maximize the function of the hands and feet.

Infancy: Protecting the Brain (0–12 Months)

The most critical surgeries occur in the first year of life. Because the skull bones are fused (craniosynostosis), there is not enough room for the brain to grow [1]. Early cranial surgery is essential to relieve intracranial pressure (ICP) and provide the brain the space it needs to develop, which is key to preventing intellectual disability [2][3].

Your team may recommend one of two primary approaches:

  • Posterior Vault Distraction Osteogenesis (PVDO): This procedure expands the back of the skull using small devices called distractors that gradually move the bone [4]. PVDO often provides a larger increase in skull volume compared to traditional methods [5].
  • Fronto-Orbital Advancement (FOA): This surgery reshapes the forehead and the upper part of the eye sockets to protect the eyes and expand the front of the skull [6].

Toddlerhood: Hand and Foot Reconstruction (1–3 Years)

Separating the fused fingers (syndactyly) is typically done in stages to create a functional, five-digit hand that allows the child to grasp objects [7][8].

  1. Staged Approach: Because there is not enough skin to cover all fingers at once, surgeries are spaced out [9]. Surgeons often begin by separating the thumb and the pinky to immediately improve pinch and grip [10][11].
  2. Foot Surgery: Fused toes are also separated, but this usually requires fewer stages. The primary goal for the feet is not fine dexterity, but rather creating a stable foot that comfortably fits into standard shoes for walking [12].

Childhood to Adolescence: Breathing and Alignment

As the child grows, the focus shifts to the middle of the face and the airway.

  • Midface Advancement (Le Fort III): Due to midface hypoplasia (underdevelopment of the upper jaw and cheekbones), children may develop severe sleep apnea or dental misalignment [1][13].
  • Distraction Osteogenesis: Surgeons may use a “distractor” device to slowly pull the midface forward over several weeks [14]. This significantly improves the airway and changes the facial profile [15].
  • Timing: While sometimes done in early childhood to treat life-threatening breathing issues, midface advancement is often performed in later childhood or early adolescence [16].

Summary of the Surgical Roadmap

Age Range Primary Focus Common Procedures
0–12 Months Brain & Eye Protection PVDO or FOA; Shunt (if needed) [6][17]
1–4 Years Hand & Foot Function Staged syndactyly release [18][11]
4–12 Years Airway & Midface Midface advancement (Le Fort III) [16][13]
12+ Years Jaw Alignment Orthognathic (jaw) surgery; Rhinoplasty [19]

Note: Some children may also require earlier interventions for the airway, such as a tonsillectomy or a tracheostomy, if breathing is severely obstructed [20].

Common questions in this guide

Why do babies with Apert syndrome need head surgery in their first year?
Early cranial surgery is essential because the skull bones fuse prematurely, leaving insufficient room for the brain to grow. Surgery relieves intracranial pressure and allows the brain the space it needs to develop safely.
How is hand surgery managed for children with Apert syndrome?
Hand surgery is performed in multiple stages to separate fused fingers, typically starting with the thumb and pinky to improve grasp. The procedures must be spaced out because there is not enough skin to cover all the separated fingers at once.
What is midface advancement surgery and when does it happen?
Midface advancement, often a Le Fort III surgery, brings the underdeveloped upper jaw and cheekbones forward to improve breathing and facial alignment. It is typically performed in later childhood or early adolescence, though it may be done sooner for severe sleep apnea.
What is PVDO surgery?
Posterior Vault Distraction Osteogenesis (PVDO) is a procedure that expands the back of the skull using small devices that gradually move the bone. This increases skull volume to safely accommodate the growing brain.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Does our child’s current head shape or intracranial pressure suggest a preference for PVDO over FOA?
  2. 2.How many stages of hand surgery do you anticipate for our child’s specific hand type?
  3. 3.Which digits will be separated first, and what is the typical recovery time between stages?
  4. 4.At what age do you usually perform midface advancement at this center, and which technique (Le Fort III or distraction) do you prefer?
  5. 5.How will you monitor our child’s cognitive development and intracranial pressure as they grow?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (20)
  1. 1

    Apert syndrome: craniofacial challenges and clinical implications.

    Singh N, Verma P, Bains R, Mutalikdesai J

    BMJ case reports 2024; (17(7)) doi:10.1136/bcr-2024-260724.

    PMID: 39013624
  2. 2

    Two patients with Apert syndrome with different mutations: the importance of early diagnosis.

    Işık E, Atik T, Onay H, Özkınay F

    Turk pediatri arsivi 2017; (52(4)):231-235 doi:10.5152/TurkPediatriArs.2016.3305.

    PMID: 29483804
  3. 3

    Apert syndrome: Surgical outcomes and perspectives.

    Breik O, Mahindu A, Moore MH, et al.

    Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery 2016; (44(9)):1238-45.

    PMID: 27378001
  4. 4

    Apert syndrome: neurosurgical outcomes and complications following posterior vault distraction osteogenesis.

    Raposo-Amaral CE, Vincenzi-Lemes M, Medeiros ML, et al.

    Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2024; (40(8)):2557-2563 doi:10.1007/s00381-024-06436-2.

    PMID: 38700706
  5. 5

    Comparing the Increased Intracranial Volume From Different Surgical Methods for Syndromic Craniosynostosis.

    Fang C, Ji M, Dong C, et al.

    The Journal of craniofacial surgery 2022; (33(8)):2529-2533 doi:10.1097/SCS.0000000000008791.

    PMID: 36173686
  6. 6

    Optimising anaesthetic management during fronto-orbital advancement in an infant with Apert syndrome.

    Florendo GL, Jose GRB

    BMJ case reports 2025; (18(9)) doi:10.1136/bcr-2025-266714.

    PMID: 40953851
  7. 7

    Apert Syndrome Type III Hand: Prevalence and Outcomes.

    Raposo-Amaral CE, Medeiros LL, Raposo-Amaral CA

    The Journal of craniofacial surgery 2023; (34(4)):1170-1173 doi:10.1097/SCS.0000000000009107.

    PMID: 36730868
  8. 8

    Treatment of Apert Hand Syndrome: Strategies for Achieving a Five-Digit Hand.

    Raposo-Amaral CE, Denadai R, Furlan P, Raposo-Amaral CA

    Plastic and reconstructive surgery 2018; (142(4)):972-982 doi:10.1097/PRS.0000000000004815.

    PMID: 29994846
  9. 9

    A Case of Complex Syndactyly with Apert Syndrome Treated with a Two-stage Interdigital Reconstruction Using Adipose Flaps.

    Kitada A, Yotsuyanagi T, Harada J, et al.

    Journal of plastic and reconstructive surgery 2026; (5(1)):27-33 doi:10.53045/jprs.2024-0023.

    PMID: 41631027
  10. 10

    Outcome of Bilateral Hand Reconstruction in a Child Presenting Late With Apert Syndrome: A Case Report and Literature Review.

    Lim B, Shalan M

    Cureus 2023; (15(8)):e43641 doi:10.7759/cureus.43641.

    PMID: 37719615
  11. 11

    Central Coalition Osteotomy of Phalangeal Synostoses in the Management of the Type III Apert Hand.

    Theman TA, Upton J, Taghinia AH, et al.

    The Journal of hand surgery 2018; (43(11)):1042.e1-1042.e8 doi:10.1016/j.jhsa.2018.03.050.

    PMID: 29891270
  12. 12

    Unilateral Coronal Craniosynostosis in an Apert-Like Patient.

    Pourtaheri N, Wang DZ, Lesko RP, et al.

    Plastic surgery (Oakville, Ont.) 2019; (27(1)):78-82 doi:10.1177/2292550318800322.

    PMID: 30854365
  13. 13

    Long-term Management of a Patient with Apert Syndrome.

    Horiuchi S, Sato H, Iwasa A, et al.

    The journal of contemporary dental practice 2021; (22(10)):1184-1190.

    PMID: 35197388
  14. 14

    Improvement of Periorbital Appearance in Apert Syndrome After Subcranial Le Fort III With Bipartition and Distraction.

    Chetty V, Haber SE, Khonsari RH, Arnaud E

    The Journal of craniofacial surgery 2020; (31(3)):711-715 doi:10.1097/SCS.0000000000006233.

    PMID: 32011541
  15. 15

    Long-term morphometric and functional outcomes of frontofacial advancement in syndromic craniosynostosis.

    Romeo DJ, Akarapimand P, Sussman JH, et al.

    Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2026; (42(1)):45 doi:10.1007/s00381-025-07069-9.

    PMID: 41580524
  16. 16

    Respiratory and craniofacial management in children with Apert syndrome.

    Khirani S, Paternoster G, Luscan R, et al.

    Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery 2025; (53(8)):1080-1087 doi:10.1016/j.jcms.2025.03.019.

    PMID: 40246667
  17. 17

    Apert Syndrome Outcomes: Comparison of Posterior Vault Distraction Osteogenesis Versus Fronto Orbital Advancement.

    Raposo-Amaral CE, Oliveira YM, Raposo-Amaral CA, Ghizoni E

    The Journal of craniofacial surgery 2022; (33(1)):66-69 doi:10.1097/SCS.0000000000007959.

    PMID: 34261966
  18. 18

    Management of Paronychia in Patients With Apert Syndrome.

    Kim JS, Block LM, Zhu X, Davit AJ

    Techniques in hand & upper extremity surgery 2020; (25(1)):30-34 doi:10.1097/BTH.0000000000000295.

    PMID: 32398550
  19. 19

    Apert syndrome: Cranial procedures and brain malformations in a series of patients.

    Munarriz PM, Pascual B, Castaño-Leon AM, et al.

    Surgical neurology international 2020; (11()):361 doi:10.25259/SNI_413_2020.

    PMID: 33194294
  20. 20

    Airway Analysis in Apert Syndrome.

    Forte AJ, Lu X, Hashim PW, et al.

    Plastic and reconstructive surgery 2019; (144(3)):704-709 doi:10.1097/PRS.0000000000005937.

    PMID: 31461034

This timeline provides an overview of common surgeries for Apert syndrome and is for informational purposes only. Always consult your child's craniofacial team to discuss a personalized surgical plan.

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