1. What is the meaning of craniosynostosis?

2. Normal closure time of sutures?

3. Types of skull deformities due to craniosynostosis

4. Syndrome associated with craniosynostosis

5. MCQs associated with craniosynostosis

6. Figure -1 (Types of craniosynostosis)

1. What is the meaning of craniosynostosis?

Craniosynostosis is a condition where one or more of the skull sutures close too early in babies. These sutures normally stay open for some time to allow the brain to grow. The major sutures affected include the metopic, coronal, sagittal, and lambdoid, while the minor ones include frontonasal, temporosquamosal, and frontosphenoidal.

2.When Do Skull Sutures Normally Close?

• Metopic Suture/Frontal suture: Starts closing at 2 months

• Sagittal, Coronal, and Lambdoid Sutures: Close around 22-26 months

• Frontonasal and Frontozygomatic Sutures: Close last at 68-72 months

If a suture closes too soon, it restricts skull growth in that direction. The skull then grows in other areas to compensate, leading to different head shapes based on the affected suture.

3. Types of Skull Deformities Due to Craniosynostosis:

1. Scaphocephaly: Long, narrow skull (due to sagittal suture closure)

2. Plagiocephaly: Asymmetrical skull (due to coronal or lambdoid suture closure)

3. Trigonocephaly: Triangular forehead (due to metopic suture closure)

4. Cloverleaf Skull: Severe skull deformity due to multiple sutures closing early, often linked to hydrocephalus

5. Tower Skull (Acrocephaly): High, pointed skull from multiple sutures fusing, seen in Apert and Crouzon syndrome

6. Oxycephaly/Turricephaly: Tall skull due to delayed treatment of brachycephaly, often part of syndromes

4. Syndromes Associated with Craniosynostosis

1. Apert Syndrome

• Caused by Fibroblast growth factor (FGF) gene mutation (autosomal dominant or random occurrence)

• Key Features:

  • Bicoronal synostosis (both coronal sutures close early)

  • Flat forehead and midface

  • Wide-set eyes and slanted eye shape

  • Hand and foot abnormalities (mitten-like fusion of fingers and toes)

  • Cleft palate, dental issues, and breathing difficulties

2. Crouzon Syndrome

• Caused by FGFR2 or FGFR3 gene mutation (autosomal dominant)

• Key Features:

  • Bicoronal synostosis leads to a tall, flat forehead

  • Proptosis (bulging eyes) and beaked nose

  • Midface underdevelopment

  • No cleft palate or hand/foot fusion, unlike Apert syndrome

  • Some patients may have spine issues

Other conditions linked to craniosynostosis include Carpenter syndrome and Pfeiffer syndrome, which also involve early skull suture closure and facial abnormalities.

 For more information, consult a podiatric specialist or a genetic counselor if the child has signs of craniosynostosis. Early diagnosis and treatment can help manage skull growth and improve outcomes.

5. MCQs associated with the craniosynostosis

1. What is craniosynostosis?

a) A condition where brain growth is delayed

b) A condition where skull sutures close too early

c) A genetic disorder affecting bones

d) A neurological condition

Answer: b) A condition where skull sutures close too early

Rationale: Craniosynostosis occurs when one or more skull sutures close prematurely, affecting skull and brain development.

2. Which of the following sutures closes first in normal skull development?

a) Coronal suture

b) Lambdoid suture

c) Metopic suture

d) Sagittal suture

Answer: c) Metopic suture

Rationale: The metopic suture begins closing at around 2 months, whereas other sutures close much later.

3. At what age do sagittal, coronal, and lambdoid sutures typically close?

a) 2 months

b) 6 months

c) 22-26 months

d) 68-72 months

Answer: c) 22-26 months

Rationale: These sutures close around 22-26 months, allowing for skull and brain growth.

4. Which skull deformity results from premature sagittal suture closure?

a) Trigonocephaly

b) Plagiocephaly

c) Scaphocephaly

d) Brachycephaly

Answer: c) Scaphocephaly

Rationale: Early sagittal suture closure leads to a long, narrow skull shape.

5. What is the characteristic skull shape seen in trigonocephaly?

a) Tower-shaped

b) Triangular forehead

c) Flattened occiput

d) High, pointed skull

Answer: b) Triangular forehead

Rationale: Trigonocephaly results from metopic suture closure, leading to a triangular forehead.

6. Which craniosynostosis type is often linked to hydrocephalus?

a) Brachycephaly

b) Cloverleaf skull

c) Plagiocephaly

d) Scaphocephaly

Answer: b) Cloverleaf skull

Rationale: Multiple sutures closing early cause a severe skull deformity known as the cloverleaf skull, often associated with hydrocephalus.

7. Which of the following syndromes is NOT commonly linked to craniosynostosis?

a) Apert syndrome

b) Marfan syndrome

c) Crouzon syndrome

d) Pfeiffer syndrome

Answer: b) Marfan syndrome

Rationale: Marfan syndrome primarily affects connective tissue and does not typically involve craniosynostosis.

8. What gene mutation causes Apert syndrome?

a) FGFR1

b) FGFR2

c) FGFR3

d) PAX6

Answer: b) FGFR2

Rationale: Apert syndrome is caused by a mutation in the FGFR2 gene, leading to skull and limb abnormalities.

9. What is the key difference between Apert and Crouzon syndromes?

a) Crouzon syndrome causes limb abnormalities

b) Apert syndrome does not affect the skull

c) Crouzon syndrome does not involve hand and foot fusion

d) Apert syndrome does not cause bi-coronal synostosis

Answer: c) Crouzon syndrome does not involve hand and foot fusion

Rationale: Unlike Apert syndrome, Crouzon syndrome affects the skull and facial bones but does not cause limb fusion.

10. Which suture closure leads to oxycephaly?

a) Lambdoid suture

b) Sagittal suture

c) Coronal suture

d) Multiple sutures

Answer: d) Multiple sutures

Rationale: Oxycephaly results from the premature fusion of multiple sutures, leading to a high, tower-shaped skull.

11. Which condition results from early Bicoronal suture closure?

a) Brachycephaly

b) Trigonocephaly

c) Scaphocephaly

d) Dolichocephaly

Answer: a) Brachycephaly

Rationale: Premature closure of the coronal sutures causes a short, wide skull shape known as brachycephaly.

12. What is a common feature of Crouzon syndrome?

a) Cleft palate

b) Beaked nose and proptosis

c) Hand and foot fusion

d) Limb shortening

Answer: b) Beaked nose and proptosis

Rationale: Crouzon syndrome commonly presents with a beaked nose and bulging eyes (proptosis).

13. Which genetic mutation is responsible for Crouzon syndrome?

a) FGFR1

b) FGFR2

c) FGFR3

d) RUNX2

Answer: b) FGFR2

Rationale: Crouzon syndrome is caused by a mutation in FGFR2, affecting skull and facial bone development.

14. Which suture closes last in normal skull development?

a) Metopic suture

b) Sagittal suture

c) Coronal suture

d) Frontozygomatic suture

Answer: d) Frontozygomatic suture

Rationale: The frontozygomatic and frontonasal sutures close last, around 68-72 months.

#Craniosynostosis#Types_of_Craniosynostosis #Skulldeformities_in_infants #Sagittalsynostosis (Scaphocephaly) #Plagiocephalyvscraniosynostosis #Metopicsynostosis(Trigonocephaly) #Cloverleafskull #syndrome #Acrocephalyandoxycephaly #Craniosynostosiscausesandtreatment #SignsOfCraniosynostosisInBabies #HeadShapeAbnormalitiesInInfants #WhatCausesEarlySkullFusion #CraniosynostosisSurgeryOptions #IsCraniosynostosisGenetic#NonSurgicalTreatmentForSkullDeformities#CraniosynostosisAndBrainDevelopment#HowToDiagnoseCraniosynostosis #CranialSutureClosureTimeline#BestHospitalsForCraniosynostosisTreatment  #ApertSyndromeSkullDeformity #CrouzonSyndromeHeadShape #CarpenterSyndromeAndCraniosynostosis #PfeifferSyndromeSkullAbnormalities #CraniosynostosisGeneticSyndromes