Craniofacial surgery is a specialized field within plastic surgery that focuses on correcting congenital or acquired abnormalities in the head and face. A/Prof Damian Marucci is a consultant Craniofacial Surgeon at the Sydney Children’s Hospital Network (SCHN) campus at the Children’s Hospital at Westmead (CHW). The SCHN Craniofacial Service is a Multi Disciplinary Team (MDT) across two campuses (CHW and Sydney Children’s in Randwick) comprising plastic surgeons, neurosurgeons, ophthalmologists, speech therapists, ENT, orthotics, orthoptics, radiologists, anaesthetists and specialised senior Registered Nurses. A/Prof Marucci completed Fellowships in Craniofacial Surgery at the unit in Oxford as well as at Great Ormond Street Hospital for Sick Children in London. A/Prof Marucci has been part of the Craniofacial Service at CHW for the past 16 years. His work there comprises running clinics assessing children with craniofacial issues as well as busy operating lists and performing research.

A/Prof Marucci assesses children with craniofacial conditions in the MDT at CHW. He does not see children with craniofacial conditions in his private rooms, as all MDT members are important in the assessment and management of these often complex conditions.Medical referrals to the Craniofacial MDT can be made through Consultmedwww.consultmed.co.

One of the more common conditions treated by the Craniofacial MDT is craniosynostosis, a condition where the sutures between the bones in an infant’s skull close prematurely. Below is a brief overview of the different types of craniosynostosis, various surgical options, and potential complications associated with each procedure.

I. Understanding Craniosynostosis:        

Craniosynostosis refers to the premature fusion of one or more of the sutures in an infant’s skull. Sutures are fibrous bands of tissue that connect the individual bones of the skull, and their premature fusion can lead to abnormal skull growth and shape. It can also put pressure on the brain, resulting in developmental delay and raised intracranial pressure. There are several types of craniosynostosis, each affecting different sutures:

1. Sagittal Synostosis: The sagittal suture runs from the fontanel (soft spot) at the top of the head to the back. Premature fusion of this suture leads to a long, narrow skull shape, called “scaphocephaly”.
2. Coronal Synostosis: The coronal sutures run from ear to ear on either side of the skull. If one half of the coronal suture is fused (UnicoronalSynostosis), it results in an asymmetrical forehead with a bulge on one side and flattening of the other. On the affected side, the roof of the orbit is elevated and the root of the nose is deviated. If both side of the coronal suture are fused (BicoronalSynostosis), the face and forehead will be symmetrical, but the head looks “short” when viewed from the side. Bicoronal synostosis may be associated with other issues such as webbing/fusion of the fingers, hearing loss, cleft palate and underdevelopment of the cheekbones.
3. Metopic Synostosis: The metopic suture runs from the top of the head to the nose. Early closure can cause a triangular-shaped forehead, giving the appearance of a pointed skull. This is called “trigonocephaly”.
4. Lambdoid Synostosis: The lambdoid sutures run along the back of the skull. Fusion in this area can lead to flattening or asymmetry of the back of the head. This is rarest type of craniosynostosis. It needs to be differentiated from positional flattening from a baby lying more on one of the head or the other, a condition called “positional plagiocephaly”. Positional Plagiocephaly is discussed in more detail below

II. Surgical Options for Craniosynostosis:

Craniofacial surgeons employ various surgical techniques to address craniosynostosis, depending on the type and severity of the condition and the age of the child. Here are some common surgical options:

A. Strip Craniectomy and Helmet Therapy:

1. Procedure: Strip craniectomy involves the removal of a strip of bone from the fused suture, allowing the brain to grow more freely. Subsequently, helmet therapy is often employed to mold the skull into a more normal shape as the infant’s head continues to grow.The surgery usually takes less than an hour and children are discharged home the following day. The helmet therapy starts a few days later.
2. Indications: This approach can be used for all types of craniosynostosis involving one suture. It is most effective when performed early in infancy, between the ages of 2.5 – 4 months of age.
3. Advantages: Strip craniectomy is less invasive compared to some other procedures. Only one or two small scars are used to remove the strip of fused bone. The helmet therapy can help guide the skull into a more typical shape. Blood loss is minimised as is the risk of blood transfusion. It has a high success rate, meaning that children avoid the larger, more invasive types of surgical correction.
4. Complications: Potential complications include incomplete correction, discomfort during helmet therapy, the small need for a blood transfusion, infection, and the need for additional interventions as the child grows. The helmet ideally needs to be worn 23 hours a day until the child is over the age of 9 months.

B. Fronto Orbital Advancement (FOA) Surgery:

1. Procedure: FOA involves removing the bones of forehead and above the orbits, reshaping them and then replacing them onto the skull base using dissolving plates and screws. It is considered to be a routine but major craniofacial procedure. The surgery takes a few hours and is performed by plastic surgeons and neurosurgeons working together. Children remain in hospital for around 5 days afterwards.
2. Indications: FOA is used to treat types of craniosynostosis of the front of the skull, where the forehead needs to be reshaped or repositioned, such as metopic craniosynostosis, unicoronal synostosis or bicoronal craniosynostosis.
3. Advantages: FOA provides a more dramatic improvement in forehead shape and givers the brain room to grow. It is a routine “workhorse” craniofacial procedure performed at busy craniofacial units around the world.In experienced units, like the Craniofacial Service at the SCHN, the complication rate is low.
4. Complications: Complications may include the risk of infection, bleeding, risk of damaging the dura (outside lining of the brain), asymmetry, scarringand the need for further surgeries to optimise the aesthetic outcome when the child has finished growing.

C. Spring Cranioplasty:

1. Procedure: Spring craniectomy is an option for children with sagittal craniosynostosis who are too old for strip craniectomy and helmet therapy. It is a two stage procedure. The first procedure involves unfusing the fused suture by cutting through it. Two metallic springs are then placed to make the skull wider. These are removed at a second smaller procedure 3 months later.
2. Indications: Spring cranioplasty is mainly used to treat sagittal craniosynostosis, although a similar technique can be used to treat patients with bicoronal craniosynostosis to move back of the skull posteriorly.. It is most effective when performed early in infancy, between the ages of 5-7 months of age.
3. Advantages: Spring cranioplasty is less invasive compared to some other procedures. Blood loss is minimised as is the risk of blood transfusion. It has a high success rate, meaning that children avoid the larger, more invasive types of surgical correction.
4. Complications: Potential complications include incomplete correction, discomfort during helmet therapy, the small need for a blood transfusion, infection of the springs, and the need for additional interventions as the child grows.

D. Total Calvarial Remodelling:

1. Procedure: This extensive procedure involves the complete removal of the affected skull bones, reshaping them, and then reattaching them. It allows for a thorough reconstruction of the entire skull.
2. Indications: Total calvarial remodelling is most commonly usedto treat children with sagittal synostosis who are too old for either strip craniectomy or spring cranioplasty.
3. Advantages: This approach provides a comprehensive and customized solution for severe head shape deformities. The procedure gives the brain room to grow and normalises the shape of the skull.
4. Complications: This is a major surgical procedure. Blood transfusions are commonly required. Other risks include infection, asymmetry, risks of damaging the dura (lining of the brain),asymmetryand the need for subsequent interventions.

III. Potential Complications of Craniofacial Surgery:

1. Infection: Surgical procedures, especially those involving the skull, carry a risk of infection. Infections may occur in the skin, deeper down around the bone or brain, in the lungs or in the bladder. Surgeons take precautions, such as administering antibiotics, to minimize this risk.
2. Bleeding: The blood vessels around the brain are large and significant blood loss can occur, requiring blood transfusions. The surgical and anaesthetic teams are careful to minimise bleeding, support the blood pressure, replace blood as needed and carefully monitor the patient during the surgery.
3. Scarring: Incisions made during surgery may result in visible scars within the scalp. However, craniofacial surgeons are skilled in placing incisions strategically to minimize the visibility of scars.
4. Cerebrospinal Fluid (CSF) Leak: The brain floats is cerebrospinal fluid (CSF). The CSF is contained within a tough bag or membrane, called the dura. The bone sits outside the dura. In removing bones of the skull, sometime a hole can be made in the dura. This is usually repaired straight away inorder to prevent a CSF leak. Rarely, CSF can continue to leak after the surgery, which may require further surgery or treatment.
5. Neurological Issues: While rare, complications such as neurological deficits or developmental delays may occur. Regular follow-up appointments and developmental assessments are essential to monitor a child’s progress.
6. Need for Revisions: In some cases, additional surgeries may be required to address ongoing issues or optimize the cosmetic outcome as the child grows.

III. Positional Plagiocephaly:

Plagiocephaly, a flattening of one side of the back of the head, is a common condition affecting infants. It is estimated that 40 -50% of all children have some degree of positional plagiocephaly and it forms the overwhelming majority of all cases referred to the Craniofacial MDT for assessment. While positional plagiocephaly, caused by external pressure on the head, is typically harmless and resolves on its own, lambdoid craniosynostosis, premature fusion of the lambdoid suture, can lead to serious complications if left untreated. Differentiating between these two conditions is crucial for ensuring appropriate management.

Understanding Positional Plagiocephaly

Positional plagiocephaly arises from consistent pressure on one side of the infant’s head. This can occur due to factors such as:

• Sleeping position: Infants who spend excessive time on their backs, especially without tummy time, are more prone to positional plagiocephaly.
• Torticollis: A tightness in the neck muscles can restrict head movement, leading to uneven pressure on the head.
• Intrauterine positioning: Head molding during pregnancy can contribute to plagiocephaly. It is especially common in twin and triplet births.

Characteristics of Positional Plagiocephaly

Positional plagiocephaly typically presents with the following characteristics:

• Flattening of one side of the back of the head, often with a parallelogram or trapezoidal shape
• Visible asymmetry of the ears, with one ear appearing more forward than the other

Additional Diagnostic Tools

In some cases, additional diagnostic tools may be employed to confirm the diagnosis:

• Plain Skull X-Ray (SXR): while the accuracy is moderate, a SXR may demonstrate that the sutures have not fused, in which case the diagnosis of positional plagiocephaly becomes more likely
• Computed tomography (CT) scan: Provides a cross-sectional view of the skull to visualize the sutures. This is the best way to tell if an unusual head shape is due to positional plagiocephaly or craniosynostosis.

Implications of Positional Plagiocephaly

Positional plagiocephaly is a benign condition that does not affect the development of the underlying brain in any way. It tends to improve slightly, but often not completely with time. Children are never teased about the shape of the back of their head. There has never bee a 5 year old, or a 15 year old or a 25 year old present to the Craniofacial MDT requested cosmetic correction for a persistent positional plagiocephaly.

Management of Positional Plagiocephaly

Positional plagiocephaly typically resolves on its own as the infant becomes more mobile and spends less time on their back. Simple measures, such as encouraging tummy time and varying sleeping positionscan help facilitate normal head shape development. A consultation with a physiotherapist with an interest in paediatrics is useful to ensure that there are no issues with neck movement.

Many parents are interested in helmet therapy for positional plagiocephaly. This is not available through the Children’s Hospital at Westmead. No randomised control trials have demonstrated any benefit of helmet therapy over simple repositioning. However, parents are free to pursure helmet therapy privately. Private helmet providers can be found in all major cities and towns in Australia.

Conclusion:

Craniofacial surgery plays a crucial role in addressing craniosynostosis, a condition that can significantly impact a child’s skull development. The choice of surgical intervention depends on the type and severity of craniosynostosis, and each procedure carries its own set of potential complications. However, with advancements in surgical techniques and careful postoperative management, craniofacial surgeons can achieve remarkable results, improving both the aesthetic appearance and functional outcomes for affected children. It is important for parents and caregivers to consult with experienced craniofacial surgeons like A/Prof Damian Marucci, discuss potential risks and benefits, and actively participate in the decision-making process to ensure the best possible outcome for their child.