Craniofacial surgery involves correcting and reconstructing both congenital and acquired abnormalities affecting the head, skull, face, jaw, and related structures.

This complex treatment necessitates a collaborative approach, with specialists such as plastic surgeons, craniofacial surgeons, neurosurgeons, pediatric anesthetists and intensivists, ophthalmologists, ENT surgeons, speech therapists, and orthodontists working together.

Sakra World Hospital stands as a unique healthcare facility in India, offering innovative techniques like Minimally Invasive Endoscopic-assisted surgery and 3D-printed helmet therapy for children with such conditions in managing craniosynostosis, a congenital condition where an infant's skull sutures fuse prematurely, altering the skull's growth pattern.

How does craniosynostosis effect the skull?

Because the skull cannot expand perpendicular to the fused suture of the skull, it compensates by growing more in the direction parallel to the fused sutures. This growth adjustment occasionally offers sufficient room for the developing brain, but it also results in unusual head shapes and facial features.

When the compensatory growth isn't adequate for accommodating the growing brain, craniosynostosis leads to heightened pressure within the skull. This heightened pressure can potentially lead to vision problems, challenges in eating and sleeping, and cognitive developmental issues, often accompanied by a significant decrease in IQ.

Types of Craniosynostosis

• Non syndromic craniosynostosis – Metopic, Coronal, , Sagittal, Lambdoid, Multisuture.
• Syndromic craniosynostosis – Craniosynostosis occurs as a part of a syndrome, Apert, Pfeiffer, Crouzon, Muenke, Saethre­Chotzen.

Presentations of various craniosynostosis

• Unicoronal craniosynostosis - Unicoronal craniosynostosis is when one of the coronal sutures in the skull fuses before birth, leading to a flattened forehead and raised eye socket on one side (Plagiocephaly). These coronal sutures extend from the anterior fontanelle down the side of the forehead.
• Bicoronal craniosynostosis - Bicoronal craniosynostosis occurs when both coronal sutures fuse before birth, resulting in a wider head that becomes flattened from front to back (Brachycephaly). These coronal sutures also extend from the anterior fontanelle down the side of the forehead.
• Metopic craniosynostosis - Metopic craniosynostosis is characterized by the fusion of the metopic suture before birth, causing the head to have a triangular shape (Trigonocephaly). The metopic suture runs along the front of the skull.
• Sagittal craniosynostosis - Sagittal craniosynostosis involves the fusion of the sagittal suture before birth, causing the head to become narrower and longer (Scaphocephaly). The sagittal suture runs along the center of the skull.
• Lambdoid craniosynostosis - Lambdoid craniosynostosis occurs when one of the lambdoid sutures at the back of the head fuses before birth, resulting in a flatness at the back of the head (posterior plagiocephaly or brachiocephaly).
• Multisuture Synostosis - Multisuture Synostosis involves the premature closure of many or all sutures of the skull, leading to a small head (Microcephaly) and severe restriction of brain growth.

Diagnosis

Diagnosis is established through clinical evaluation aided by skull X-ray, 3D CT scan of the head, and (sometimes) MRI to assess the condition of the brain.

Treatment of craniosynostosis

• Age less than 6 months - An open surgery (Fronto-orbital remodeling or cranial vault remodeling) is performed for reshaping the skull of the child. The cut is made at the top of the head from one ear to the other ear. The affected suture is removed by a neurosurgeon and the bones of the skull are moulded into a normal shape by a craniofacial plastic surgeon.
• Age more than 6 months - Minimally invasive (Keyhole), endoscopic assisted strip craniectomy followed by Helmet (3D printed) therapy for 1-2 years. In this, the fused suture in question is removed using a keyhole incision with minimally invasive techniques. It involves shorter hospital stays, minimal blood loss, avoidance of blood transfusion, and quick recovery. This is followed by 1-2 years of helmet therapy to mould the shape of the skull and facilitate proper growth of the brain.

Syndromic craniosynostosis

• Posterior vault expansion/Fronto-orbital advancement - An open procedure to increase the size of the skull to give more space for the brain to grow. This is usually done in the first year of life. Multiple surgeries are often required in syndromic craniosynostosis, and the timing of these surgeries depends upon the condition of the child. Lefort III osteotomy and advancement after age 4 are sometimes required to improve obstructive sleep apnoea and airway issues. Extra support for your child is sometimes required. They are – Physical therapy, Occupational therapy, Speech-language therapy, and Special education.

What happens in the keyhole skull expansion surgery?

Your child will undergo a thorough assessment by a pediatric neuroanesthetist before the surgery. An eye examination will also be conducted. The surgery, performed under general anesthesia, typically lasts 1-2 hours and involves a small scalp incision, resulting in minimal scarring and minimal blood loss. Following the procedure, your child will spend a night in the ICU and might occasionally require a blood transfusion. Pain management by our critical care specialists will be highly effective in the ICU. Throughout the recovery period, we encourage regular feeding. The next day, your child will be moved to a standard ward. Generally, hospital discharge occurs around the 3rd day after the head dressing is removed. Subsequently, you'll be able to give your baby a normal head shower.

What happens after discharge?

Following a post-operative examination at one week, Helmet therapy is usually initiated in collaboration with our Orthotic partners (KARE or Osteo3D). This treatment will continue for a period of 1-2 years until a desirable head shape is attained. Depending on the progress observed, there might be a need to replace the helmet once or twice. Regular appointments will be scheduled for monitoring with the Craniofacial surgeon and Neurosurgeon.

What happens in an open Fronto orbital advancement surgery?

A pre-operative evaluation will be conducted by a pediatric neuroanesthetist and an ophthalmologist. The surgery, performed under general anesthesia, typically lasts for about 4-5 hours. Blood transfusion is commonly required during and after the surgery for most children. To access the skull, an incision will be made from one ear to the other. While the neurosurgeon safeguards the brain, the plastic surgeon or craniofacial surgeon will reshape the forehead bones. Absorbable plates and screws are usually used to secure the bones, occasionally supplemented by stainless steel wires or stitches. The incision will be closed using absorbable stitches, and a headbandage will be applied.

The first night after the surgery will be spent in the ICU, and afterward, your child will be moved to a regular ward. Expect notable swelling of the head and face, possibly causing the eyes to close for a few days. Over the course of the week, the swelling will gradually diminish. Typically, your child will be discharged around the 6th or 7th day after the head bandage is removed. Following that, you'll be able to give your baby a head shower.

What happens after discharge?

You will attend regular follow-up appointments with the plastic/craniofacial surgeon and neurosurgeon. This is usually a one-time procedure, and if there is no associated syndrome, that’s all your child will ever need. After the open remodeling procedure, there is no requirement for Helmet therapy. You may observe some gaps in the skull, which usually close naturally within 2-3 years. Additionally, the scar is commonly discreetly concealed within the hair.

What complications can I expect?

This is a major surgery, and it's important for you to have a comprehensive understanding of potential complications. Common issues include infection, bleeding, scarring, swelling, and pain, which can generally be addressed with medications. There might be small tears in the brain's covering, which are typically addressed during the surgery. Extremely rarely, there could be a slight risk of eye or brain injury.