A review of the management of single-suture craniosynostosis, past, present, and future

Abstract

<jats:sec><jats:title>BACKGROUND</jats:title><jats:p>Craniosynostosis is a condition in which 2 or more of the skull bones fuse prematurely. The spectrum of the disorder most commonly involves the closure of a single suture in the skull, but it can also involve syndromic diagnoses in which multiple skull bones and/or bones outside of the cranium are affected. Craniosynostosis can result in cosmetic deformity as well as potential limitations in brain growth and development, and the neurocognitive impact of the condition is just starting to be studied more thoroughly. Our knowledge regarding the genetics of this condition has also evolved substantially. In this review, the authors explore the medical and surgical advancements in understanding and treating this condition over the past century, with a focus on how the diagnosis and treatment have evolved.</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>In this review article, the authors, who are the leaders of a craniofacial team at a major academic pediatric hospital, focus on single-suture craniosynostosis (SSC) affecting the 6 major cranial sutures and discuss the evolution of the treatment of SSC from its early history in modern medicine through the current state of the art and future trends. This discussion is based on the authors’ broad experience and a comprehensive review of the literature.</jats:p></jats:sec><jats:sec><jats:title>SUMMARY</jats:title><jats:p>The management of SSC has evolved substantially over the past 100 years. There have been major advances in technology and medical knowledge that have allowed for safer treatment of this condition through the use of newer techniques and technologies in the fields of surgery, anesthesia, and critical care. The use of less invasive surgical techniques along with other innovations has led to improved outcomes in SSC patients. The future of SSC treatment will likely be guided by elucidation of the causes of neurocognitive delay in these children and assessment of how the timing and type of surgery can mitigate adverse outcomes.</jats:p></jats:sec>

Craniofacial Corrective Surgery in Syndromic Craniosynostosis

“…Unexpected airway issues are other complications following craniofacial surgery, in which emergency reintubation or even prolonged ventilation is needed due to severe upper airway oedema. Decision for steroid covers for upper airway oedema is most of the time anecdotal but some reported beneficial in preventing facial oedema postoperatively [45][46][47][48][49][50].…”

“…Infection is the most common complication in the form of osteitis/osteomyelitis, meningitis, or an intracranial abscess, occurring in 6.2% of transcranial cases [25]. Overall reported infection rates ranging from 1-14% in large centre series [27,32,[39][40][41][42][47][48][49][50][51].…”

“…Majority of craniofacial surgery confined to extradural showed lower incidence of neurologic impairment. Several reports from major craniofacial centers has shown very low or no permanent neurologic deficits directly attributable to surgery [27,30,37,[50][51][52]. Blindness for example, is an unwanted complication in craniofacial surgery that post a real risk of blindness following surgery.…”

“…Since 1970's to 1988, rate of mortality in craniofacial surgery were reported ranging from 1 to 2% all over the world [47][48][49][50][51]. However, with development of the technology, deeper understanding of the craniofacial surgery and its risks with emphasis on multidisciplinary approach, the numbers of mortality have dropped to 0.1-0.8% in some centers [24][25][26][27][28][29].…”

“…Early surgical intervention is always aiming for better corneal protection and to create spaces for constricted brain. Late surgical intervention conversely aiming for more stable bony correction and less likely for subsequent surgical intervention [50]. However, our center practice on more indication and need-based approach, surgical intervention at the best possible timing for patients in term of growing stage, the indication need for the surgery and patient's general health condition [4].…”