Introduction: Craniosynostosis is defined as premature fusion of the cranial sutures. This can lead to abnormal head shape, abnormal facial features, visual impairment due to papilloedema, obstructive sleep apnea due to midface hypoplasia or an impairment of neuro-cognitive development.
Methods: After obtaining ethical clearance, we conducted a cross sectional study in our department which included 60 cases of craniosynostosis operated in our hospital from 2010 till 2015 and who were on regular follow up. Data was collected including name, age, gender, involved sutures, other medical comorbidities, whether syndromic craniosynostosis or not, whether signs of intracranial hypertension were present or not, any findings on MRI, type of surgery performed, complications of surgery (if any) and findings at follow up. To be able to analyse the surgical results, we used the seven category classification system used by Sloan et al.
Results: In non syndromic cases (53), scaphocephaly and trigonocephaly groups had the maximum number of cases (11 each).
4 out of 5 cases of Crouzon’s syndrome (80%) and 6 out of 8 cases of oxycephaly (75%) had signs of raised intracranial pressure.
Chiari malformation was present in 80% of the cases of Crouzon’s syndrome and 62.5% of the cases of oxycephaly.
Mean age at first surgery in the 60 cases was 3.85years. Mean age at first surgery was earliest in the anterior plagiocephaly and trigonocephaly groups, i.e. 1.67 years and 1.83 years respectively.
There was a significant association between syndromic craniosynostosis/ oxycephaly and higher Sloan et al class (compromised correction).
All 11 patients of trigonocephaly showed class I correction.
Conclusions: The incidence of metopic synostosis is rising.
Mean age at first surgery was very high due to late presentation of the cases.
Chiari malformation, intracranial hypertension and peri-operative complications were a frequent finding in multi-sutural and syndromic craniosynostosis.
The best surgical outcome and the least peri-operative complications were seen in the trigonocephaly group.
Patient Care: This paper highlights the difference between 2 subgroups of craniosynostosis -
1) Multi-sutural and syndromic craniosynostosis
2) Single suture craniosynostosis
Multi-sutural and syndromic craniosynostosis are frequently associated with chiari malformation, visual symptoms due to raised intra-cranial pressure and a higher frequency of peri - operative complications when compared with single suture craniosynostosis.
Mean age at first surgery in our study was 3.85 years which is very high. It is very important to treat cases of craniosynostosis at an early age (around 6 months of age). Paediatricians/ Obstetricians and medical teams should be vigilant so that cases of craniosynostosis are detected and treated early.
Learning Objectives: Syndromic craniosynostosis and multi-sutural craniosynostosis surgeries are complex surgeries and require a certain level of expertise. Peri- operative complications are more common in these cases as compared to single suture craniosynostosis.
MRI Brain and cranio-vertebral junction should be done in patients of craniosynostosis especially syndromic cases to look for Chiari malformation or intrinsic brain abnormalities.
References: Slater BJ, Lenton KA, Kwan MD, Gupta DM, Wan DC, Longaker MT. Cranial sutures: a brief review. Plast Reconstr Surg. 2008 April;121(4):170e–8e.
Sloan GM, Wells KC, Raffel C, McComb JG. Surgical treatment of craniosynostosis: Outcome Analysis of 250 consecutive patients. Pediatrics. 1997 July; Vol 100(1):E2