Craniosynostosis not only results in cosmetic deformities but also impacts the growth and development of the child, including speech, behavior, and psychology. The re-operation rates mentioned in the literature vary. Wall et al reported the re-operation rates for single suture synostosis cases to be 5.2% (5 out of 97) [12]. McCarthy et al reported this rate in non-syndromic patients as 13.5% (14 out of 104) [7]. In our study, among the 70 patient who had been operated upon, 6 (8.5%) underwent repeat surgery due to re-synostosis. Therefore, the operation rates due to re-synostosis in non-syndromic patients in this study are comparable with the rates mentioned in the literature.
Several studies till date have focused on specific types of craniosynostosis, and the rates of re-operation mentioned in the literature greatly vary. Wagner et al, who studied non-syndromic bicoronal synostosis cases, reported that 36% of them needed re-operation [11]. Another study focusing on isolated sagittal synostosis reported the re-operation rate to be as high as 70.9% [2]. In our case, it was 20% and 12.5% for bicoronal and sagittal synostosis, respectively. This huge difference in the re-synostosis rates between other studies and ours could be attributed to the fact that we included only patients who underwent repeat surgery in our study and excluded re-synostosis cases that did not require surgery.
The re-synostosis rates have been reported to be higher in syndromic patients compared to non-syndromic patients, in the literature [4]. Foster et al reported in their study that multiple-suture synostosis was noted in 50% of the cases with total re-synostosis of which, 37.5% had sagittal suture synostosis. Proven syndromic cases were excluded from our study; however, multiple-suture synostosis was noted in 2 cases. No evidence of syndromic craniosynostosis was found on genetic analysis and physical examination of these patients and also no re-synostosis was detected.
An increase in the intracranial pressure before the first operation in about 50% of the patients with re-synostosis has been reported in earlier studies [3, 10]. It was suggested that intracranial pressure measurement in craniosynostosis may be helpful, which cannot be determined clearly because of wide variations of the disease [4]. In our study, no intracranial pressure measurement of re-synostosis cases was performed. Diagnosis and treatment were based on the computed tomography scan results and clinical condition of the patients.
Wall et al reported higher re-operation rates in infants who underwent primary surgery before 6 months of age than those who underwent surgery later [12]. However, several studies in the literature could not find any correlation between re-operation rate and the age at which the patient underwent surgery [4, 13]. In our study, the mean age of the patients who underwent their first surgery due to craniosynostosis was 10.9 ± 7.8 months. No correlation between the age at first operation and re-operation was found in this study. However, while determining the appropriate time for the first surgery, it must be kept in mind that craniosynostosis is a progressive deformity of the skull base which may be difficult to fix later.
As various studies in the literature have used numerous surgical techniques and reported a wide variation in the re-operation rate, it is difficult to form a correlation between the type of primary surgery and re-operation [4]. Foster et al reported no difference in the mean increase in head circumference between the groups with and without re-synostosis. Additionally, they demonstrated that enlargement of the skull following the first operation was not related to re-operation [4]. In our study, with the exception of 2 cases with pansynostosis, the head circumference correlated with the age. During the follow-up after surgery, re-synostosis was detected due to cosmetic deformity.
One of the main limitations of this study was the small population size. Cases with early onset were operated earlier while late-onset and mild cases underwent surgery at an older age. All the patients were operated by the same surgeon. Therefore, if there was a technical insufficiency, it might have affected the results.
Early detection and intervention followed by developmental monitoring are vital for improving the chances of infants with craniosynostosis and reducing its associated risks, including developmental delay. A multispecialty-team approach involving Orthopedics, Pediatrics, Neurosurgery, and Plastic Surgery should be considered for better management of such patients.