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Asian Spine J > Volume 20(4); 2026 > Article
Jain, Rathod, Ved, and Vashishtha: Response to the letter to the editor: Dissecting the enigma: insights from a retrospective study from India on congenital craniovertebral junction anomalies
We sincerely thank the authors for their careful reading of our manuscript [1] and for raising important points. We appreciate the opportunity to clarify these issues. Their comments highlight some of the limitations inherent to a retrospective study of a relatively rare and anatomically heterogeneous condition.

Assessment of irreducibility

As described in the methods section, our patients who did not demonstrate reduction on dynamic radiographs underwent sustained cervical traction under general anesthesia with complete muscle paralysis for 10 minutes, using Gardner-Wells tongs and increasing weights, in accordance with the criteria by Wang et al. [2]. Reduction was assessed on intraoperative lateral fluoroscopy.
We acknowledge, however, that this assessment was not independently verified by a second observer, and no defined quantitative threshold, such as a specific residual atlantodental interval, was used to supplement the qualitative assessment of reduction. The decision was based on assessment of qualitative reduction of the dislocation by the senior surgeon as seen on the intraoperative fluoroscopy.
In retrospect, we agree that incorporating a quantitative radiological criterion would have been more accurate. This is particularly relevant in congenital craniovertebral junction abnormalities, where reduction may be incomplete rather than simply reducible or irreducible. We also recognize that the subsequent choice of anterior release was influenced not only by the response to traction but by the underlying bony and vascular anatomy. Thus, the 10-minute traction assessment should be viewed as one component of the overall surgical decision-making process rather than a standalone criterion.

Statistical interpretation of subgroup comparisons

Due to the rarity of the condition, our cohort consisted of only 25 patients, and some of the subgroup cells were necessarily very small. The p-values presented in Tables 2 and 3 of our study [1] were intended as exploratory analyses rather than as confirmatory hypothesis testing.
We accept that, with such sparse data, chi-square approximations may be unreliable and that Fisher’s exact test would generally be more appropriate for comparisons involving very small cell counts. We acknowledge this as a methodological limitation of the analysis which may affect the accuracy of results as a result of a type 1 error.
Accordingly, we would caution against interpreting any individual subgroup p-value as evidence of a definitive association. The main aim of these calculations was to highlight that, within this small cohort, we could not demonstrate an association between anomalies and recovery. The results should therefore be regarded as suggestive rather than definitive. We agree that larger multicenter datasets, with adequately powered analyses and appropriate correction for multiple comparisons, are required before such associations can be meaningfully established.

Referral pattern and generalizability of the congenital cohort

We appreciate this observation and agree that referral bias may have influenced not only the proportion of congenital cases but also the clinical and anatomical spectrum within the congenital cohort.
Our institution is a tertiary referral center that receives a substantial number of patients with traumatic and infective, particularly tuberculous, atlantoaxial instability (AAI). Of the 103 patients with AAI identified during the study period, only 25 (24.27%) had congenital pathology. This differs considerably from the 73% congenital proportion reported by Mehrotra et al. [3]. The difference was one of the reasons we specifically discussed the referral characteristics of our institution.
It is entirely possible that the same referral pattern influenced the congenital subgroup itself. Patients referred to a tertiary center may preferentially represent those with more complex deformities, neurological deficits, unusual bony anatomy, or difficult surgical anatomy, while less severe or asymptomatic congenital abnormalities may remain undiagnosed or be managed elsewhere. Consequently, the anomaly spectrum and severity observed in our cohort may not reflect the full spectrum of congenital AAI in the community.
We therefore agree that our proposed algorithm should not be interpreted as a universally validated treatment pathway. Rather, it represents an experience-based approach developed from the anatomical patterns and surgical challenges encountered at our center. The algorithm is intended to assist with systematic preoperative assessment and planning, while recognizing that the final surgical strategy must remain individualized according to the patient’s anatomy, reducibility, vascular course, and neurological status. We have also acknowledged the need for larger multicenter studies to validate these observations.
Our paper has given direction, in some of the more challenging cases that were encountered by the team, and generated the algorithm accordingly. The same workflow can be followed for simpler cases, by simply omitting the steps which are meant for more complex deformities associated with bony and vascular anomalies. Hence, it may be more widely applicable; however, it would also require some degree of individualization on a case-by-case basis, as rightly pointed out by the authors.

Duration of follow-up and long-term construct durability

We agree with this important limitation. Our study included a 1-year clinical and radiological follow-up, and dynamic radiographs at 1 year were used to assess bone bridge formation. The primary purpose of the study was to evaluate early neurological and radiological outcomes and to describe the surgical strategies used for this complex group of patients.
As the authors rightly suggested, a 1-year follow-up period is insufficient to establish the long-term durability of an occipitocervical or upper cervical construct. Our findings therefore demonstrate the early maintenance of the achieved correction rather than definitive long-term construct durability. We agree that longer radiological surveillance would provide more meaningful information regarding maintenance of the corrected C1–C2 angle, posterior occipitocervical angle, and other craniovertebral parameters, as well as the development of late implant- or adjacent-segment-related complications. Because of the retrospective nature of the study, longer and uniform follow-up data were not available for all patients and could not be reliably incorporated into the present analysis. It also opens the doors to the possibility of following up these patients in the future and publishing the results of these patients in a long-term follow-up.
We are grateful for these observations. They appropriately place the findings of our study in perspective. Our intention was not to suggest that a small, singlecenter retrospective series could provide definitive evidence for the management of congenital AAI, but rather to describe the anatomical variations, surgical challenges, and practical decision-making encountered in a rare and high-risk condition. The study itself has acknowledged its retrospective design, limited sample size, absence of formal power analysis, potential measurement bias, and limited generalizability. We agree that prospective multicenter studies with standardized radiological assessment, longer follow-up, and appropriately powered statistical analyses would be valuable for validating and refining the proposed treatment algorithm.
We thank the authors once again for their constructive comments. We believe that these discussions are particularly valuable in a condition as anatomically variable and surgically demanding as congenital craniovertebral junction instability, where careful interpretation of limited clinical experience remains essential.

Notes

Conflict of Interest

No potential conflict of interest relevant to this article was reported.

Author Contributions

All authors conceived, drafted, critically revised, and approved the final version of this letter.

References

1. Jain D, Rathod T, Ved YP, Vashishtha A. Dissecting the enigma: insights from a retrospective study from India on congenital craniovertebral junction anomalies. Asian Spine J 2026 Mar 17 [Epub]. https://doi.org/10.31616/asj.2025.0267
crossref
2. Wang C, Yan M, Zhou HT, Wang SL, Dang GT. Open reduction of irreducible atlantoaxial dislocation by transoral anterior atlantoaxial release and posterior internal fixation. Spine (Phila Pa 1976) 2006;31:E306–13. https://doi.org/10.1097/01.brs.0000217686.80327.e4
crossref pmid
3. Mehrotra A, Nair AP, Das K, et al. Congenital paediatric atlantoaxial dislocation: clinico-radiological profile and surgical outcome. Childs Nerv Syst 2012;28:1943–50. https://doi.org/10.1007/s00381-012-1801-1
crossref pmid


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