To the Editor,

We read the paper written by Rojas-Gutierrez et al.¹ on sigmoid volvulus (SV) complicating Hirschsprung disease (HD). HD complicated by SV is a very rare clinical entity with about 34 cases reported in 26 papers to date²,³. Our comments relate to this comorbidity based on our 56.5-year (from June 1966 to January 2023) and 1063-case SV experience, the most comprehensive monocenter SV series over the world⁴.

First, HD is seen in 0.6-3% of SV cases². Hence, 2 (0.2%) of our patients had SV complicating HD; both were young adults such as the authors’ case and with SV recurrence following endoscopic decompression. Most likely due to the rarity of this comorbidity, its pathophysiology is not clearly defined in the literature²,⁵. In our opinion and experience, the cause and effect relation between SV and HD may be explained by two different mechanisms. In some cases, HD initiates SV by triggering the twisting of the sigmoid colon, while HD mimics SV by inducing an obstruction-like clinical picture in the remained patients. No matter which mechanism is more effective, our experiments demonstrate that the denominator is the impairment or absence of the ganglion cells of the intestinal plexus in SV and HD.

Second, although the authors could not take the advantages of endoscopy, definitive surgery following endoscopic decompression of SV and histopathological evaluation of rectal biopsy materials is the preferred management option in patients with HD complicated by SV²,⁵. On the other hand, inadequate resection and unnoticeable HD are the most important causes of recurrent SV. For this reason, in our opinion, resection of a maximum length of the colon involving all aganglionic segments and enabling a tension-free anastomosis is essential in such cases.

We congratulate the authors and we look forward to their reply.