Abstract
Background/PurposeIn Hirschsprung disease (HD) surgery, confirming ganglionic bowel is essential. A faster diagnostic method than the current frozen biopsy is desirable. This study investigated whether aganglionic and ganglionic intestinal wall can be distinguished from each other by ultra high frequency ultrasound (UHF ultrasound). MethodsIn an HD center during 2019, intestinal walls of recto-sigmoid specimens from HD patients were examined ex vivo with a 70 MHz UHF ultrasound transducer. Data from four sites were described. Histopathologic analysis was compared to the ultrasonography outcome at each site. Each patient's specimen served as its own control. Results11 resected recto-sigmoid specimens (median 20 cm long [range 6.5–33]) with transition zones of 5 cm (2–11 cm) were taken from children aged 22 days (13–48) weighing 3668 g (3500–5508); 44 key sites were analyzed. There was full concordance for 42/44 (95%) key sites and 10 of 11 (91%) specimens. The specimen with discordance of two key sites contained a segment of aganglionosis (3 cm) and a transition zone (1 cm): the site discordance was limited to the transition zone ends. ConclusionsThis first report on UHF ultrasound in recto-sigmoid HD shows promising results in identifying aganglionosis, transition zones and ganglionic bowel. Further in vivo studies are required.
Highlights
Hirschsprung disease (HD) is characterized by a lack of ganglion cells in the submucosal and myenteric nerve plexuses of the intestinal wall identified by rectal biopsies [1,2,3]
Absence of ganglion cells leads to constriction and obstruction of the affected segment, and HD is treated by surgical resection of the aganglionic intestinal segment
Three children were excluded from the study: two due to aganglionosis stretching above the transversal part of the colon and one primarily operated on at another hospital, undergoing re-TERPT
Summary
Hirschsprung disease (HD) is characterized by a lack of ganglion cells in the submucosal and myenteric nerve plexuses of the intestinal wall identified by rectal biopsies [1,2,3]. Absence of ganglion cells leads to constriction and obstruction of the affected segment, and HD is treated by surgical resection of the aganglionic intestinal segment. The segment to be resected stretches in the oral direction from the anus, most commonly affecting the recto-sigmoid portion, but exact lengths vary individually [4,5,6]. In order to predict the extension of the aganglionic segment when planning surgery, a preoperative colorectal contrast enema could be performed [7,8], but for securing normo-ganglionic bowel, fresh frozen intestinal biopsy is required during surgery [9,10,11]. To date there is no other intraoperative method other than frozen biopsy to secure the level of ganglionic bowel
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