The Extent of the Transition Zone in Hirschsprung Disease

Coyle, David; O’Donnell, Anne Marie; Tomuschat, Christian; Gillick, John; Puri, Prem

doi:10.1016/j.jpedsurg.2019.04.017

Cited by 30 publications

(23 citation statements)

References 19 publications

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“…However, when taking into account TZ lengths much greater than 5 cm, this idiom alone may not prevent a TZ pullthrough. These findings are also in accordance with another recently published series of patients in whom the TZ length was found to be extending up to 13 cm [10].…”

Section: Discussionsupporting

confidence: 93%

Variability of the transition zone length in Hirschsprung disease

Thakkar

Blackburn

Curry

et al. 2020

Journal of Pediatric Surgery

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Section: Discussionsupporting

confidence: 93%

Variability of the transition zone length in Hirschsprung disease

Thakkar

Blackburn

Curry

et al. 2020

Journal of Pediatric Surgery

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“…Given that the length of bowel resected in patients who underwent a TZPT in our series was a mean of 6cm, perhaps we could conclude that 'large' resections were avoided. This is in line with the recommendations of excising >2cm of proximal bowel by White and Langer[ 1 ], or >5cm by Kapur[ 12 ] and Coyle[ 4 ], on the basis of their studies, in order to avoid a TZPT.…”

Section: Discussionsupporting

confidence: 87%

“…I t is well recognized that in Hirschsprung's disease (HD), the change in bowel innervation from distal, abnormal bowel (aganglionic, thickened nerves) to proximal, normal bowel (ganglionic, no thick nerves) is not abrupt but gradual; this change can be rather variable in degree, in depth, and in its distribution around the circumference and length of bowel affected,[ 1 2 3 4 ] and the term “transition zone” (TZ) is used to describe this intervening length of bowel. Therefore, bowel from the TZ is occasionally pulled through in error, resulting in a TZ pull-through (TZPT) – one of the well-recognized complications of surgery for HD.…”

Section: Introductionmentioning

confidence: 99%

Routine use of circumferential ‘Doughnut’ biopsy in pull through surgery for hirschsprung's disease: Advantages and limitations

Murthi

Townley²,

Lindley³

et al. 2021

J Indian Assoc Pediatr Surg

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Aims: The aim of this study was to review the use of the on-table “doughnut” biopsy for frozen section assessment of bowel in the operative management of Hirschsprung's disease (HD). Methods: This was a single-center retrospective review of doughnut histopathology reports, operation notes, and slides from 2010 to 2017. Data were assessed for the presence of transition zone (TZ) features and the subsequent decision as to the level of pull-through. Results: Fifty-five patients had a doughnut biopsy taken as part of their intraoperative frozen section histopathology for pull-through for HD during the study period. Forty-eight required a single doughnut, six required a second more proximal doughnut, and one required a third doughnut. Of the 55 first doughnuts, 37 were identified as normal bowel, 17 were TZ, and not defined in the report in one case. Of the 17 TZ doughnuts, 8 were accepted for pull-through and 7 underwent second doughnuts (normal = 4 and TZ = 3). The third doughnut (one case) was normal. TZ was accepted for pull-through in 10/54 (18.5%) patients despite the use of a doughnut. However, TZ was avoided in six (11.1%), where the single-point biopsy was “normal.” Conclusions: The doughnut allows the entire circumference of pull-through level to be assessed, enabling TZ identification that can be missed by single seromuscular biopsies. This allows identification and avoidance of TZ pull-through, although sometimes, it is accepted for other reasons.

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“…The peri-operative waiting time for microscopy of the frozen biopsy means a prolonged anesthesia time for the child, which constitutes a problem, particularly if repeated biopsies are needed due to difficulties in determining the right level of biopsy macroscopically. With the aim of avoiding any excessive waiting for repeat biopsies, the surgeon might collect the biopsy more proximally than the recommended 5 cm marginal to the transition zone [7] and the resection length taken then becomes inexact. To date there is no other intraoperative method other than frozen biopsy to secure the level of ganglionic bowel.…”

Section: Introductionmentioning

confidence: 99%

Ultra high frequency ultrasonography to distinguish ganglionic from aganglionic bowel wall in Hirschsprung disease: A first report

Granéli

Erlöv

Mitev

et al. 2021

Journal of Pediatric Surgery

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Background/Purpose: In 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). Methods: In 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. Results: 11 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. Conclusions: This 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.

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The Extent of the Transition Zone in Hirschsprung Disease

Cited by 30 publications

References 19 publications

Variability of the transition zone length in Hirschsprung disease

Variability of the transition zone length in Hirschsprung disease

Routine use of circumferential ‘Doughnut’ biopsy in pull through surgery for hirschsprung's disease: Advantages and limitations

Ultra high frequency ultrasonography to distinguish ganglionic from aganglionic bowel wall in Hirschsprung disease: A first report

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