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Table 1 Review of literature on isolated hypoganglionosis from 1978 to 2009

From: Isolated adult hypoganglionosis presenting as sigmoid volvulus: a case report

First author/reference

No. of cases

Specimen

Staining

Treatment

Mortality

Zhang et al. [5]

17

Rectal suction and full-thickness biopsy

AchE

Resection and pull through

No

Rolle et al. [6]

6

Rectal suction and full-thickness biopsy

AchE, NADPH, ICC, c-kit

5 Resection and pull through, 1 enterostomy

No

Kobayashi et al. [2]

3

Full-thickness biopsy

AchE

Enterostomy

All died

Kubota et al. [7]

6

Full-thickness biopsy

S-100

Enterostomy

All died

Meier-Ruge et al. [3]

7

Full-thickness biopsy

AchE, LDH, SDH

Resection and pull through

No

Schaerli et al. [8]

7

Full-thickness biopsy

AchE, LDH, SDH

Resection and pull through

No

Ure et al. [9]

9

Rectal suction biopsy

AchE, LDH, SDH

7 resection and pull through, 2 sphincter myotomy

1 died

Yamataka et al. [10]

1

Full-thickness biopsy

AchE, ICC, c-kit

Enterostomy

No

Munakata et al. [11]

12

Rectal suction and full-thickness biopsy

AchE, silver impregnation

11 resection and pull through, 1 not reported

No

Meier-Ruge [12]

18

Full-thickness biopsy

AchE, LDH, SDH

Not reported

No

Munakata et al. [13]

6

Full-thickness biopsy

AchE, silver impregnation

Not reported

No

  1. Adapted from Dingemann J, Puri P: Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect. Pediatr Surg Int 26:1111-1115 [1] (Permission granted).
  2. AchE = acetylcholinesterase; ICC = interstitial cells of Cajal; LDH = lactate dehydrogenase; NADPH = nicotinamide adenine dinucleotide phosphate; SDH = succinate dehydrogenase.