European J Pediatr Surg Rep. 2015; 03(01): 003-006
DOI: 10.1055/s-0035-1552559
Case Report
Georg Thieme Verlag KG Stuttgart · New York

The Appendix and Aganglionosis. A Note of Caution—How the Histology Can Mislead the Surgeon in Total Colonic Hirschsprung Disease

Victoria Alison Lane
1   Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States
2   Center for Surgical Outcomes Research, Nationwide Children's Hospital, Columbus, Ohio, United States
,
Marc A. Levitt
1   Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States
,
Peter Baker
3   Department of Pathology, Nationwide Children's Hospital, Columbus, Ohio, United States
,
Peter Minneci
1   Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States
2   Center for Surgical Outcomes Research, Nationwide Children's Hospital, Columbus, Ohio, United States
,
Katherine Deans
1   Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States
2   Center for Surgical Outcomes Research, Nationwide Children's Hospital, Columbus, Ohio, United States
› Author Affiliations
Further Information

Publication History

09 December 2014

30 March 2015

Publication Date:
28 May 2015 (online)

Abstract

We present the case of a child with presumed total colonic Hirschsprung disease (HD) to highlight the problems the surgeon is likely to encounter if he/she relies on the appendix alone for histopathologic diagnosis. A newborn male infant, who was presumed to have total colonic aganglionosis when the appendix was found to be aganglionic at the time of initial exploratory laparoscopy, was managed with an ileostomy in the newborn period; however, at the time of his planned pull-through procedure, the rectal biopsy revealed normal ganglion cells. The child was subsequently managed with ileostomy closure and observed for normal feeding and stooling prior to discharge home. We discuss the histopathologic findings of the appendix in separate cases of confirmed total colonic HD seen in our center, and review the normal histopathologic findings of the appendix.

 
  • References

  • 1 Shih TY, Chuang JH, Huang CC. Aganglionosis of the appendix: is it reliable for diagnosis of total colonic aganglionosis?. J Pediatr Gastroenterol Nutr 1998; 27 (3) 353-354
  • 2 Anderson KD, Chandra R. Segmental aganglionosis of the appendix. J Pediatr Surg 1986; 21 (10) 852-854
  • 3 Kamoshita S, Landing BH. Distribution of lesions in myenteric plexus and gastrointestinal mucosa in lipidoses and other neurologic disorders of children. Am J Clin Pathol 1968; 49 (3) 312-318
  • 4 Hanani M. Multiple myenteric networks in the human appendix. Auton Neurosci 2004; 110 (1) 49-54
  • 5 Singh UR, Malhotra A, Bhatia A. Eosinophils, mast cells, nerves and ganglion cells in appendicitis. Indian J Surg 2008; 70 (5) 231-234
  • 6 Xiong S, Puri P, Nemeth L, O'Briain DS, Reen DJ. Neuronal hypertrophy in acute appendicitis. Arch Pathol Lab Med 2000; 124 (10) 1429-1433
  • 7 Miller SM, Narasimhan RA, Schmalz PF , et al. Distribution of interstitial cells of Cajal and nitrergic neurons in normal and diabetic human appendix. Neurogastroenterol Motil 2008; 20 (4) 349-357
  • 8 Knowles CH, De Giorgio R. Observations on a vestigial organ: a potential surrogate for enteric neuromesenchymal disease. Neurogastroenterol Motil 2008; 20 (4) 263-268
  • 9 N-Fékété C, Ricour C, Martelli H, Jacob SL, Pellerin D. Total colonic aganglionosis (with or without ileal involvement): a review of 27 cases. J Pediatr Surg 1986; 21 (3) 251-254
  • 10 Sharp NE, Pettiford-Cunningham J, Shah SR , et al. The prevalence of Hirschsprung disease in premature infants after suction rectal biopsy. J Surg Res 2013; 184 (1) 374-377
  • 11 Knowles CH, De Giorgio R, Kapur RP , et al. Gastrointestinal neuromuscular pathology: guidelines for histological techniques and reporting on behalf of the Gastro 2009 International Working Group. Acta Neuropathol 2009; 118 (2) 271-301