Endorectal pullthrough surgery is integral in the treatment of patients with Hirschsprung disease. Several different surgical procedures exist, which share as common goals to excise the aganglionic segment and upstream transition zone and attach ganglionic bowel just proximal to the anal canal. The operation requires collaboration between surgeon and pathologist to localize ganglionic bowel and prevent retention of transition zone. Intraoperative frozen sections are extremely important, first to establish that ganglion cells are present and subsequently to exclude features of transition zone (partial circumferential aganglionosis, myenteric hypoganglionosis, and submucosal nerve hypertrophy) at the proximal surgical (anastomotic) margin. Postoperative histopathological analysis of resection specimens should be tailored to document distal aganglionosis, document the length of the aganglionic segment and its proximity to the anastomotic margin, and confirm that transition zone has been resected completely. Adherence to the recommendations described in this review will reduce the likelihood of transition zone pullthrough and should decrease the incidence of persistent postoperative obstructive symptoms.
FrongiaGGuntherPSchenkJP, et al.Contrast enema for Hirschsprung disease investigation: diagnostic accuracy and validity for subsequent diagnostic and surgical planning. Eur J Pediatr Surg2016; 26: 207–214.
2.
ProctorMLTraubiciJLangerJC, et al.Correlation between radiographic transition zone and level of aganglionosis in Hirschsprung’s disease: implications for surgical approach. J Pediatr Surg2003; 38: 775–778.
3.
SwensonOBillAHJr. Resection of the rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megaoclon. Surgery1948; 24: 212–220.
4.
HoehnerJCEinSHShandlingBKimPC. Long-term morbidity in total colonic aganglionosis. J Pediatr Surg1998; 33: 961–965; discussion 965–966.
5.
CoranAGTeitelbaumDH. Recent advances in the management of Hirschsprung’s disease. Am J Surg2000; 180: 382–387.
6.
WildhaberBETeitelbaumDHCoranAG. Total colonic Hirschsprung’s disease: a 28-year experience. J Pediatr Surg2005; 40: 203–206. discussion 206–207.
7.
BischoffALevittMAPenaA. Total colonic aganglionosis: a surgical challenge. How to avoid complications?Pediatr Surg Int2011; 27: 1047–1052.
8.
LaneVALevittMABakerPMinneciPDeansK. The appendix and aganglionosis. A note of caution – how the histology can mislead the surgeon in total colonic Hirschsprung disease. Eur J Pediatr Surg Rep2015; 3: 3–6.
9.
ShihT-YChuangJ-HHuangC-C. Aganglionosis of the appendix: is it reliable for diagnosis of total colonic aganglionosis?J Pediatr Gastroenterol Nutr1998; 27: 353–354.
10.
AndersonKDChandraR. Segmental aganglionosis of the appendix. J Pediatr Surg1986; 21: 852–854.
KapurRPKennedyAJ. Histopathologic delineation of the transition zone in short-segment Hirschsprung disease. Pediatr Dev Pathol2013; 16: 252–266.
14.
CoeACollinsMHLawalTLoudenELevittMAPenaA. Reoperation for Hirschsprung disease: pathology of the resected problematic distal pull-through. Pediatr Dev Pathol2012; 15: 30–38.
15.
SoaveF. A new surgical technique for treatment of Hirschsprung’s disease. Surgery1964; 56: 1007–1014.
16.
TeitelbaumDHWulkanMLGeorgesonKELangerJCHirschsprung disease. In: ZieglerMM, et al.(ed). Operative Pediatric Surgery, New York, NY: McGraw Hill, 2014, pp. 571–591.
TeitelbaumDHCilleyREShermanNJ, et al.A decade of experience with the primary pull-through for hirschsprung disease in the newborn period: a multicenter analysis of outcomes. Ann Surg2000; 232: 372–380.
19.
LangerJCDurrantACde la TorreL, et al.One-stage transanal Soave pullthrough for Hirschsprung disease: a multicenter experience with 141 children. Ann Surg2003; 238: 569–583. discussion 583–565.
20.
SantosMCGiacomantonioJMLauHY. Primary Swenson pull-through compared with multiple-stage pull-through in the neonate. J Pediatr Surg1999; 34: 1079–1081.
21.
KapurRP. Histology of the transition zone in Hirschsprung disease. Am J Surg Pathol2016; 40: 1637–1646.
22.
GherardiGJ. Pathology of the ganglionic-aganglionic junction in congenital megacolon. Arch Pathol1960; 69: 520–528.
23.
WhiteFVLangerJC. Circumferential distribution of ganglion cells in the transition zone of children with Hirschsprung disease. Pediatr Dev Pathol2000; 3: 216–222.
24.
SwaminathanMKapurRP. Counting myenteric ganglion cells in histologic sections: an empirical approach. Hum Pathol2010; 41: 1097–1108.
25.
KnowlesCHVeressBKapurRP, et al.Quantitation of cellular components of the enteric nervous system in the normal human gastrointestinal tract – report on behalf of the Gastro 2009 International Working Group. Neurogastroenterol Motil2011; 23: 115–124.
26.
DoiTKobayashiHYamatakaALaneGJMiyanoT. Complete innervation profile of whole bowel resected at pull-through for Hirschsprung’s disease. Unexpected findings. Pediatr Surg Int2005; 21: 889–898.
27.
MiuraHOhiRTsengSWTakahashiT. The structure of the transitional and aganglionic zones of Auerbach's plexus in patients with Hirschsprung’s disease: a computer-assisted three-dimensional reconstruction study. J Pediatr Surg1996; 31: 420–426.
28.
KapurRPArnoldMAConcesMR, et al.Remodeling of rectal innervation after pullthrough surgery for Hirschsprung disease: relevance to criteria for the determination of retained transition zone. Pediatr Dev Pathol2019; 22: 292–303.
29.
Monforte-MunozHGonzales-GomezIRowlandJMLandingBH. Increased submucosal nerve trunk caliber in aganglionosis. A “positive” and objective finding in suction biopsies and segmental resections in Hirschsprung’s disease. Arch Pathol Lab Med1998; 122: 721–725.
30.
LawalTAChatoorgoonKCollinsMHCoeAPenaALevittMA. Redo pull-through in Hirschprung’s disease for obstructive symptoms due to residual aganglionosis and transition zone bowel. J Pediatr Surg2011; 46: 342–347.
31.
LowichikAWeinbergAG. Eosinophilic infiltration of the enteric neural plexuses in Hirschsprung’s disease. Ped Pathol Lab Med1997; 17: 885–891.
SchmittenbecherPPSacherPCholewaD, et al.Hirschsprung’s disease and intestinal neuronal dysplasia – a frequent association with implications for the postoperative course. Pediatr Surg Int1999; 15: 553–558.
34.
Meier-RugeW. Epidemiology of congenital innervation defects of the distal colon. Virchows Arch A Pathol Anat Histopathol1992; 420: 171–177.
35.
UreBMHolschneiderAMMeier-RugeWA. Neuronal intestinal malformations: a retro- and prospective study on 203 patients. Eur J Pediatr Surg1994; 4: 279–286.
36.
SwaminathanMOronAPChatterjeeS, et al.Intestinal neuronal dysplasia-like submucosal ganglion cell hyperplasia at the proximal margins of Hirschsprung disease resections. Pediatr Dev Pathol2015; 18: 466–476.
37.
ThakerAIKapurRP. Colonic adventitial fibromuscular dysplasia: a nonspecific arteriopathy associated with Hirschsprung disease and other obstructive disorders. Pediatr Dev Pathol2018; 21: 363–370.
38.
TaguchiTSuitaSHirataYHiroseRYamadaTToyoharaT. Abnormally shaped arteries in the intestine of children with Hirschsprung’s disease: etiological considerations related to ischemic theory. J Pediatr Gastroenterol Nutr1994; 18: 200–204.
39.
YamatakaAOhshiroKKobayashiHFujiwaraTSunagawaMMiyanoT. Intestinal pacemaker C-KIT+ cells and synapses in allied Hirschsprung’s disorders. J Pediatr Surg1997; 32: 1069–1074.
40.
TaguchiTSuitaSMasumotoKNagasakiA. An abnormal distribution of C-kit positive cells in the normoganglionic segment can predict a poor clinical outcome in patients with Hirschsprung’s disease. Eur J Pediatr Surg2005; 15: 153–158.
41.
RolleUPiotrowskaAPNemethLPuriP. Altered distribution of interstitial cells of Cajal in Hirschsprung disease. Arch Pathol Lab Med2002; 126: 928–933.
42.
ChenZHZhangYCJiangWF, et al.Characterization of interstitial Cajal progenitors cells and their changes in Hirschsprung’s disease. PLoS One2014; 9: e86100.
43.
NewmanCJLauriniRNLesbrosYReinbergOMeyratBJ. Interstitial cells of Cajal are normally distributed in both ganglionated and aganglionic bowel in Hirschsprung’s disease. Pediatr Surg Int2003; 19: 662–668.
44.
KapurRPReyes-MugicaM. Intestinal neuronal dysplasia type B: an updated review of a problematic diagnosis. Arch Pathol Lab Med2019; 143: 235–243.
45.
MeyratBJLesbrosYLauriniRN. Assessment of the colon innervation with serial biopsies above the aganglionic zone before the pull-through procedure in Hirschsprung’s disease. Pediatr Surg Int2001; 17: 129–135.
46.
MeyratBJLauriniRN. Plasticity of the enteric nervous system in patients with intestinal neuronal dysplasia associated with Hirschsprung’s disease: a report of three patients. Pediatr Surg Int2003; 19: 715–720.
47.
O'DonnellAMCoyleDPuriP. Decreased expression of hyperpolarisation-activated cyclic nucleotide-gated channel 3 in Hirschsprung’s disease. World J Gastroenterol2015; 21: 5635–5640.
O' DonnellANakamuraHPuriP. Altered ryanodine receptor gene expression in Hirschsprung’s disease. Pediatr Surg Int2019; 35: 923–927.
50.
O'DonnellAMNakamuraHTomuschatCMarayatiNFPuriP. Altered expression of KCNG3 and KCNG4 in Hirschsprung’s disease. Pediatr Surg Int2019; 35: 193–197.
51.
O'DonnellAMNakamuraHTomuschatCMarayatiNFPuriP. Abnormal Scn1b and Fxyd1 gene expression in the pulled-through ganglionic colon may influence functional outcome in patients with Hirschsprung’s disease. Pediatr Surg Int2019; 35: 9–14.
52.
O'DonnellAMCoyleDPuriP. Decreased expression of Kv7 channels in Hirchsprung’s disease. J Pediatr Surg2017; 52: 1177–1181.
53.
TomuschatCO'DonnellAMCoyleDDreherNKellyDPuriP. Altered expression of a two-pore domain (K2P) mechano-gated potassium channel TREK-1 in Hirschsprung’s disease. Pediatr Res2016; 80: 729–733.
54.
TomuschatCO'DonnellAMCoyleDDreherNKellyDPuriP. Altered expression of ATP-sensitive K(+) channels in Hirschsprung’s disease. J Pediatr Surg2016; 51: 948–952.
VerasLVArnoldMAvansinoJR, et al.Guidelines for synoptic reporting of surgery and pathology in Hirschsprung disease. J Pediatr Surg2019; 21: 21.
58.
KnowlesCHDe GiorgioRKapurRP, et al.Gastrointestinal neuromuscular pathology: guidelines for histological techniques and reporting on behalf of the Gastro 2009 International Working Group. Acta Neuropathol2009; 118: 271–301.
59.
DoodnathRPuriP. A systematic review and meta-analysis of Hirschsprung’s disease presenting after childhood. Pediatr Surg Int2010; 26: 1107–1110.
60.
HackamDJReblockKKRedlingerREBarksdaleEMJr. Diagnosis and outcome of Hirschsprung’s disease: does age really matter?Pediatr Surg Int2004; 20: 319–322.
MaerzheuserSBassirCRotheK. Hirschsprung disease in the older child: diagnostic strategies. Clin Pediatr2012; 51: 1087–1090.
63.
VorobyovGIAchkasovSIBiryukovOM. Clinical features’ diagnostics and treatment of Hirschsprung’s disease in adults. Colorectal Dis2010; 12: 1242–1248.
64.
StensrudKJEmblemRBjornlandK. Late diagnosis of Hirschsprung disease – patient characteristics and results. J Pediatr Surg2012; 47: 1874–1879.
65.
EkenzeSONgaikediCObasiAA. Problems and outcome of Hirschsprung’s disease presenting after 1 year of age in a developing country. World J Surg2011; 35: 22–26.
66.
ChungPHYYuMONWongKKYTamPKH. Risk factors for the development of post-operative enterocolitis in short segment Hirschsprung’s disease. Pediatr Surg Int2019; 35: 187–191.
67.
DasguptaRLangerJC. Evaluation and management of persistent problems after surgery for Hirschsprung disease in a child. J Pediatr Gastroenterol Nutr2008; 46: 13–19.
68.
KimHYOhJT. Stabilization period after 1-stage transanal endorectal pull-through operation for Hirschsprung disease. J Pediatr Surg2009; 44: 1799–1804.
69.
SohHJNatarajaRMPacilliM. Prevention and management of recurrent postoperative Hirschsprung’s disease obstructive symptoms and enterocolitis: systematic review and meta-analysis. J Pediatr Surg2018; 53: 2423–2429.
70.
LangerJCRollinsMDLevittM, et al.Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int2017; 33: 523–526.
71.
GosainAFrykmanPKCowlesRA, et al.Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int2017; 33: 517–521.
LevittMADickieBPenaA. Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure. Semin Pediatr Surg2010; 19: 146–153.
MenezesMCorballyMPuriP. Long-term results of bowel function after treatment for Hirschsprung’s disease: a 29-year review. Pediatr Surg Int2006; 22: 987–990.
80.
SoodSLimRCollinsL, et al.The long-term quality of life outcomes in adolescents with Hirschsprung disease. J Pediatr Surg2018; 53: 2430–2434.
81.
KyrklundKNeuvonenMIPakarinenMPRintalaRJ. Social morbidity in relation to bowel functional outcomes and quality of life in anorectal malformations and Hirschsprung’s disease. Eur J Pediatr Surg2018; 28: 522–528.
82.
CollinsLCollisBTrajanovskaM, et al.Quality of life outcomes in children with Hirschsprung disease. J Pediatr Surg2017; 52: 2006–2010.
83.
TannuriACFerreiraMAMathiasALTannuriU. Long-term results of the Duhamel technique are superior to those of the transanal pullthrough: a study of fecal continence and quality of life. J Pediatr Surg2017; 52: 449–453.
84.
MillsJLKonkinDEMilnerRPennerJGLangerMWebberEM. Long-term bowel function and quality of life in children with Hirschsprung’s disease. J Pediatr Surg2008; 43: 899–905.
