Double check: diagnosis and management of adult Hirschsprung disease

Hirschsprung disease (HD) is a gut motility disorder usually diagnosed acutely in infancy, although variants of HD may present later in life with indolent symptoms. This report highlights the rarity of diagnosing HD and hypoganglionosis in adulthood and the nuances that need consideration for their surgical management. We present a report of a 49-year-old man presenting with chronic constipation. A full thickness rectal biopsy confirmed aganglionosis, and HD in adulthood was diagnosed. He underwent a defunctioning left-sided colostomy to ensure histological confirmation of ganglia in his left colon, and adequate colonic function via the colostomy.This served also as an assessment of the proximal conduit for any future anastomosis. He later underwent ultra-low anterior resection, coloanal anastomosis and loop ileostomy with subsequent reversal. His final histology revealed hypoganglionosis of the resected segment, with normal innervation to the site of the colostomy. He made full recovery with normal bowel movements.

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Image of the Month: Decision-Making in Surgery for Late Onset Hirschsprung Disease

European Journal of Pediatric Surgery Reports ◽

10.1055/s-0040-1721049 ◽

2020 ◽

Vol 08 (01) ◽

pp. e99-e101

Author(s):

Anisha Apte ◽

Elise McKenna ◽

Marc A. Levitt

Keyword(s):

Decision Making ◽

Chronic Constipation ◽

Anorectal Manometry ◽

Late Onset ◽

Hirschsprung Disease ◽

Rectal Biopsy ◽

Poor Growth

AbstractWe present a case of a 14-year-old boy with chronic distension, poor growth, and chronic constipation. He undergoes anorectal manometry and rectal biopsy, confirming the diagnosis of Hirschsprung disease (HD). The case is presented with a key image and associated questions to prompt discussion on strategies for management and treatment of HD in late-diagnosed children.

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Autopsy Study of Calretinin Immunohistochemistry in the Anorectal Canal in Young Infants and Potential Implications for Rectal Biopsy Approach in the Neonatal Period

Pediatric and Developmental Pathology ◽

10.1177/10935266211030172 ◽

2021 ◽

pp. 109352662110301

Author(s):

Heather Rytting ◽

Zachary J Dureau ◽

Jose Velazquez Vega ◽

Beverly B Rogers ◽

Hong Yin

Keyword(s):

Neonatal Period ◽

Ganglion Cells ◽

Hirschsprung Disease ◽

Rectal Biopsy ◽

Autopsy Study ◽

Pathologic Diagnosis ◽

Young Infants ◽

Autopsy Specimens ◽

Pectinate Line ◽

Age Range

Background Absent submucosal ganglion cells in biopsies 1-3 cm above the pectinate line establishes the pathologic diagnosis of Hirschsprung Disease (HD). Calretinin stains both ganglion cells and their mucosal neurites and has gained importance in HD diagnosis. Absent calretinin positive mucosal neurites in biopsies at the appropriate level above the pectinate line is highly specific for HD. Whether this applies to lower biopsies is uncertain. To address this, we studied anorectal canal autopsy specimens from infants. Methods We performed an autopsy study of infant anorectal canal specimens to describe calretinin staining in this region. Calretinin staining was correlated with histologic and gross landmarks. Results In all 15 non-HD specimens, calretinin positive mucosal neurites were present in glandular mucosa up to the anorectal line where neurites rapidly diminished. Age range was preterm 26 weeks to 3 months. Conclusions Calretinin positive mucosal neurites are present in glandular mucosa up to the anorectal line in young infants. This is potentially important regarding neonatal HD biopsy level and diagnosis. Positive calretinin staining at the anorectal line favors normal innervation making HD unlikely. Absent calretinin positive neurites in glandular mucosa is worrisome for HD in young infants, regardless of location.

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Comparison of the clinical outcomes of skin bridge loop ileostomy and traditional loop ileostomy in patients with low rectal cancer

Scientific Reports ◽

10.1038/s41598-021-88674-x ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Hui Ye ◽

Shujuan Huang ◽

Jie Yu ◽

Qichang Zhou ◽

Changlei Xi ◽

...

Keyword(s):

Rectal Cancer ◽

Low Anterior Resection ◽

Clinical Evidence ◽

Loop Ileostomy ◽

Loop Group ◽

Clinical Results ◽

Low Rectal Cancer ◽

Analog Scale ◽

Traditional Group ◽

Bridge Group

AbstractTo compare the clinical results of patients with low rectal cancer who underwent skin bridge loop ileostomy and traditional loop ileostomy, and provide clinical evidence for choosing a better ostomy method. We retrospectively collected data of 118 patients with rectal cancer who underwent low anterior resection and loop ileostomy. To investigate the patients characteristics, postoperative stoma-related complications and the frequency of exchanged ostomy bags. The differences of these indicators between the two groups of patients who underwent skin bridge loop ileostomy and traditional loop ileostomy were compared. The Visual Analog Scale (VAS) score of the skin bridge loop ileostomy group was lower than that of the traditional ileostomy loop group (P < 0.05). The skin bridge group had a lower Discoloration, Erosion, Tissue overgrowth (DET) score and incidence of mucocutaneous separation than the traditional group at the 1st and 2nd weeks after operation (P < 0.05). The average number of weekly exchanged ostomy bags was significantly less in the skin bridge group than in the traditional group within 4 weeks after surgery (P < 0.05). Our experience demonstrates that the skin bridge loop ileostomy may significantly reduce early postoperative stoma-related complications, the frequency of exchanged ostomy bags and patients’ medical costs after discharge.

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Treatment of Rectal Cancer by Low Anterior Resection with Coloanal Anastomosis

Annals of Surgery ◽

10.1097/00000658-199404000-00007 ◽

1994 ◽

Vol 219 (4) ◽

pp. 365-373 ◽

Cited By ~ 145

Author(s):

Philip B. Paty ◽

Warren E. Enker ◽

Alfred M. Cohen ◽

Gregory Y. Lauwers

Keyword(s):

Rectal Cancer ◽

Anterior Resection ◽

Low Anterior Resection ◽

Coloanal Anastomosis

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The association of age with decline in renal function after low anterior resection and loop ileostomy for rectal cancer: a retrospective cohort prognostic factor study

BMC Geriatrics ◽

10.1186/s12877-020-02001-z ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Amal Rhemouga ◽

Stefan Buettner ◽

Wolf O. Bechstein ◽

Guido Woeste ◽

Teresa Schreckenbach

Keyword(s):

Rectal Cancer ◽

Rectal Carcinoma ◽

Anterior Resection ◽

Low Anterior Resection ◽

Older Patients ◽

Retrospective Cohort ◽

Loop Ileostomy ◽

Linear Regression Analysis ◽

Multivariate Linear Regression Analysis ◽

Group B

Abstract Background Low anterior resection (LAR) is often performed with diverting loop ileostomy (DLI) for anastomotic protection in patients with rectal cancer. We aim to analyze, if older patients are more prone to a decline in kidney function following creation and closure of DLI after LAR for rectal carcinoma versus younger patients. Methods A retrospective cohort study from a database including 151 patients undergoing LAR for rectal carcinoma with DLI was used. Patients were divided in two age groups (Group A: <65 years, n = 79; Group B: ≥65 years, n = 72). For 123 patients undergoing DLI reversal prognostic factors for an impairment of serum creatinine (SCr) and estimated glomerular filtration rate (eGFR) 3 months after DLI reversal was analyzed using a multivariate linear regression analysis. Results SCr before LAR(T0) was significant higher in Group B (P = 0.04). Accordingly, the eGFR at T0 in group B was significantly lower (P < 0.001). No patients need to undergo hemodialysis after LAR or DLI reversal. Age and SCr at T0were able to statistically significant predict an increase in SCr (P<0.001) and eGFR (P=0.001) three months after DLI reversal (The R² for the overall model was .82 (adjusted R² = .68). Conclusion DLI creation may result in a reduction of eGFR in older patients 3 months after DLI closure. Apart from this, patients do not have a higher morbidity after creation and closure of DLI resulting from LAR regardless of their age.

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The impact of timing of stoma closure on low anterior resection syndrome in patient who underwent low anterior resection for rectal cancer

Kurdistan Journal of Applied Research ◽

10.24017/science.2021.1.13 ◽

2021 ◽

pp. 126-134

Author(s):

Hemn Hussain Kaka Ali ◽

Qalandar Hussein Abdulkarim ◽

Karzan Seerwan ◽

Barham M. M .Salih

Keyword(s):

Rectal Cancer ◽

Anterior Resection ◽

Low Anterior Resection ◽

Anal Verge ◽

Loop Ileostomy ◽

Low Anterior Resection Syndrome ◽

Anterior Resection Syndrome ◽

Closure Of Ileostomy ◽

The Mean ◽

The Impact

This is a multi-center retrospective study of patients underwent low anterior resection for rectal cancer. Ileostomy had been done to protect low lying Colo-rectal anastomosis, closure of ileostomy had been delayed in some patients due to patient own will, surgical complications (anastomotic leak) or coarse of chemotherapy. This study aimed to find the effect of temporary ileostomy on post-operative bowel defunction which is called Low anterior resection syndrome (LARS), and include; urgency, difficulty in emptying of bowel, and incontinence for feces and flatus. A total of 50 patients included in this study, the age ranges from the 19 to 80 years old with a mean age of 51.96 years. The total number of males was (33, %66). Majority of patients were overweight (21, 42%). The distance of tumors from the anal verge were less than 10 cm in (31,62%). The mean duration of fecal diversion was 7.17 months. Loop ileostomy were closed before six months in (27,54%). The mean duration of diversion of patients developed no LARS was 6.87 months which is shorter than those of developed LARS (7.31). Lower BMI patients are more prone to develop LARS, while Obese patients are more susceptible to develop major LARS. Nineteen cases developed LARS among those patient’s ileostomy closed before six months, and 15 cases developed LARS in those ileostomies closed after six months.

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Anastomotic Sinus After Low Anterior Resection and Diverting Loop Ileostomy

Colorectal Surgery Consultation ◽

10.1007/978-3-030-11181-6_54 ◽

2019 ◽

pp. 241-243

Author(s):

Daniel L. Feingold ◽

Garrett Friedman

Keyword(s):

Anterior Resection ◽

Low Anterior Resection ◽

Loop Ileostomy ◽

Diverting Loop Ileostomy ◽

Anastomotic Sinus

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Treatment of severe actinic rectitis

Sao Paulo Medical Journal ◽

10.1590/s1516-31801998000100007 ◽

1998 ◽

Vol 116 (1) ◽

pp. 1629-1633

Author(s):

Benedito Mauro Rossi ◽

Wilson Toshihiko Nakagawa ◽

José Augusto P. Fernandes ◽

Ademar Lopes ◽

Lauma Dzidra Paegle

Keyword(s):

Postoperative Complications ◽

Surgical Technique ◽

Anal Canal ◽

Venous Thrombosis ◽

Deep Venous Thrombosis ◽

Coloanal Anastomosis ◽

Left Colon ◽

Sphincter Function ◽

First Case ◽

Absorbable Sutures

BACKGROUND: The authors report the treatment of three female patients with severe actinic rectitis, with stenosis or perforation, submitted to anterior proctosigmoidectomy and transanal coloanal anastomosis. METHODS: In all cases surgery consisted of total proctosigmoidectomy, mucosectomy of the anal canal, lowering of the left colon through the pelvis and transanal anastomosis performed manually at the level of the pectineal line using separate absorbable sutures. A protective intestinal shunt was performed in all cases. RESULTS: The three patients did not present transoperative or immediate postoperative complications, but the first patient developed deep venous thrombosis of the leg that was submitted to successful clinical treatment. The intestinal shunts were later closed in all three cases. Sphincter function was considered very good in the first case and regular in the remaining two. CONCLUSION: The surgical technique utilized was considered to be adequate for the cases reported and is the first option for the maintenance of transit in patients with severe actinic rectitis since the anastomosis is performed using non-irradiated colon with the pectineal line, practically outside the pelvis.

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