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Majority of patients with rare episodes: reassurance and explanation of symptom complex.
Patients with frequent symptoms:
uncertain benefit from:
– Nitroglycerine: interrupting acute episode?
– Calcium antagonists?
– Inhalation of salbutamol (
-adrenergic agonist): shortening of 20 minutes episodes of proctalgia.
– Clonidine (
-agonist).
– Acupuncture?
Operative Treatment
INDICATION
Surgery not indicated.
OPERATIVE APPROACH
Not applicable.
Outcome
Generally benign and self-limited.
Follow-Up
Recheck patients as needed.
Cross-Reference
Constipation (Ch. 1)
Pain, perirectal (Ch. 1)
Anophysiology studies (Ch. 2)
Defecating proctogram (defecography) (Ch. 2)
Magnetic resonance imaging (MRI) (Ch. 2)
Anal fissure (565.0) (Ch. 4)
Functional disorders—chronic constipation (564.0X) (Ch. 4)
Functional anorectal pain—levator ani syndrome (569.42) (Ch. 4)
"INCIDENTALOLOGY"—APPROACH TO INCIDENTAL FINDINGS
Overview
More sophisticated imaging tools and systematic intraoperative evaluation will invariably reveal unexpected findings and
pathologies that are (1) unrelated to the primary disease, (2) not consistent with, or (3) exceed the preoperative working
hypothesis and treatment plan. While anticipated combinations of separate surgeries may be very rational, particularly in
symptomatic patients, the decision to perform a potentially harmful extension of the primary procedure should be based on
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scientific evidence.
Classification
Related to primary disease worse than expected findings.
Unrelated to primary disease findings without immediate pathologic value.
Unrelated to primary disease findings with current or potential future pathologic impact.
Common Problems/Dilemmas
Possible extension or change of the surgery not previously discussed with patient, lack of informed consent decision
"in the patient's best interest."
Evidence about natural course potentially not available "best guess" decision.
Unnecessary prolongation of the procedure.
Risk of possible additional complications to be considered.
Final pathology might be at variance with intraoperative assessment: eg, tumor despite adherence not invading
another organ, etc.
Specific Scenarios
NORMAL APPENDIX
Question: incidental appendectomy yes/no?
Natural course without procedure: 6–10% lifetime risk of acute appendicitis diagnosis remains difficult, access to
appendix potentially more difficult after previous major abdominal surgery, morbidity not negligible.
Attributable risk of complications: minimal (leak).
Practical approach:
– Appendectomy—yes: absence of contraindications, appendix after primary procedure in different location or
difficult to access. In addition: appendectomy of abnormal-appearing appendix.
– Appendectomy—no: otherwise clean operation with implant (eg, rectopexy), malnourished patient, poor
intestinal condition (LBO, diffuse peritonitis), major extra effort to mobilize appendix.
MECKEL DIVERTICULUM
Question: incidental Meckel diverticulectomy yes/no?
Natural course without procedure: 2% prevalence 6–7% overall lifetime risk of complications: pain, bleeding,
obstruction (intussusception, volvulus, fibrous band), morbidity/mortality of symptomatic Meckel diverticulum: 2%
mortality, > 10% morbidity.
Attributable risk of complications: risk < 2% (leak, obstruction).
Practical approach:
– Resection—yes: absence of contraindications, diverticulum > 2 cm, presence of fibrous band.
– Resection—no: otherwise clean operation with implant (eg, rectopexy), malnourished patient, poor intestinal
condition (LBO, diffuse peritonitis).
CHOLELITHIASIS
Question: incidental cholecystectomy yes/no?
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Natural course without procedure: 10–25% of gallstone carriers become symptomatic.
Attributable risk of complications: < 3%.
Practical approach:
– Cholecystectomy—yes: symptomatic patient, gallbladder with signs of chronic or recurrent inflammation
(despite lack of history), no disproportionate extension of incision—eg, RUQ intervention (eg, right
hemicolectomy), laparoscopic procedure.
– Cholecystectomy—no: if extension of incision needed only for this purpose, asymptomatic patient with
absence of any inflammatory changes.
ADRENAL MASS
Question: workup needed? Adrenalectomy yes/no?
Natural course without procedure: depending on nature (endocrine active/inactive adenoma, metastasis,
pheochromocytoma, hyperplasia, etc).
Attributable risk of complications: endocrine crisis if inadequate perioperative priming.
Practical approach:
– Workup needed: < 3 cm
monitoring, 3–6 cm endocrine evaluation, > 6 cm evaluation and resection.
– Adrenalectomy—yes: only if endocrine workup done, anesthesia management adjusted.
NORMAL-APPEARING OVARIES
Question: ovariectomy in postmenopausal women undergoing cancer operation?
Natural course without procedure: 2–3% lifetime risk of ovarian cancer, 3–4% ovarian metastases from colorectal
cancer (CRC), poor prognosis if CRC involving the ovaries (stage IV).
Attributable risk of complications: ureteral injury 0.1–0.2%.
Practical approach: theoretical benefit of prophylactic oophorectomy questionable incidental adnexectomy
increasingly abandoned by colorectal surgeons as it is more likely to avoid GYN malignancy than improve CRC survival.
HERNIA
Question: hernia repair yes/no? With or without mesh?
Natural course without procedure: 5% lifetime risk of incarceration, enlargement, worse outcome for emergency
procedure.
Attributable risk of complications: infection of foreign body, recurrent hernia.
Practical approach: additional incisions or extension of incision not recommended for just that purpose; repair with
mesh not recommended in contaminated field
reinforcement with biologic material (collagen, etc).
TUMOR INVOLVING OTHER ORGANS
Question: more extensive resection? Ostomy? Abort procedure?
Situations: rectal cancer invading the prostate pelvic exenteration?; involvement of GYN organs hysterectomy,
adnexectomy?; involvement of ureter/kidney
nephrectomy yes/no? carcinomatosis bypass/ostomy?, etc.
Natural course without procedure: incomplete resection clinical recurrence (adjuvant therapy does not make up for
insufficient operation.
Attributable risk of complications: depending on the type of operation.
Practical approach:
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– Proceed: if more extensive surgery likely to improve chance of cure and not resulting in measurable impact on
quality of life: eg, nephrectomy, adnexectomy, hysterectomy.
– Abort and discuss: if more extensive surgery associated with significantly increased morbidity/mortality or a
potentially altered quality of life (eg, pelvic exenteration).
– Ostomy: yes if needed
routine preoperative discussion and marking avoids that conflict.
CANCER INSTEAD OF EXPECTED DIVERTICULITIS
Question: change in surgical approach yes/no?
No because resection for diverticulitis should anyway follow oncological principles.
SIGMOID DIVERTICULITIS INSTEAD OF EXPECTED APPENDICITIS
Question: definite surgical treatment for diverticulitis yes/no?
Natural course without procedure: largely outdated data, 16–20% chance of recurrent attacks, up to 50% after
complicated attack.
Attributable risk of complications: anastomotic leak, recurrent diverticulitis, need for ostomy.
Practical approach: if preoperative symptoms so significant that no time for imaging studies definitive treatment
with surgical resection favored (primary anastomosis with/without colonic lavage); potential alternative: lavage and
drainage alone, followed by antibiotic treatment?
RIGHT-SIDED DIVERTICULITIS INSTEAD OF EXPECTED APPENDICITIS
Question: Ileocecal resection/right hemicolectomy yes/no?
Natural course without procedure: overwhelming majority of cases responding to antibiotic treatment, low risk of
recurrence.
Attributable risk of complications: anastomotic leak, SBO.
Practical approach: if preoperative symptoms so significant that no time for imaging studies definitive treatment
with surgical resection favored (primary anastomosis).
IBD INSTEAD OF EXPECTED APPENDICITIS
Question: appendectomy yes/no? Ileocecal resection yes/no?
Natural course without procedure: nearly 100% recurrent disease and risk of future surgical intervention; only 50%
risk after ileocecal resection (in addition, benefit of having histologic confirmation).
Attributable risk of complications: leak, recurrent disease, short bowel syndrome.
Practical approach:
– Ileocecal resection—yes: if disease limited to ileocecal region.
– Ileocecal resection—no: if multisegmental involvement.
Appendectomy—yes: if access through McBurney incision.
Appendectomy—no: if access through midline and appendix normal.
TUMOR INSTEAD OF EXPECTED APPENDICITIS
Question: Oncologic resection (right hemicolectomy) yes/no?
Natural course without extension of procedure (ie, appendectomy only): risk of lymph node metastases, increased
risk of tumor recurrence, need for subsequent surgery for oncological resection.
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Attributable risk of complications: anastomotic leak, SBO, no relevant impact on bowel function.
Practical approach: clinical evaluation of intraoperative findings and immediate pathologic assessment of specimen
(including frozen section if available): if malignancy confirmed or very likely
definitive treatment with surgical
resection favored (primary anastomosis).
Cross-Reference
Pain, abdominal (Ch. 1)
Computed tomography (CT scan) (Ch. 2)
CT colonography ("virtual colonoscopy") (Ch. 4)
Magnetic resonance imaging (MRI) (Ch. 2)
Colorectal cancer—colon cancer (153.X) (Ch. 4)
IBD—Crohn disease (555.X) (Ch. 4)
Diverticular disease (diverticulosis, diverticulitis, 562.1X) (Ch. 4)
PEDIATRICS—CONGENITAL AGANGLIONIC MEGACOLON
(HIRSCHSPRUNG DISEASE, 751.3)
Overview
Anorectal malformation with initially normal macroanatomy that results from incomplete population of the distal colon with
neuronal elements. Development of enteric nervous system: migration of neuroenteric cells from neural crest to the GI tract
from proximal to distal, first to myenteric (Auerbach) plexus, then to submucosal (Meissner) plexus.
Hirschsprung disease: incomplete migration results in a lack of neuroenteric network in the distal rectum and extending in
continuity toward proximal rectum for varying lengths
lack of peristalsis and inability to relax muscle tone in aganglionic
segment (including internal anal sphincter) + increased distal smooth muscle tone and spasticity constipation and
increasing dilation of colon proximal to aganglionic segment necrotizing enterocolitis (acute) or megacolon (chronic).
Severity of symptoms and untreated natural course with some variability. Increased incidence of other anomalies (Down
syndrome, intestinal atresia).
Treatment consists of resecting the pathologic aganglionic distal segment with respective reconstruction. Decision-making
process includes assessment of whether:
Untreated condition is life-threatening (eg, acute necrotizing enterocolitis, perforation)?
Other life-threatening anomalies that need priority attention are present (eg, cardiac)?
Primary reconstruction (with/without defunctioning colostomy) or secondary reconstruction (bridging with colostomy)
is needed?
Epidemiology
Incidence: 1 in 4400 to 1 in 7000 live births with male/female ratio of 4:1 (subgroup long-segment: 1:1). Incidence of
familial cases: 6–10%.
Associated anomalies: Down syndrome 4–16%, anal/intestinal atresia 0.5–3.5%.
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In adolescents and adults: variable incidence (3–20%).
Symptoms
Neonates (80–90%): symptomatic during first 24–72 hours of life delayed passage of meconium > 24–48 hours,
constipation, abdominal distention, poor feeding, emesis, failure to thrive. Complications: Hirschsprung-associated
enterocolitis, cecal perforation. Variant: relatively asymptomatic.
After newborn period: history of severe constipation, cycles of constipation/distention with breakthrough episodes of
explosive diarrhea, abdominal distention (chronic), enterocolitis (acute distention), failure to thrive. Variant: relatively
asymptomatic.
Adulthood (3–20%): history of chronic constipation since childhood, abdominal distention.
Differential Diagnosis
Meconium plug.
Cystic fibrosis (meconium ileus).
Morphologic obstruction (atresia, anorectal malformations; caveat: possible combination of both).
Intestinal neuronal dysplasia: on biopsy neural hyperplasia and increased number of ganglia.
Total intestinal aganglionosis: entire GI tract involved.
Small left colon syndrome (transient dysmotility in children of diabetic mothers).
Endocrine imbalance: hypothyroidism, adrenal insufficiency.
Adulthood: Chagas disease.
Other causes of constipation.
Pathology
MACROSCOPIC
Depending on duration of symptoms: varying degrees of preaganglionic colonic distention.
Acute complication of enterocolitis.
MICROSCOPIC
Normal: paucity of ganglia within 1.5–2 cm proximal to dentate line.
Hirschsprung: absence of ganglion cells in submucosal and myenteric plexus of distal large intestine > 2 cm from
dentate line, possible neural hyperplasia (adrenergic, cholinergic), otherwise normal colonic architecture.
Evaluation
REQUIRED MINIMAL STANDARD
Diagnosis of Hirschsprung disease:
Abdominal x-ray: megacolon? Small bowel dilation?
Anorectal manometry (best to diagnose short-segment Hirschsprung, of limited practical use in infants and children):
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absence of RAIR (reflex relaxation with rectal distention).
Barium enema: spastic distal segment with characteristic transition zone to dilated proximal bowel, retention of
barium at 24 hours? May be false negative in short-segment Hirschsprung.
Biopsy: diagnostic gold standard (accuracy 99%): serial biopsies that include a thickness of submucosa equal to the
mucosa, starting 2 cm proximal to dentate line:
no submucosal plexus, but increased acetyl cholinesterase stain.
(caveat: last 1.5–2 cm do not contain ganglia even in normal patients!)
ADDITIONAL TESTS (OPTIONAL)
Evaluation of associated anomalies.
Classification
Classical form of Hirschsprung (80–90%): < 40 cm of distal colon affected.
Long-segment Hirschsprung (7–10%): aganglionic segment extending proximally to hepatic flexure, or even total
colonic aganglionosis, ie, complete involvement of colon.
Ultrashort-segment Hirschsprung: only distal rectum involved.
Nonoperative Treatment
Colonic decompression, rectal irrigation through large-diameter tube: bridging to surgery.
Operative Treatment
INDICATION
Neonatal/childhood period: any confirmed Hirschsprung disease (unless in combination with lethal anomalies).
Adults: depending on presentation and failure of conservative management.
OPERATIVE APPROACH
Poor condition: colostomy, secondary reconstruction later.
Good condition: primary reconstruction with/without diversion:
1. Modern approach: one-stage pull-through procedure normoganglionic bowel (frozen sections):
a. Laparoscopic-assisted endorectal pull-through.
b. Transanal endorectal pull-through in the newborn.
2. Classical operations: Rehbein, Swenson, Duhamel, Soave.
Massive megacolon: colonic contractility impairment persisting even after resection of aganglionic segment (decompensated
Frank-Starling mechanism): proctocolectomy and ileoanal pull-through procedure.
Outcome
Mortality: 1–2%.
Anastomotic leak: 5–7%.
Postoperative enterocolitis: 6–20%.
Long-term good bowel function: 80–90%.
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Other complications: stricture, perirectal fistula, incontinence, persistent constipation (particularly after Duhamel and Soave
procedures).
Follow Up
Functional reassessment in regular intervals.
Cross-Reference
Constipation (Ch. 1)
Megacolon (Ch. 1)
Pain, abdominal (Ch. 1)
Anophysiology studies (Ch. 2)
Defecating proctogram (defecography) (Ch. 2)
Embryologic colorectal development (Ch. 3)
Toxic megacolon (556.9) (Ch. 4)
Large bowel obstruction (LBO, 560.0X) (Ch. 4)
Functional disorders—chronic constipation (564.0X) (Ch. 4)
Pediatrics—congenital malformations (751.2) (Ch. 4)
PEDIATRICS—CONGENITAL MALFORMATIONS (751.2)
Overview
Anorectal malformations comprise a large number of macromorphologic anatomic anomalies that result from incomplete or
incorrect embryologic development and fusion steps and manifest as abnormal number and form of orifices.
Timing of the genetic program errors and stops results in different levels and severities of anatomic defects: high (severe),
intermediate (moderate), low (mild). Prognosis depends on (1) severity of the anorectal malformation and (2) impact of other
anomalies.
Frequent association with chromosomal and other morphologic defects (cardiac, urogenital system, skeleton, etc).
For treatment planning
assessment whether:
Untreated condition is life-threatening (eg, complete atresia without fistula)?
Other life-threatening anomalies that need priority attention are present (eg, cardiac)?
Primary reconstruction (with/without defunctioning colostomy) or a secondary reconstruction (bridging with
colostomy) is needed?
Epidemiology
Incidence: 1 in 3500 to 1 in 5000 births with male preponderance (55–65%).
Associated malformations (in 60–65%):
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Chromosomal abnormalities (15%).
VACTERL (10–15%): Vertebral anomalies, Anal atresia, Cardiac defect (eg, ventricular septum defect), Tracheo-
Esophageal fistula, Renal abnormalities, Limb abnormalities.
Cardiac: 15–30% (Fallot tetralogy, ventricular septal defect).
GI: tracheoesophageal fistula 8–10%, Hirschsprung disease 3%, duodenal atresia 2%.
Urogenital: vesicoureteral reflux 60%, renal agenesis/malformation.
Vertebral: spinal dysraphism 15–35% (eg, tethered cord).
Symptoms
Asymptomatic unless not recognized and treated in time obstructive symptoms, urinary tract infections.
Differential Diagnosis
Only with regards to different variants and associated morphologic and genetic defects.
Pathology
Macropathology of the various subforms of malformations (see classification).
Evaluation
REQUIRED MINIMAL STANDARD
Visible abnormality on first comprehensive examination at birth:
Flat perineum, short sacrum, little muscle contraction high anomaly.
Females: single perineal opening cloacal malformation.
After 24 hours
gas and meconium moving toward rectum:
Males urinalysis for meconium; females meconium through vagina.
Gas or meconium visible in perineal raphe low anomaly.
Lateral pelvic radiograph ("babygram") in upside-down position (with marking of perineum/anus): distance of defect?
Ultrasound/MRI: assessment of pelvic floor anatomy.
ADDITIONAL TESTS (OPTIONAL)
Depending on conclusiveness of the presentation.
Search for associated anomalies: ultrasound
hydronephrosis? skeletal x-rays, etc.
Classification
Type of defect:
Imperforate anus/rectal atresia/anal stenosis with/without fistula.
Fistula (to perineum, urinary system, vestibulum/vagina).
Cloaca.
Level of defect:
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High lesions (supralevator): 25%.
Intermediate lesions: 30–35%.
Low lesions (infralevator): 40–45%.
More detailed classifications: Wingspread classification, Pena classification.
Nonoperative Treatment
Temporarily only at initial presentation: NPO, insertion of NGT.
Long-term problems: management of incontinence, bowel regimen, etc.
Operative Treatment
INDICATION
Any anorectal malformation.
OPERATIVE APPROACH
Temporizing Measures
Colostomy in double-barrel technique: complete diversion, reduced risk of urinary tract infection, access to mucus fistula,
no risk of closed loop.
Reconstruction (Primary or Secondary)
Posterior sagittal anorectoplasty (PSARP): preferred approach for majority of patients: prone-jackknife position,
electrical stimulation device for evaluation/localizing of sphincter contraction:
– Low anomalies: anoplasty or minimal PSARP without colostomy.
– High and intermediate anomalies: colostomy
PSARP at 4–8 weeks.
Abdominoperineal pull-through procedure: not needed in majority of patients.
Reoperations for Persistent Dysfunction (Eg, Incontinence)
Muscle good, but bowel previously misplaced: relocation to correct site.
Absent muscle function: artificial bowel sphincter, graciloplasty, MACE procedure. Permanent colostomy usually not
needed unless patient miserable and above-mentioned local reconstructive efforts fail.
Outcome
Fecal continence: low anomalies 85–90%, high anomalies 30–40%. Manifest fecal incontinence: 25–40%.
Urinary incontinence: 2–18% (except cloaca
up to 60–70%).
Other complications: anal stenosis 30%, rectal mucosal prolapse, constipation 25% (more frequent in low anomalies with
megarectum)
overflow incontinence.
Follow-Up
Functional reassessment at regular intervals.