Mistakes in... 2024 - 24

ueg education
Mistakes in... 2024
Visceral myopathies
Mitochondrial cytopathies
Congenital
Visceral neuropathies
Abnormalities in myenteric
plexus development
Mesenchymopathies
Multisystemic smooth muscle dysfunction syndrome (ACTA2 gene), visceral myopathy 1 (ACTG2 gene), X-linked
intestinal pseudo-obstruction (L1CAM, FLNA genes), megacystis-microcolon-intestinal hypoperistalsis
syndrome (MYH11, MYL9, ACTG2, MYLK genes)
Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (mitochondrial encephalomyopathy
with lactic acidosis and stroke-like episodes MELAS ) (MT-TL1, MT-ND5 genes), myoclonic epilepsy with
ragged red fibres (myoclonic epilepsy with ragged red fibres MERRF ) (MTTK gene), mitochondrial
neurogastrointestinal encephalopathy (MNGIE ) (TYMP, LIG3, POLG genes)
Chronic atrial and intestinal dysrhythmia (SG1 gene), familial visceral neuropathy (ERBB3, ERBB2 genes),
neuropathic pseudo-obstruction (SOX10 gene)
Aganglionosis, Neural dysplasia
Abnormal Interstitial Cells of Cajal development, depletion or total loss
Central nervous involvement
Neurological
Medullar involvement
Autonomic systemic dysfunction
Neuromuscular
Endocrine
Post-infectious
Acquired
Immune-mediated
Connective tissue diseases
Inflammatory
Antibody-mediated CIPO
Paraneoplastic syndromes
Medication related
Miscellaneous
Idiopathic
Table 1 | Causes of CIPO.
particularly abdominal sepsis and catheter-related
infections in patients receiving total parenteral
nutrition, are recognised triggers for acute
exacerbations that can worsen dysmotility
symptoms. Additionally, adverse effects of
medications, such as opioids and anticholinergics,
should be considered, and any identified culprit
medication should be discontinued. For pain
management, it is important to avoid opioids
and prioritise other analgesic options. In chronic
opioid users, peripherally acting opioid
receptor antagonists (PAMORAs), which block
gastrointestinal opioid receptors while preserving
analgesic effects, can be considered.
Furthermore, electrolyte disturbances
and compromised kidney function, primarily
attributed to factors like vomiting, dehydration,
or third-space fluid sequestration, are frequently
encountered and contribute to the perpetuation
of abnormal gut motility.
Another frequent complication in patients
with CIPO is small intestinal bacterial overgrowth
(SIBO).27
symptoms such as flatulence, chronic diarrhoea,
14
and steatorrhoea; deficiencies in fat-soluble
vitamins and vitamin B12; or increased folate
levels. The treatment of SIBO in patients with
small bowel motility disorders involves two main
strategies: enhancing gastrointestinal propulsive
activity with prokinetic agents and reducing
Cerebrovascular accident, central nervous system
tumours, encephalitis
Post-trauma, vascular accidents
Diabetes mellitus, amyloidosis, multiple system atrophy,
Parkinson's disease, Shy-Drager syndrome
Myotonic dystrophy (Steinert), muscular dystrophy, myasthenia gravis
Hypothyroidism, MEN2b, hyperparathyroidism, hyperthyroidism
Epstein-Barr virus, JC virus, varicella-zoster virus, Chagas disease, herpes simplex virus, cytomegalovirus
Autoimmune disease
Systemic lupus erythematosus, dermatomyositis,
polymyositis, Sjögren syndrome
Systemic sclerosis, mixed-connective tissue disease
Autoimmune myositis or ganglionitis, lymphocytic
ganglionitis, eosinophilic ganglionitis
ANNA-1 or anti Hu, anti-VGKC, anti-CRMP-5/anti-CV2, anti
gAChR, Guillain-Barre syndrome
Small cell lung cancer, carcinoid, thymoma, prostate cancer (anti-Hu antibodies)
Opioids and narcotics, anti-cholinergics, anti-psychotics
Celiac disease, sarcoidosis, cystic fibrosis, intestinal ischemia, porphyria, Fabry disease, anorexia nervosa,
radiation enteritis
Circular muscular layer
Myenteric plexus
Longitude muscle layer
This should be suspected in patients with
Figure 2 | Full-thickness jejunal biopsy of a patient with an autoimmune form of CIPO associated to myasthenia
gravis and achalasia. The histological image shows a lymphocytic CD3+ infiltrate in the myenteric plexus
between the circular and longitudinal muscle layers of the small bowel (myenteric ganglionitis). Image
courtesy of Dr. S. Landolfi, Pathology Department, Vall d'Hebron University Hospital.
Mistakes in... 2024

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