With respect to the image depicted below:
- What are the abnormal features and what is the diagnosis?
- List how this condition may impact on ICU management.
b) List the clinical features of acute bowel ischaemia.
Briefly describe the distribution of the arterial blood supply to the large and small bowel.
i. Abnormal features and diagnosis:
Tight, indurated skin with limited mouth opening and beak-like facies Telangiectasia
Scleroderma (or CREST syndrome if other features are present)
ii. Management problems:
- Difficult intubation
- Aspiration risk
- Risk of oesophageal perforation with instrumentation
- Limited respiratory reserve
- Pulmonary hypertension
- Difficulty palpating peripheral pulses
- Difficult vascular access and risk of digital gangrene with arterial lines and vasopressors
- Increased risk of renal failure
- Malabsorption and nutritional deficiencies
- Skin breakdown / pressure areas
- Clinical features of acute bowel ischaemia:
- Abdominal pain
- Abdominal tenderness
- Bloody diarrhoea or haematochezia
- Evidence of ileus (vomiting, large NG aspirates, feed intolerance)
- Absent/reduced bowel sounds
- Briefly describe the distribution of the arterial blood supply to the large and small bowel.
- Extensive collateral circulation
- Coeliac axis supplies the gut down to the opening of the bile duct (mid duodenum).
- SMA supplies the bowel from the entrance of the bile duct (mid duodenum) to a level just short of the splenic flexure of the colon. (to splenic flexure or right two thirds of the transverse colon acceptable)
- IMA supplies the bowel from just short of the splenic flexure of the colon, to the rectum and anus. The rectum and anus also receive blood via branches of the internal iliac artery.
More stolen images; though I expect the watermarks identify them well. The face is from dermis.net, and represents scleroderma. The hands are from the Rheumatology Image Bank; the digits demonstrate features of acrosclerosis, contracture and terminal phalangeal resorption.
Issues with these patients can be discussed in a systematic manner:
- Difficult intubation:
- Limited neck extension
- Limited mouth opening
- Respiratory involvement:
- Pulmonary fibrosis
- Restrictive lung disease
- Pulmonary hypertension
- SpO2 monitoring may be frustrated by poor end-digital perfusion
- The rapidly fatal "scleroderma-pulmonary-renal syndrome (SPRS)" may develop, which manifests as a fulminant course of acute normotensive renal failure associated with diffuse alveolar hemorrhage.
- Cardiovascular involvement of the disease process:
- Cardiac problems:
- Myocardial fibrosis (thus, restrictive diastolic failure)
- Pericardial stricture (also restricts diastolic filling)
- Vascular problems
- Difficult vascular access: the skin, being very thick, makes it difficult to palpate vessels (veins and arteries both)
- Poor distal perfusion of the extremities, leading to gangrene- as one might expect this is not improved by arterial cannulation.
- Poor skin perfusion promotes pressure areas
- Cardiac problems:
- Neurological sequelae:
- Corticosteroid-associated psychosis
- Cerebral vasculitis
- Electrolyte disturbances
- Hyponatremia and fluid retention due to corticosteroid therapy
- Hyperkalemia due to renal failure
- Renal involvement
- Renal failure, renal artery stenosis
- Scleroderma "renal crisis"
- Gastrointestinal and nutritional issues
- Poor gut motility
- Decreased feed tolerance
- Risk of aspiration.
- Risk of oesophageal perforation
- Telangiectasia is present also on mucosal surfaces; there is an increased risk of bleeding from upper GI sites
- Haematological problems:
- Anaemia of chronic disease is coupled with the poor EPO synthesis from damaged kidneys.
- Bone marrow function may be suppressed in other ways, particularly if serious immunosuppresants are in use (eg. cyclophosphamide)
- Immunosuppressive therapy
- Increased infection risk
Clinical features of acute bowel ischaemia:
- Abdominal pain, out of proportion to the signs
- Abdominal distension
- Absent bowel sounds
- Bloody stool
- Metabolic acidosis
- Hemodynamic instability
Blood supply to the bowel
This is easy anatomy to remember; there are only two arteries.
- Extensive collateral supply
- Coeliac axis: up to duodenum
- SMA: duodenum to splenic flexure
- IMA: splenic flexure to anus.
Farber, Harrison W., Robert W. Simms, and Robert Lafyatis. "Analytic Review: Care of Patients With Scleroderma in the Intensive Care Setting." Journal of intensive care medicine 25.5 (2010): 247-258.
Legerton 3rd, C. W., Edwin A. Smith, and Richard M. Silver. "Systemic sclerosis (scleroderma). Clinical management of its major complications."Rheumatic diseases clinics of North America 21.1 (1995): 203-216.
Oldenburg, W. Andrew, et al. "Acute mesenteric ischemia: a clinical review."Archives of internal medicine 164.10 (2004): 1054-1062.
Thorek, Philip. "Blood Supply of the Gut." Anatomy in Surgery. Springer New York, 1985. 558-562.