Anterograde Continence Enema (ACE) Procedure
Answer: A
This woman has had a life-long struggle with complications of spina bifida, the most common neurologic congenital defect worldwide. It presents with variable degrees of spinal cord dysfunction, neurogenic bowel and/or bladder, and orthopedic and cerebellar sequelae.
Neurogenic bowel and bladder problems are common in patients with spina bifida. In the past, surgeries for urinary incontinence left patients still using diapers due to fecal soiling, or having to resort to daily enemas or local manipulation in order to defecate. These patients would often have to depend on a family member or caregiver to assist, greatly limiting independence.
Thirty years ago, Dr Patrick Malone popularized the anterograde continence enema (ACE) procedure. This involves using the appendix as a conduit for external lavage: after bowel preparation, the appendix is isolated and brought up to anastamosis with the umbilicus or right lower quadrant in a stoma. The base of the appendix is imbricated much like a Nissen fundoplication, to avoid reflux of stool. When healed, the patient can catheterize the stoma and irrigate the bowel in an anterograde fashion:
This woman has had a life-long struggle with complications of spina bifida, the most common neurologic congenital defect worldwide. It presents with variable degrees of spinal cord dysfunction, neurogenic bowel and/or bladder, and orthopedic and cerebellar sequelae.
Neurogenic bowel and bladder problems are common in patients with spina bifida. In the past, surgeries for urinary incontinence left patients still using diapers due to fecal soiling, or having to resort to daily enemas or local manipulation in order to defecate. These patients would often have to depend on a family member or caregiver to assist, greatly limiting independence.
Thirty years ago, Dr Patrick Malone popularized the anterograde continence enema (ACE) procedure. This involves using the appendix as a conduit for external lavage: after bowel preparation, the appendix is isolated and brought up to anastamosis with the umbilicus or right lower quadrant in a stoma. The base of the appendix is imbricated much like a Nissen fundoplication, to avoid reflux of stool. When healed, the patient can catheterize the stoma and irrigate the bowel in an anterograde fashion:
Our patient’s right stoma is her Malone ACE. Her umbilical stoma is a separate conduit to the urinary bladder, created using a technique called the Monti procedure, which assists in evacuation of the urinary bladder.
At home, patients will often use water or home-made saline solution to irrigate and gently stimulate peristalsis: a lubricated catheter is placed in the ACE stoma, and a large syringe or bag is used to infuse the liquid slowly.
Prolonged constipation in this patient may be problematic, as she may not sense sequelae until later, such as fecal impaction or stercal ulcers (C). Good bowel care should be initiated in this patient as soon as possible (A). A simple irrigation in the ED and reexamination will allow the physician and patient to be reassured that the patient will continue to do well at home.
Patients with any spinal cord injury and a potentially painful stimulus (constipation, urinary retention, trauma, etc) should be screened for developing autonomic dysreflexia. The patient may be insensate and the painful stimulus below the level of the lesion does not register to him or her; however, the strong sensory input evokes a reflex sympathetic surge, potentially causing a hypertensive crisis. Early symptoms are piloerection, restlessness, flushed face, and diaphoresis. The patient may have headache, nausea, bradycardia, and hypertension. Sit the patient up, remove constrictive clothing, and find and remove or treat the source of the stimulus. If this does not resolve the hypertension, treat like any hypertensive urgency/emergency (in this case, nifidepine and/or nitrates may be used).
Polyethylene glycol (Miralax) is an osmotic agent used to increase the water content of stool. Although she may take this as part of her bowel regimen, the maximum recommended dose for adults is fixed at 17 grams. Doubling her dose is unlikely to assist in bowel movement (B), and will most likely cause bloating.
Prescribing a gentle stimulant laxative such as senna (Sennakot) may be an adjunct to her bowel care. However, its use is probably best in the routine prevention of chronic constipation, rather than in the treatment of obstipation. Stimulating obstipated bowel may only serve to precipitate autonomic dysreflexia without resolving the obstipation (D).
Reference
Krassioukov A, Eng JJ, Claxton G, Sakakibara BM, Shum S. Neurogenic bowel management after spinal cord injury: a systematic review of the evidence. Spinal Cord. 2010 Oct;48(10):718-33. doi: 10.1038/sc.2010.14. Epub 2010 Mar 9.