Perioperative Guidelines for Anesthesia Care for Patients with EB
Airway assessment is critical
• Oral scarring results in limited mouth opening (microstomia)
• Mucosal lesions may be severe
• Dental caries may be present
• Tongue may be fused to floor of mouth
• Teeth may be angled inward
• Esophageal strictures may be very high
• Anticipate difficult intubation, consider oral fiberoptic intubation
Multiple Systems May be involved
Pulmonary: Frequent respiratory infections; Aspiration; Decreased pulmonary function
Cardiac: Dilated Cardiomyopathy in patients with RDEB possibly related to carnitine and/or selenium deficiency). Consider preoperative echocardiogram
Musculoskeletal: Extensive contractures; Digital fusion (mitten deformities); Difficult IV access
Nutrition: Increased caloric demand; Growth failure and failure to thrive; Anemia of iron deficiency and chronic disease
Skin: Extreme fragility; Blisters and erosions; Squamous cell carcinoma; Infection related to
compromised skin integrity; poor immunity due to malnutrition
Common Surgical Procedures
• Plastic surgery to correct pseudosyndactyly of hands, feet
• Balloon esophageal dilation under fluoroscopy
• Skin biopsies to rule out squamous cell carcinoma
• Excisional surgery with grafting for squamous cell carcinoma
• Dental rehabilitation
• Whirlpool treatments for skin debridement
• PEG or open gastrostomy
• GI Endoscopy
• Central lines
• Dressing changes
General Management Principles
o No shearing forces can be applied to skin to minimize bulla formation.
o Compressive forces to the skin are tolerated.
o Lift, do not slide patient during transfer.
o All adhesive tape, ECG leads, adhesive pulse oximeter probes must be avoided.
o If patient dressings are in place and not in the way, leave in place.
o Columnar mucosa of nares, larynx, trachea distal to vocal cords are not affected.
o Tracheal intubation is acceptable.
• Warm room
• Padded OR table
• Gentle transfer of patient.
• Egg crate mattress which stays under patient throughout perioperative period
• Lubricate eyes with preservative-free, non-lanolin ointment (Refresh®)and cover eyes with moistened gauze pads or use non-adherent adhesive (Mepitel® or Mepiform®)
• Assemble all necessary supplies ahead of time.
Suggested Supply Kit:
• Non adhesive dressing: Mepitel®, Mepiform®, Mepitac tape® (Molnlycke Healthcare, Goteburg, Sweden, www.molnlycke.com) or Vaseline gauze/Telfa®
• Coflex® (Andover) wrap or Coban® (3M), gauze
• Cotton tape to secure endotracheal tube (ETT))
• Aquaphor® ointment to lubricate anesthesia mask
• Water based lubricant (i.e. Surgilube® by Fougera) to lubricate oral airways, laryngoscope blade
• Clip pulse oximeter probe
Oral Premedication to minimize struggling for mask induction
Monitoring: Reasonable Minimum
• Gauze padding under BP cuff
• Nonadhesive pulse oximeter probe (clip probe)
• Nonadhesive ECG leads (needle electrodes or normal electrode pads with adhesive cut off and secured with Mepitac tape® or gauze wrap)
• Axillary temperature probe if needed
• Mask induction common for pediatric patients
• Gentle pressure with well-lubricated mask
• IV induction may be difficult due to poor venous access
• Tourniquet placed over gauze padding
• Secure IV with Mepitel or Mepitac
• Wrap with Kling® (J&J), Webril® (Kendall) Coban® (3M) or Coflex® (Andover)
• No adhesives!
• Check IV site frequently since IVs tend to become dislodged more easily.
• Mask lubricated with ointment (eg Aquaphor®)
• Avoid oral airway if possible, may cause oral blistering
• Gentle intubation with well lubricated laryngoscope and small ETT
• Anticipate difficult intubation
• Oral fiberoptic intubation if needed, avoid nasal intubation unless absolutely necessary.
• Laryngeal Mask Airway (LMA) may cause pharyngeal bullae.
• Secure ETT with nonadhesive cotton tape or suture to teeth.
• General endotracheal anesthesia advisable for Esophageal dilation; Dental rehabilitation; Abdominal surgery, major operations
• Mask anesthesia for brief procedures as appropriate
• Other options:
Total Intravenous Anesthesia (TIVA) with propofol + remifentanil or ketamine for whirlpool treatments, peripheral surgery; Regional anesthesia: axillary block, spinal, epidural, caudal
• Muscle relaxants including succinylcholine are fine
• Avoid histamine releasing drugs e.g. morphine to minimize postoperative pruritus
Emergence / Post operative Care:
• Emergence should be smooth to avoid airway, skin trauma
• Suction gently when needed with lubricated suction catheter
• Awake extubation to minimize airway obstruction and need for mask pressure on face
• Appropriate analgesia
• Prophylactic antiemetics to prevent post-operative nausea and vomiting
• Care for new skin lesions
• Monitor for airway compromise
Cannot use adhesive grounding pads
Appropriate perioperative antibiotics
When prepping the skin with betadine solution, apply without friction and remove excess betadine gently blotting with alcohol
Eric Wittkugel, MD
Associate Professor, Clinical Anesthesia and Pediatrics
University of Cincinnati College of Medicine
Director of Anesthesia Preoperative Services
Staff Anesthesiologist, Anesthesia Consultant to EB Center
Cincinnati Children’s Hospital
The information provided herein is intended to educate the reader about certain medical conditions and certain possible treatments. It is not a substitute for examination, diagnosis, and medical care provided by a licensed and qualified health professional. If you believe you, your child, or someone you know, suffer from conditions described herein, please see your healthcare provider. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision.