JUNCTIONAL MEDICAL RECORDS
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Medical Record of the anesthetic management of Ava Elizabeth
Medeiros who had a rare form of JEB associated with pyloric atresia
(PA-JEB).
(Due to patient confidentiality, Children's Hospital of Boston did not use Ava's name.)

Abstract ID: A46
Abstract Title: Continuous caudal anesthesia for an eight week old baby with severe
Junctional Epidermolysis Bullosa (JEB)
Poster Type: Either


ABSTRACT BODY
Introduction:Epidermolysis bullosa (EB) is a group of mechanobullous disorders
characterized by excessive susceptibility of the skin and mucosa to separate from
underlying tissues and form bullae. There are three major subtypes (dystrophic EB, EB
simplex and junctional EB) based on genetics, clinical features and histology. JEB is
associated with severe panmucosal involvement (skin, respiratory, genitourinary and
gastrointestinal)(1). We present the anesthetic management of a patient who had a rare
form of JEB associated with pyloric atresia (PA-JEB).
Case report:Our patient (AN) was born prematurely at 34 weeks with a birth weight of
1900 gms. Apart from multiple ulcerated skin lesions she had pyloric stenosis that was
repaired on day of life # 2. Due to airway involvement she had recurrent bouts of stridor
and hypoxemia. At 7 weeks, she developed seizures and lethargy due to sagittal sinus
thrombosis because of dehydration. She presented to the operating room requiring
placement of femoral venous access. Because of the existing laryngeal blisters and
mucositis, we chose to avoid tracheal intubation. Our choice of anesthetic was a
continuous caudal infusion of 3% chloroprocaine for the duration of the surgery. The
operating room was prepared with radiant warming lights. To reduce skin damage, she
was placed on a crease-free gel roll with foam padding of extremities. No EKG stickers
were applied. A blood pressure cuff was placed on the lower extremity after the skin was
covered with paraffin gauze and cycled once every 15 minutes. An existing scalp
intravenous catheter was used to give fluids.Following preparation of the skin with
chlorhexidine spray, an 18 G intravenous catheter was inserted into AN’s caudal space.
A 20 G epidural catheter was threaded and advanced to the upper lumbar level. Instead
of taping , the catheter was secured by sterile petroleum gauze applied to the back.
After negative aspiration from the catheter, a dose of 2cc/kg chloroprocaine bolus was
given over 20 minutes and a 2cc/kg/hr infusion was started. To avoid irritating her face,
we provided oxygen blowby under the drapes. Temperature monitoring was done every
20 minutes from the axilla using a digital temperature probe. A.N did not receive opioids
or benzodiazepines during the procedure. To avoid placing the discharging pad on the
patient’s skin, the surgeons used bipolar diathermy. The catheter was removed at the
end of the one-hour surgery. No new lesions developed secondary to this procedure.
Discussion: Anesthesiologists caring for children with EB should avoid frictional
mechanical forces that cause new bullae to form. The choice of anesthetic technique
depends upon the patient’s disease and severity of existing lesions. Although general
endotracheal anesthesia has been attempted successfully in other subtypes of EB (2,
3), airway manipulation should be avoided in JEB. Subcutaneous infiltration of local
anesthetic is avoided because of the pathology of the disease. Therefore, neuraxial or
regional blockade is an excellent option. Techniques like catheter placement are ideal
for long-term postoperative pain control since oral and rectal routes are unavailable.
References:
1. Herod J. et al,Pediatr Anesth 2002, 2. Iohom G. et al., Euro Acad of Anesth 2001
3. Benavente M.A et al., Paediatr Anaesth 2003
ATTACHED FILES



Reg Anesth Pain Med 2004; 29(2):A46
GeneTests, Links  
EPIDERMOLYSIS BULLOSA WITH PYLORIC ATRESIA

7-14-06
17q11-qter, Chr.2 OMIM PubMed [EB simplex with pyloric atresia]
[Epidermolysis bullosa] Epidermolysis bullosa with pyloric atresia Posted
02-16-06
Junctional epidermolysis bullosa and pyloric atresia in two siblings. Egan N,
Ward R, Olmstead M, Marks JG Jr. Posted 2-16-06
Integrin beta 4 mutations associated with junctional epidermolysis ... Pyloric
atresia associated with junctional epidermolysis bullosa (PA-JEB), is a rare
inherited disor... Posted 12/30/05
Prenatal diagnosis of junctional epidermolysis bullosa associated with pyloric atresia
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V Nazzaro, U Nicolini, L De Luca, E Berti and R Caputo  
I Clinica Dermatologica, University of Milan, Italy.  
Homozygous alpha6 integrin mutation in junctional epidermolysis bullosa with congenital duodenal
atresia
Human Molecular Genetics, Vol 6, 669-674, Copyright © 1997 by Oxford University Press
L Pulkkinen, VE Kimonis, Y Xu, EN Spanou, WH McLean and J Uitto
Department of Dermatology and Cutaneous Biology, Jefferson Medical College, Thomas Jefferson University,
Philadelphia, PA 19107, USA.
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