Anestesia Pediatrica e Neonatale

The Epidermolysis Bullosa (EB) is a rare genetic disease characterized by the presence of extremely fragile skin and recurrent blisters formation resulting from mechanical friction or trauma.

Simplex EB (blisters within the epidermis),
Junctional EB (at the level of lamina lucida within the basemente membrane zone)
Dystrofic EB (lesions are located at level of lamina densa, with an important scarring component).

The most serious forms can be deadly since intrauterine e/o neonatal age; the first signs are manifested in age neonatale or in the first months of life.

EB involves the skin of the whole body and the great part of the surfaces mucous, inclusive those of the oral hollow, esophagus, stomach, intestines, lungs, bladder, genital region and eyes.

Patients generally die in the second - third ten of life and the exitus is related to the cachettic state correlated to the MOF.

From the month of January 2001 to today, near the Departmente of University if Foggia ( Italy ), 13 patients have been treated with microsurgical technique (range 10-40 years old; 7M - 6F ; bodily regions submitted to surgical intervention: hands 4 - feet 5).

All the subjects joints to my observation have been performed through the use of one among the followings of anesthesiological technicals :

• - deep sedazione with fentanile and propofol + infiltration with local anesthetic

• - deep sedazione with Ketamina and fentanest + infiltration with local anesthetic

All the patients have been submitted to numerous medications performed previous narcosis performed through the use of facial mask (alogenato: sevorane). It's fundamental results the use of fat gauzes or special medications (es.: Mepilex – soft silicone foam dressing with special adhesive surface that can be disconnected from the cutaneous surface without practicing such pressures to provoke the cutaneous lesion).

The gotten results allow us to affirm that the association of microsurgical technique with techniques of local anesthesia and sedazione are able to reduce operative and postoperative risk, reducing notably the recovery times and hospitalization of the patients.

Figure 1. An 18-month-old child with epidermolysis bullosa simplex (EBS), autosomal recessive type, with bullae on the distal tarsal aspect of the foot and a healing, open blister on the medial malleolus of the left foot.

Figure 2. Electron microscopy demonstrating an intraepidermal blister with few inflammatory cells in an 18-month-old patient with EBS, autosomal recessive type. (The original specimen was stained with uranylacetate and lead citrate and magnified 5 x 103).
BC - basal cell, B - parts of a basal cell, M - mitochondrion, arrows - remnants of basement membrane

1. Griffin RP, Mayou BJ: The anaesthetic management of patients with dystrophic epidermolysis bullosa. Anaesthesia 1993; 48: 810-5.

3. Hagen R, Langenberg C: Anaesthetic management in patients with epidermolysis bullosa dystrophica. Anaesthesia 1988; 43: 482-5.

4. Smith GB, Shribman AJ: Anaesthesia and severe skin disease. Anaesthesia 1984; 39: 443-55.

5. Lee C, Nagel EL: Anesthetic management of a patient with recessive epidermolysis bullosa dystrophica. Anesthesiology 1975; 43: 122-4.

6. Patch MR, Woodey RD: Spinal anaesthesia in a patient with epidermolysis bullosa dystrophica. Anaesth Intensive Care 2000; 28: 446-8.

7. Smith GB, Shibman AJ: Anaesthesia and severe skin disease. Anaesthesia 1984; 39: 443-55.

8. White JE: Minocycline for dystrophic epidermolysis bullosa. Lancet 1989; 1: 966-9.

9. Kaplan R, Strauch B: Regional anesthesia in a child with epidermolysis bullosa. Anesthesiology 1987; 67: 262-4.

10. Kelly RE, Koff HD, Rothaus KO, Carter DM, Artusio JF: Brachial plexus anesthesia in eight patients with recessive dystrophic epidermolysis bullosa. Aneslh Analg 1987; 66: 1318-20.

11. Sptelman FJ, Mann ES: Subarachnoid and epidural anaesthesia for patients with epidermolysis bullosa. Can Anaesth Soc J 1984; 31: 549-55.

12. Idvall J: Ketamine monoanaesthesia for major surgery in epidermolysis bullosa. Ada Anaesthesiol Scand 1987; 31: 658-60.

Anesthesia and Epidermolysis Bullosa (EB): Referral Center in University Hospital of Foggia, Italy

Salvatore Meola, MD


		Anesthesia and Epidermolysis Bullosa (EB): Referral Center in University Hospital of Foggia, Italy
		Salvatore Meola, MD Responsible for Plastic and Reconstructive Anesthesia University Department of Anesthesia and Intensive Care, University Hospital "Ospedali Runiti" of Foggia, Italy Vol. 5, N. 2, Luglio 2007
	The Epidermolysis Bullosa (EB)* is a rare genetic disease characterized by the presence of extremely fragile skin and recurrent blisters formation resulting from mechanical friction or trauma.* Pathology manifests him in different clinical pictures: Simplex EB (blisters within the epidermis), Junctional EB (at the level of lamina lucida within the basemente membrane zone) Dystrofic EB (lesions are located at level of lamina densa, with an important scarring component). The most serious forms can be deadly since intrauterine e/o neonatal age; the first signs are manifested in age neonatale or in the first months of life. EB involves the skin of the whole body and the great part of the surfaces mucous, inclusive those of the oral hollow, esophagus, stomach, intestines, lungs, bladder, genital region and eyes. Patients generally die in the second - third ten of life and the exitus is related to the cachettic state correlated to the MOF. From the month of January 2001 to today, near the Departmente of University if Foggia ( Italy ), 13 patients have been treated with microsurgical technique (range 10-40 years old; 7M - 6F ; bodily regions submitted to surgical intervention: hands 4 - feet 5). All the subjects joints to my observation have been performed through the use of one among the followings of anesthesiological technicals : • - ALR (anesthesia of the brachial plesso) • - deep sedazione with fentanile and propofol + infiltration with local anesthetic • - deep sedazione with Ketamina and fentanest + infiltration with local anesthetic All the patients have been submitted to numerous medications performed previous narcosis performed through the use of facial mask (alogenato: sevorane). It's fundamental results the use of fat gauzes or special medications (es.: Mepilex – soft silicone foam dressing with special adhesive surface that can be disconnected from the cutaneous surface without practicing such pressures to provoke the cutaneous lesion). The gotten results allow us to affirm that the association of microsurgical technique with techniques of local anesthesia and sedazione are able to reduce operative and postoperative risk, reducing notably the recovery times and hospitalization of the patients. Figure 1. An 18-month-old child with epidermolysis bullosa simplex (EBS), autosomal recessive type, with bullae on the distal tarsal aspect of the foot and a healing, open blister on the medial malleolus of the left foot. Figure 2. Electron microscopy demonstrating an intraepidermal blister with few inflammatory cells in an 18-month-old patient with EBS, autosomal recessive type. (The original specimen was stained with uranylacetate and lead citrate and magnified 5 x 103). BC - basal cell, B - parts of a basal cell, M - mitochondrion, arrows - remnants of basement membrane References 1. Griffin RP, Mayou BJ: The anaesthetic management of patients with dystrophic epidermolysis bullosa. Anaesthesia 1993; 48: 810-5. 2. Lin AN, Carter DM: Epidermolysis bullosa. Annu Rev Med 1993; 44: 189-99. 3. Hagen R, Langenberg C: Anaesthetic management in patients with epidermolysis bullosa dystrophica. Anaesthesia 1988; 43: 482-5. 4. Smith GB, Shribman AJ: Anaesthesia and severe skin disease. Anaesthesia 1984; 39: 443-55. 5. Lee C, Nagel EL: Anesthetic management of a patient with recessive epidermolysis bullosa dystrophica. Anesthesiology 1975; 43: 122-4. 6. Patch MR, Woodey RD: Spinal anaesthesia in a patient with epidermolysis bullosa dystrophica. Anaesth Intensive Care 2000; 28: 446-8. 7. Smith GB, Shibman AJ: Anaesthesia and severe skin disease. Anaesthesia 1984; 39: 443-55. 8. White JE: Minocycline for dystrophic epidermolysis bullosa. Lancet 1989; 1: 966-9. 9. Kaplan R, Strauch B: Regional anesthesia in a child with epidermolysis bullosa. Anesthesiology 1987; 67: 262-4. 10. Kelly RE, Koff HD, Rothaus KO, Carter DM, Artusio JF: Brachial plexus anesthesia in eight patients with recessive dystrophic epidermolysis bullosa. Aneslh Analg 1987; 66: 1318-20. 11. Sptelman FJ, Mann ES: Subarachnoid and epidural anaesthesia for patients with epidermolysis bullosa. Can Anaesth Soc J 1984; 31: 549-55. 12. Idvall J: Ketamine monoanaesthesia for major surgery in epidermolysis bullosa. Ada Anaesthesiol Scand 1987; 31: 658-60.