Background and objective Epidermolysis bullosa is a rare, genetically determined disorder characterized by excessive susceptibility of the skin and mucosa to separate from the underlying tissues after mechanical trauma. Patients suffering from this disease may have multiple medical problems, but the risk of anaesthesia is attributed mainly to oropharyngeal involvement; airway management may be hazardous and preservation of mucosa and skin integrity problematical. There is a paucity of data in the literature concerning the perioperative management of children with epidermolysis bullosa. We present our experience of managing 54 general anaesthetics (and two local anaesthetics) over the past 20 years.

Methods The case notes of 16 children with epidermolysis bullosa were reviewed. The children underwent surgical procedures under local or general anaesthesia over a 20-year period.

Results Fifty-four general and two local anaesthetics were administered for 58 procedures in 10 children (mean age 12.6 years). Surgical procedures included oesophageal dilatation (24), insertion/revision of gastrostomy (16), dental procedures (10), hand surgery (2), skin biopsy (2) and others (4). Anaesthesia was induced by inhalation in 73.4% of patients and the airway was maintained with an endotracheal tube in 64.8%. Monitoring of anaesthesia was performed with pulse oximetry (89%), whereas electrocardiography and non-invasive blood pressure monitoring were used in 16.6% of cases each. The mean duration of anaesthesia was 64 min. Tracheal intubation was difficult in two of the five children who were intubated. Mucocutaneous blistering occurred in three children, otherwise there was no attributable morbidity.

Conclusion With maximal skin and mucous membrane protection, anaesthesia in children with epidermolysis bullosa may be undertaken with few sequelae.

Laminins are a family of multifunctional macromolecules, ubiquitous in basement membranes, and represent the most abundant structural noncollagenous glycoproteins of these highly specialised extracellular matrices. Their discovery started with the difficult task of isolating molecules produced by cultivated cells or extracted from tissues. The development of molecular biology techniques has facilitated and accelerated the identification and the characterisation of new laminin variants making it feasible to identify full-length polypeptides which have not been purified. Further, genetically engineered laminin fragments can be generated for studies of their structure-function relationship, permitting the demonstration that laminins are involved in multiple interactions with themselves, with other components of the basal lamina, and with cells. It endows laminins with a central role in the formation, the architecture, and the stability of basement membranes. In addition, laminins may both separate and connect different tissues, i.e. the parenchymal and the interstitial connective tissues. Laminins also provide adjacent cells with a mechanical scaffold and biological information either directly by interacting with cell surface components, or indirectly by trapping growth factors. In doing so they trigger and control cellular functions. Recently, the structural and biological diversity of the laminins has started to be elucidated by gene targeting and by the identification of laminin defects in acquired or inherited human diseases. The consequent phenotypes highlight the pivotal role of laminins in determining heterogeneity in basement membrane functions.