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Biomedical subjects

B J Mayou

Publications and source records attributed to B J Mayou.

At least 19 recordsLinked to original sources
PubMed1995

The 'Pinocchio' nasal deformity due to cavernous lymphangioma.

The 'Pinocchio' or 'Cyrano' nose is a rare condition in which deformity of the nasal tip is produced by an underlying soft tissue tumour. Previously reported cases have been due to either capillary or cavernous haemangiomas (angiolipomas). The deformity is the cause of much teasing in children. There has been debate as to whether surgical intervention is indicated, as a proportion of cases will regress spontaneously. We report a case of 'Pinocchio' nose with a lymphangioma of the nasal tip which is previously undescribed and review the options for management.

Child, Preschool↗
PubMed1993

Cultured keratinocyte allografts and wound healing in severe recessive dystrophic epidermolysis bullosa.

BACKGROUND: Patients with recessive dystrophic epidermolysis bullosa (RDEB) frequently have painful erosions that are slow to heal. There is no definitive treatment; therefore any therapy that improves wound healing would be beneficial to these patients. OBJECTIVE: Our purpose was to assess the effects of cultured allogeneic keratinocytes on wound healing in RDEB. METHODS: Ten patients with RDEB and dermatome-induced superficial dermal wounds were studied. Cultured keratinocyte grafts were applied to part of the wound, with another part left ungrafted. Both sites were assessed clinically and microscopically, particularly with regard to basement membrane zone reconstitution. RESULTS: Apart from minor differences in keratinocyte differentiation and a moderate analgesic effect induced by the graft, there were no other distinguishing findings in wound healing in the grafted and nongrafted sites. CONCLUSION: There was little clinical benefit from cultured keratinocyte allografts in wound healing in RDEB. However, this study showed that RDEB keratinocytes have an inherent capacity to express some type VII collagen epitopes transiently during wound healing, although this was not associated with the detection of anchoring fibrils.

Adolescent↗
PubMed1993

The anaesthetic management of patients with dystrophic epidermolysis bullosa. A review of 44 patients over a 10 year period.

The case notes and anaesthetic charts of 44 patients with dystrophic epidermolysis bullosa were reviewed retrospectively. A total of 390 general anaesthetics were performed for 469 surgical procedures over a 10-year period. Procedures included repair of syndactyly, dilatation of oesophageal strictures, extraction of teeth, excision of skin tumours and other procedures related to dystrophic epidermolysis bullosa. Anaemia, thrombocytosis and hypoalbuminaemia was found in many patients pre-operatively. Damage to skin or mucosa occurred peri-operatively. Four patients developed bullae around the head and neck, two patients developed oral or pharyngeal bullae and three patients developed conjunctival abrasions including one with a new corneal ulcer. There were no reports of laryngeal bullae and no bullae associated with intramuscular injections or the use of limb tourniquets. Thirty-one patients (70%) had evidence of oesophageal strictures and six regurgitated peri-operatively. In all cases there was a history of oesophageal disease. In 10 patients (23%) intubation of the trachea was difficult. It was carried out by various methods including blind nasal and fibreoptic intubation or by using the laryngeal mask airway. One patient requiring a series of general anaesthetics had a tracheostomy formed after fibreoptic intubation. Previous reports have suggested problems with the use of suxamethonium, nondepolarising muscle relaxants and thiopentone. These agents were all used in many of our patients without any adverse effects. There was one intra-operative death in a 35-year-old woman having an oesophageal dilatation which was complicated by oesophageal rupture.

Adolescent↗
PubMed1993

The clinical spectrum of lipoatrophic panniculitis encompasses connective tissue panniculitis.

Two patients with widespread, chronic, relapsing panniculitis resulting in disfiguring lipoatrophy are reported. Histology in both cases showed a mixed septal and lobular panniculitis, with lipophagia. The clinical appearance and histology suggested a diagnosis of lipoatrophic panniculitis. Both cases had features of connective tissue panniculitis, which is likely to be a subtype of this condition. Treatment of lipoatrophic panniculitis can be difficult. Our first patient initially responded well to antimalarial therapy, allowing plastic surgical repair of the defects to be carried out.

Adult↗
PubMed1992

Mitten deformity in severe generalized recessive dystrophic epidermolysis bullosa: histological, immunofluorescence, and ultrastructural study.

Light and electron microscopy and indirect immunofluorescence techniques were used to study the nature of the mitten deformity in five adult patients with severe generalized recessive dystrophic epidermolysis bullosa undergoing release of hand and finger contractures. Although the mitten appeared largely to be clinically separated from the underlying fixed digits, histology showed mostly normal keratinocytes beneath a thickened stratum corneum. The lower margin of the mitten was formed just below the lamina densa of the basement membrane, at a level similar to that of the usual blister formation in this condition. No anchoring fibrils and only a few distinct dermal structures were noted. A substantial portion of the mitten, however, consisted of necrotic keratinocytes without an intact basement membrane. This finding suggests that the mitten is not suitable for use as an epidermal autograft and confirms the rationale for taking split thickness skin grafts to close skin defects in patients with recessive dystrophic epidermolysis bullosa undergoing plastic surgery.

Adult↗
PubMed1992

Epidermolysis bullosa complicated by squamous cell carcinoma: report of 10 cases.

Epidermolysis bullosa (EB) refers to a group of hereditary mechano-bullous conditions, many of which are associated with chronic scarring. Several forms of the disease have been reported in association with cutaneous malignancy. We present a series of 10 EB patients (eight generalised recessive dystrophic EB, one dominant dystrophic EB, one non-lethal junctional EB) aged 24-55 years with a total of 29 squamous cell carcinomas (SCC). Three patients died from metastatic disease associated with invasive, poorly differentiated SCC. Six cases had multiple primary SCC, including three patients with simultaneous multifocal disease. Twenty-eight of the 29 SCC arose on the limbs. Histology revealed that most of the SCC were well or moderately differentiated (22/29). Unusual histological findings included two verrucous SCC, as well as a spindle cell (angiosarcoma-like) SCC. Most of the SCC developed in areas of chronic non-healing ulceration (10/29) or longstanding hyperkeratotic crusting (14/29). The dermis around or beneath the carcinomas was densely scarred, more so than in non-malignant areas. In some cases it was difficult to distinguish the clinical appearances of certain areas of chronic ulceration, scarring, and crusting typical of dystrophic EB from many of the SCC. This study underlines the need for constant vigilance for the development of carcinomas in this group of patients, the occasional diagnostic difficulty, and the potential for metastasis.

Adult↗
PubMed1991

Pain and forehead expansion.

Tissue expansion of the forehead by intermittent bolus injection in three patients was noted to be extremely painful. This stimulated a prospective study in four patients whose discomfort was found to be related to a sharp rise in intraluminal pressure after bolus inflation. Tolerable infusion volumes were small (mean 6.25 cc) and associated with high pain scores (mean 7/10). Adequate expansion was protracted (mean 45 1/2 days) and was limited by pain. To eliminate the sharp rises in pressure associated with expansion by bolus injection, a syringe pump was used in two further inpatients. Adequate expansion was both quicker (mean 8.5 days) and less painful (mean pain score 2.6/10).

Aged↗
PubMed1991

Metastatic squamous cell carcinoma resembling angiosarcoma complicating dystrophic epidermolysis bullosa.

We report a patient with generalized recessive dystrophic epidermolysis bullosa (RDEB) who developed 3 squamous cell carcinomas. The tumours appeared simultaneously at acral sites on both upper limbs and were poorly differentiated. Despite surgery and radiotherapy the patient died from metastatic disease within 6 months of presentation. This case highlights many of the typical features of this complication of RDEB, including the overall poor prognosis. Of particular interest was the histology of one of the tumours which caused diagnostic difficulties: haematoxylin and eosin staining suggested an angiosarcomatous pathology, but the use of immunocytochemistry proved that the tumour was a squamous cell carcinoma in origin.

Carcinoma, Squamous Cell↗
PubMed1989

The eye in dystrophic epidermolysis bullosa: clinical and immunopathological findings.

The ocular findings in a series of patients with dystrophic epidermolysis bullosa are presented. An immunopathological technique using monoclonal antibodies for examining components of the epithelial basement membrane of the conjunctiva is described and the results obtained on a conjunctival biopsy from a patient with dystrophic epidermolysis bullosa are presented.

Adolescent↗
PubMed1988

Congenital divided naevus of the eyelids.

A newborn infant presenting with a divided congenital melanocytic naevus of the eyelid is described. Because of the severe disfigurement, risk of later malignant change in the lesion, and the possibility of deprivation amblyopia, early surgical treatment is recommended for all medium and large congenital melanocytic naevi of the eyelid. Surgery in the first few months of life gives the best cosmetic results.

Dermabrasion↗
PubMed1982

The scapular flap--an anatomical and clinical study.

The scapular flap is a medium-sized flap combining many of the good qualities of others, such as thinness and minimal donor site deficit, with the potentialities of a consistently long pedicle that allows combination with other adjacent flaps. The anatomy of the flap and its application in nine clinical cases is described.

Adolescent↗
PubMed1982

The Laser Doppler flowmeter for microvascular monitoring: a preliminary report.

A preliminary report is presented of a free-flap monitoring technique using the Doppler shift of Laser light. It has provided useful information in five patients with free flaps. The technique is non-invasive and the apparatus is compact, portable and simple to use. Although a long term evaluation with a larger number of patients is required the technique has many advantages over others currently available and is sufficiently promising to warrant further investigation.

Blood Flow Velocity↗
PubMed1981

Free microvascular and microneural transfer of the extensor digitorum brevis muscle for the treatment of unilateral facial palsy.

The operation of transfer of the extensor digitorum brevis muscle to the face in the treatment of unilateral facial palsy (Thompson and Gustavson, 1976) has been further modified by immediate anastomosis of its vascular pedicle to the superficial temporal vessels. Six of our ten patients showed some new movement but in only three did this approach symmetry with the other side. Long term follow-up showed that improvement can be expected for up to two years after transfer. Our technique is assessed critically and suggestions are made for further improvement.

Adult↗
PubMed1981

An onlay vein patch technique for the repair of small vessels.

A method for the repair of defects in small blood vessels is described, using an oversized onlay autogenous vein patch tacked to the adventitia. The technique has been evaluated experimentally and it has been shown that vessels repaired with a patch remain patient with normal blood flow. By contrast, vessels repaired by direct suturing have a high incidence of mural thrombus formation and sluggish flow. The clinical usefulness of the method is illustrated by a case report describing the salvage of a free flap with a damaged vascular pedicle.

Adult↗