Laryngeal involvement in the Dowling-Meara variant of epidermolysis bullosa simplex with keratin mutations of severely disruptive potential.

The clinical features of the Dowling-Meara variant of epidermolysis bullosa simplex (EBS-DM) can, in an infant, be indistinguishable from other severe forms of epidermolysis bullosa (EB). Two unrelated infants with no family history of skin disease are described who, within hours of birth, developed extensive blistering of skin and oral mucosae and who both subsequently developed hoarse cries. Despite this superficial resemblance to other forms of EB, electron microscopy revealed a basal cell rupture and keratin aggregates characteristic of EBS-DM in the skin of both infants and in the vocal cord epithelium of one. Molecular analysis confirmed the diagnosis by identification of mis-sense point mutations in basal cell keratin genes in both cases. One patient carries a point mutation in keratin 14 (converting arginine at position 125 to histidine) and the other has a novel point mutation in keratin 5 (converting serine at position 181 to proline). Hoarseness is not a well documented feature of EBS-DM and is usually associated with junctional EB. These two patients demonstrate that the presence of a hoarse cry in an infant affected by severe EB does not necessarily indicate a poor prognosis.

Is the nasopharynx warmer in children than in adults?

Recent studies on the aetiology of the Sudden Infant Death Syndrome (SIDS) have suggested that some of these deaths are the consequence of an overwhelming inflammatory response to the production of pyrogenic toxins from bacteria colonizing the upper respiratory tract, particularly the nasopharynx. The pyrogenic toxins of Staphlococcus aureus, one of the likelier bacterial candidates, are only produced in temperatures of over 37 degrees C. This study examined nasopharyngeal temperatures in children. It is a preliminary study to develop an accurate means to measure how close to 37 degrees C the nasopharyngeal temperature lies in infants at the age when SIDS deaths occur. Following a pilot study and power calculation, measurements of nasopharyngeal temperature were made on 30 apyrexial children aged 4-10 years and 30 adults with no nasal pathology, undergoing surgery under general anaesthesia, using an accurately sited thermocouple probe. The mean temperature in children (35.64 degrees C) was significantly higher than in adults (34.01 degrees C). Comparable measurements attempted with the same subjects awake gave similar results.

Persistent stapedial artery preventing successful cochlear implantation: a case report.

A case of persistent stapedial artery preventing cochlear implantation is described and the literature relating to this congenital anomaly is reviewed. The computed tomographic features of the artery are illustrated, and it is recommended that preoperative computed tomography be specifically screened for this anomaly.

Blom-Singer prostheses--an 11 year experience of primary and secondary procedures.

Over the past 11 years, 25 primary and 29 secondary tracheo-oesophageal punctures have been performed in 51 laryngectomees. Pre-operative assessment is required for the secondary procedure. Both primary and secondary procedures require intensive speech therapy after fitting of the prosthesis. Fifty-four per cent of the primary and 37% of the secondary patients attained long-term speech, the latter figure rising to 58% with newer valves and increasing experience. Failures were commonly due to psychological or prosthesis-related problems. Complications were relatively minor, none being life threatening. Seventy per cent of the long-term users were self caring. Adequate rehabilitation counselling and support is necessary to ensure long-term use of the prosthesis.

The assessment of alaryngeal speech.

The objective measurement of alaryngeal speech is desirable so that results between different centres can be compared. We examined several phonatory parameters of alaryngeal speech with the Visi-pitch and correlated these with the subjective assessment of voice quality. Amplitude, percentage voiced and perturbation correlated extremely well with the subjective assessment of voice quality.

Combined study to assess the role of calcium alginate swabs and ligation of the inferior tonsillar pole in the control of intra-operative blood loss during tonsillectomy.

Bleeding is the major source of morbidity and mortality of tonsillectomy and most operative time is spent securing haemostasis. Calcium alginate reduces experimental wound blood loss. This study assessed the efficacy of alginate swabs during tonsillectomy and inferior tonsillar pole ligation in the control of blood loss during tonsillectomy. Ninety-nine tonsillectomy patients were randomized to gauze or alginate swab use. A haemoglobinometer measured blood losses collected by suction during dissection and by swab until haemostasis was secured. Operative time, complications and healing were also assessed. Mean total blood losses and operative times were similar for the two groups. Swab blood loss was significantly lower for surgeons who ligated the lower tonsillar pole, compared with surgeons who used a snare. This finding was independent of swab type. Independent assessment showed no difference in the healing rate. Alginate swabs offer no advantage over gauze in terms of blood loss, operative time or complications of tonsillectomy. The results show that routine lower pole ligation significantly reduces post-dissection blood loss. We suggest that this technique should be more widely adopted.

Ulcerative colitis and giant cell arteritis associated with sensorineural deafness.

Sensorineural deafness is rarely associated with both ulcerative colitis and giant cell arteritis. A patient is described in whom acute sensorineural deafness occurred in association with episcleritis, ulcerative colitis and clinical features suggesting giant cell arteritis.

What's new in surgery: otolaryngology.

It is a decade since the last review article on otolaryngology in this Journal. In that time there have been significant advances in the practice of an ever-changing specialty which originally started off as a surgical specialty designed to drain abscesses from the bony boxes in the head, namely the mastoids and the sinuses. It is impossible, in an article like this, to do anything other than superficially indicate the changes but a list of further reading is given for each section.

Facial schwannoma without facial paralysis.

A 64-year-old man with right aural discharge presented complaining of progressive deafness. Other otological symptoms were absent and specifically there was no seventh nerve paresis. A right aural polyp was identified and biopsied. Histology showed the polyp to be a schwannoma. Subsequent temporal bone computed tomography showed expansion of the distal facial canal. At operation, the schwannoma filled the middle ear cleft and extended from the genu to the region of the stylomastoid foramen. The floor of the middle ear had been eroded, exposing the jugular bulb. Facial paresis is the usual presenting feature of a facial schwannoma, while deafness, ageusia and reduced lacrimation are variable, dependent upon the site of the lesion. The absence of facial palsy as a presenting feature is very rare and this case illustrates the need for histological examination of all abnormal aural material.

Review. Six years experience of Plastipore.

A 6 year personal experience in the performance of Plastipore prostheses in middle ear reconstruction is presented. The good hearing results at 12 months are not maintained with TORPs or PORPs and 41.6% have been rejected. Better results are obtained with a tube prosthesis between a mobile malleus and stapes. The use of TORP and PORP has now been abandoned in favour of ossicles or homograft cartilage.

Serologic survey for leptospirosis in coyotes in northcentral Kansas.

During 1975 to 1977, serum samples were collected from 101 adult coyotes (Canis latrans) captured in northcentral Kansas. Ten samples were seropositive by microagglutination testing and six of those samples were seropositive for multiple serovars. Titers for Leptospira interrogans serovars grippotyphosa, pyrogenes, djasiman, butembo, and pomona were demonstrated.

Experimentally induced leptospirosis in coyotes (Canis latrans).

Infection of coyotes (Canis latrans) with Leptospira interrogans serovars pomona, canicola, and copenhageni was accomplished by percutaneous inoculation. Bacteriologic, serologic, histopathologic, and fluorescent antibody techniques were used to investigate the infections. Leptospiremia was established with pomona. Leptospiruria was demonstrated with the three serovars. Serovar canicola was recovered from one coyote 134 days after it was inoculated.

Iron deficiency in children undergoing adenoid-tonsillectomy.

Following a preliminary study of peripheral blood values in 30 children undergoing tonsillectomy and adenoidectomy, a biochemical survey of the iron status of another 108 children also undergoing this operation was made utilizing an automated micro-method. It was shown that 8.4% of these children were frankly iron-deficient but there was no statistical relationship between the levels of serum iron and iron-binding capacity, the incidence of throat infections and the levels of the plasma immunoglobulins. Assessment of all children undergoing this operation for the presence of iron deficiency is advocated as a possible routine screening procedure.

Simple nasal polyps and allergic manifestations.

A series of 74 consecutive patients with simple nasal polyps were interviewed with regards to features of allergy. Skin testing with a variety of antigenic extracts and estimations of peripheral blood immunoglobulins including total serum IgE were also carried out. The simple tests performed in this study have not been able to delineate a specific subgroup of atopic patients with nasal polyps. Positive skin testing with house dust mite extract may form the basis of a clinical evaluation of hyposensitization.

Guillotine tonsillectomy: anachronism or pragmatism.

The results of a retrospective survey comparing the complications of guillotine tonsillectomy with those of dissection tonsillectomy are given. The problem of tonsil tags and adenoidectomy are discussed and a reappraisal of the indications for guillotine tonsillectomy are considered. We feel that guillotine tonsillectomy should only be carried out in very special circumstances.

A study of serum IgA levels in children undergoing tonsillectomy.

Earlier work has suggested that up to 25% of children undergoing tonsillectomy because of recurrent sore throats are relatively deficient in IgA immunoglobulin and that they do less well after the operation than 'normal' children. Measurements of serum immunoglobulin levels were carried out in 96 children undergoing tonsillectomy for recurrent sore throats. Levels of IgA, IgM and IgG were found to be similar to those in healthy children, 7-29% of those studied had 'low' IgA serum levels. There was a significant relationship between serum IgA levels and the age of the child. No relationship could be established between IgA levels and pre- or postoperative clinicaal state of the children. Low IgA serum levels are probably the result of delayed immunological maturation rather than a true immunodeficient state. IgA estimations, therefore, have no bearing on selection for operation or the prognosis after operation.