Alistair R.M. Cobb

Photodynamic therapy in the management of lesions of the head and neck

Photodynamic therapy (PDT) is a promising and effective treatment for lesions of the head and neck. It uses illumination with light of a specific wavelength, which activates a photosensitising drug in the presence of oxygen. It can be used in combination with other treatments or on its own, and results in the cellular destruction of the lesion through a free-radical process. Photosensitisers can be applied topically or given systemically depending on the lesion being treated. Results indicate that PDT is an effective adjunct to standard conventional treatments. We review its use.

Frontofacial monobloc distraction in the very young: a review of 12 consecutive cases

Background: Frontofacial monobloc advancement by distraction osteogenesis is now accepted as an effective treatment for children with syndromic and other complex forms of craniosynostosis because it combines cosmetic advantages with the ability to treat such complications as raised intracranial pressure, advancing exorbitism, and upper airway obstruction in one procedure. In the severely affected child, these complications may be present at an early age, but because of the scale of monobloc surgery, surgeons have been reluctant to perform it on very young children. Methods: The authors report a series of 12 children younger than 30 months with various craniosynostosis-associated syndromes complicated by multiple functional problems—including raised intracranial pressure, exposure of the eyes, airway obstruction, and feeding problems—who underwent frontofacial monobloc surgery by distraction osteogenesis. Results: The mean age at operation was 18 months. Mean advancement achieved was 16.6 mm for the upper face and 17 mm for the midface. Ocular protection and reduction of intracranial pressure (when raised) were achieved in all children. Airway improvement was achieved in all but one child. Although not the primary indication for surgery, there was also a marked improvement in every patients appearance. Complications included two cerebrospinal fluid leaks, pin-site infections, frame slippage requiring replacement, and enophthalmos in one patient. Conclusion: The frontofacial monobloc advance incorporating distraction osteogenesis can be undertaken with acceptable morbidity even in the very young child and good functional outcomes achieved with one operation where previously more were required. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Structural outcomes in the Cleft Care UK study. Part 2: dento-facial outcomes

Structured Abstract Objectives To compare facial appearance and dento-alveolar relationship outcomes from the CSAG (1998) and CCUK (2013) studies. Setting and sample population Five-year-olds born with non-syndromic unilateral cleft lip and palate. Those in the original CSAG were treated in a dispersed model of care with low-volume operators. Those in CCUK were treated in a more centralized, high-volume operator model. Materials and methods We compared facial appearance using frontal view photographs (252 CCUK, 239 CSAG) and dental relationships using study models (198 CCUK, 223 CSAG). Facial appearance was scored by a panel of six assessors using a standardized and validated outcome tool. Dento-alveolar relationships were scored by two assessors using the 5-Year-Olds’ Index. Ordinal regression was used to compare results between surveys. Results Excellent or good facial appearance was seen in 36.2% of CCUK compared with 31.9% in CSAG. In CCUK, 21.6% were rated as having poor or very poor facial appearance compared with 27.6% in CSAG. The percentage rated as having excellent or good dento-alveolar relationships was 53.0% in CCUK compared with 29.6% in CSAG. In CCUK, 19.2% were rated as having poor or very poor dento-alveolar relationships compared to 36.3% in CSAG. The odds ratios for improved outcome in CCUK compared to CSAG were 1.43 (95% CI 1.03, 1.97) for facial appearance and 2.29 (95% CI 1.47, 3.55) for dento-alveolar relationships. Conclusions Facial and dento-alveolar outcomes were better in CCUK children compared to those in CSAG.

Lingual cyst lined by respiratory and gastric epithelium in a neonate

Congenital oral cysts of foregut origin include bronchogenic, enteric, and oesophageal cysts, and they are much rarer than the well described dermoid, epidermoid, and thyroglossal cysts. The exact aetiology is poorly understood, but they are thought to arise from misplaced embryonic rests of the primitive foregut. The presentation of cysts lined by respiratory or gastrointestinal epithelium in the oral cavity is unusual. There have been previous reports of bronchogenic or gastrointestinal epithelium-lined lingual cysts, but few report both features occurring within the same cyst. In view of the scarcity of such reports, we present the case of a lesion on the ventral surface of the tongue of a newborn boy. On removal it was found to be a cyst lined by immature squamous, respiratory, and gastric body epithelium.

Optical diagnostic techniques for use in lesions of the head and neck: review of the latest developments

Optical biopsy systems are a potential adjunct to the histopathological assessment of tissue specimens; they are not invasive and can give an immediate result. We review the most common optical biopsy techniques used to detect lesions of the head and neck: elastic scattering spectroscopy, microendoscopy, narrow band imaging, fluorescence, and optical coherence tomography, and discuss their clinical use.

The surgical management of Treacher Collins syndrome.

Treacher Collins syndrome (TCS), mandibulofacial dysostosis, or Franceschetti-Zwahlen-Klein syndrome, is a rare genetic disorder characterised by dysgenesis of the hard and soft tissues of the first and second branchial arches. Early operations focus on maintaining the airway, protecting the eyes, and supporting auditory neurological development. Later operations include staged reconstruction of the mouth, face, and external ear. Bimaxillary surgery can improve the maxillomandibular facial projection, but correction of malar, orbital rim, and temporal defects may be more difficult. We present a clinical review of the syndrome with a chronological approach to the operations.

The tear-drop sign : a trap door for the unwary?

We describe a patient who presented with features suggestive of an orbital blowout fracture. These were apparently confirmed on imaging and operation was planned. However, further radiological and orthoptic opinions diagnosed both a pre-existing eye condition and benign antral mucosal disease that had been misinterpreted as a single acute problem. The patient was discharged with no need for intervention.

Monobloc and bipartition in craniofacial surgery.

Background The frontofacial monobloc advancement with osteogenic distraction is increasingly used as a surgical treatment for children with complex craniosynostosis-associated syndromes. However, the subfrontal osteotomy cuts to free the facial skeleton from the skull base require extradural retraction of the frontal lobes. The purpose of this study was to determine the frequency and degree of radiologically identifiable frontal lobe changes and whether any such changes affected the patients’ outcome. Methods The clinical records and preoperative and postoperative computed tomography imaging of all patients undergoing monobloc frontofacial distraction advancement (with or without bipartition) were reviewed. A retrospective medical notes review was undertaken to assess any patient or surgically related factors that might predispose to frontal lobe changes and evaluate outcome from surgery. Where available, magnetic resonance imaging scans were reviewed to compare outcome with that on computed tomography. Results Fifty cases were identified as suitable for the study. Eighteen patients (36%) had no frontal lobe changes. Thirty-two cases (64%) did have changes that appeared related to the position of maximum retraction during subfrontal osteotomy cuts. There were no changes in the incidence/extent of these changes over time or of any link to the patients’ diagnosis, age at surgery, phenotype severity, surgery type, or any surgical or postoperative adverse events. We found no evidence that these changes were responsible for neurologic problems (eg, epilepsy) or reduced cognitive function. Conclusions This study reveals a high incidence of frontal lobe changes demonstrable on neuroimaging following the frontofacial monobloc procedure reflecting the retraction points during surgery. Although no postoperative disability was reported, it is clearly important to consider more detailed neuropsychologic testing and review current surgical techniques to ensure that such changes are kept to a minimum.

Craniofacial disorders that have phenotypic overlap with Treacher Collins syndrome

The mandibulofacial dysostoses are congenital disorders that result in abnormalities of the first and second pharyngeal arches. The most well known mandibulofacial dysostosis is Treacher-Collins syndrome (TCS) a disorder of craniofacial morphogenesis (Table 1). TCS is the result of mutations in the gene that encodes the protein Treacle which is needed for neural crest stability. Animal models have postulated that extensive neuroepithelial apoptosis could explain the skeletal and soft tissue deformities seen in TCS. Mandibulofacial Dysostosis with Microcephaly (MFDM) also results in the same craniofacial presentation as TCS but with bilateral zygomatic arch clefting, microcephaly, developmental delay, sensorineural hearing loss, cleft palate, choanal atresia (Table 2). In contrast, the neurocranium in TCS has been found to have normal dimensions in length, height and volume but an abnormal shape. These features identified in MFDM have been reported in the literature in patients diagnosed with TCS but have been genetically tested for Treacle mutations and found to be negative. Nager and Miller syndromes are conditions that also have facial phenotypic overlap with TCS but present

The surgical management of severe macroglossia in systemic AL amyloidosis

Amyloidosis is a disease characterised by the deposition in body tissues of amyloid: abnormal protein in a beta pleated sheet formation. It is a systemic disorder and macroglossia may be seen in all forms. Changes to the normal architecture of the tissues and systemic features of the disease and its underlying cause can complicate the surgical management of the enlarged tongue.