Aina V. H. Greig

Correcting the typical Apert face: combining bipartition with monobloc distraction.

Background: Bipartition distraction is a novel procedure combining frontofacial bipartition and monobloc distraction. Apert syndrome and other syndromic craniofacial dysostoses are often characterized by hypertelorism, with a negative canthal axis and counterrotated orbits. Central midface hypoplasia can result in a biconcave face in both midsagittal and axial planes. Bipartition distraction can correct these facial abnormalities. Methods: Twenty patients (19 Apert syndrome patients and one Pfeiffer syndrome patient, aged 1.6 to 21 years) underwent bipartition distraction. Severity of appearance was graded preoperatively and postoperatively as mild, moderate, or severe. Functional problems were documented by a multidisciplinary team. Central and lateral midface skeletal advancement were measured. Follow-up ranged from 15 months to 7 years. Results: Bipartition distraction consistently produced more central than lateral facial advancement. Mean central advancement was 13.2 ± 5.9 mm at sella-nasion and 11.7 ± 5.4 mm at sella-A point. Lateral advancement was 4.7 ± 2.8 mm. Unbending the face improved aesthetic appearance. Airway function, eye exposure, and elevated intracranial pressure were improved. Complications included six temporary cerebrospinal fluid leaks (four needing a lumbar drain), five patients with postoperative seizures, five patients requiring Rigid External Distraction frame repositioning, one palatal fistula, one velopharyngeal incompetence, five pin-site infections, one abscess under frontal bone, three cases of sepsis, nine patients with worsened strabismus, two patients with enophthalmos, one patient with partial visual field loss, and three patients who required reintubation because of aspiration. Conclusions: Bipartition distraction is an effective procedure with which to differentially advance the central face in Apert syndrome. It improves both function and aesthetics. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Parental perceptions of grommet insertion in children with cleft palate.

Otitis media with effusion is almost universal in children with cleft palate and can delay speech, language and educational development by causing hearing loss. Grommet insertion at the time of cleft palate repair is common. There is debate about whether the benefits of grommets outweigh the risk of complications. A postal questionnaire was used to investigate parental perceptions of middle-ear ventilation via grommet insertion in children attending the multidisciplinary cleft palate clinic. These childrens case notes were reviewed. Many children had speech and language delay, but parents thought this improved after grommet insertion. Overall parents were pleased with the results. This confirms that grommets have an important part to play in the management of children with cleft palate.

Solitary fibrous tumour of the deep soft tissues of the neck

A case of a solitary fibrous tumour arising in the deep soft tissues of the neck is reported. This rare tumour has not previously been described in this site. We discuss the clinical presentation and pathological features.

Reconstruction of the exenterated orbit with osseointegrated implants

Osseointegrated implant-fixated prostheses are useful for the reconstruction of the exenterated orbit. They provide good cosmesis, and are comfortable and light weight for the patient to wear. A retrospective review was performed of a series of twenty patients who had undergone orbital exenterations from 2003 to 2006, including complex cases with extraorbital and transcranial tumour excisions, nineteen of which were reconstructed with osseointegrated implant-fixated prostheses. For a successful outcome, decisions need to be made regarding the reconstruction of the orbital rim, periorbital soft tissues, indications for socket reconstruction and decisions regarding both one or two stage implantation and radiotherapy planning. Osseointegrated implants provide the optimal cosmetic and functional results in the rehabilitation of the exenterated orbit in our opinion. Close collaborative planning by both the surgeon and the prosthetist from the outset is essential.

Pfeiffer syndrome: analysis of a clinical series and development of a classification system.

Abstract Among the craniosynostosis syndromes, Pfeiffer syndrome is notable because of high mortality and the need for multiple surgical interventions. However, it is variable in severity. We propose a new classification of Pfeiffer Syndrome to define pathology and function. A retrospective review was conducted of 42 patients with Pfeiffer syndrome treated from 1975 to 2010, the largest series reported to date. The classification was based on a functional assessment of patients in terms of respiratory, ocular, otological, and neurological status. This classification was tested by scoring and stratifying patients as follows: type A (mild problems), B (moderate problems), or C (severe problems). Patients were scored both at the time of presentation and after all surgical interventions to assess change in functional outcome. The functional classification system was compared to another previously reported. Type A patients did not have any change in postoperative functional outcomes (mean preoperative score 1.6, mean postoperative score 1.6); type B patients showed functional improvement (mean preoperative score 4.1, mean postoperative score 3.4) but type C patients (mean preoperative score 7.7, mean postoperative score 4.8) demonstrated the greatest improvement in functional scores after surgical intervention. Suture pathology did not indicate the clinical severity of phenotype, a variance from a previously published classification. The proposed classification is useful to assess severity of phenotype: respiratory, ocular, otologic, and neurologic problems are key indicators of the need for treatment. The classification can provide a helpful guide in multidisciplinary treatment planning, in reporting outcomes, and in the sharing of data among craniofacial anomalies centers.

Prospective randomized controlled trial: fibrin sealant reduces split skin graft donor-site pain.

Background: Pain at split skin graft donor sites is common. Fibrin sealant has been demonstrated to reduce time to hemostasis at wound sites, and patients receiving this treatment were incidentally noted to report less pain. This study aimed to evaluate pain and incapacity in split skin graft donor sites treated with and without fibrin sealant. Methods: Fifty patients requiring thigh donor-site split skin grafts were prospectively randomized to receive either a self-adhesive fabric dressing alone or fibrin sealant plus the self-adhesive fabric dressing as primary donor-site dressings. External secondary dressings were the same. Patients were blinded with regard to treatment group. Using visual analogue scales (scored 0 to 5), patients rated their donor-site pain and incapacity for 14 days postoperatively. Secondary endpoints were length of hospital stay and duration of requirement for dressings. Results: Forty patients were included in the study analysis and completed self-reported pain and incapacity scores. Twenty received the fibrin sealant plus self-adhesive fabric dressing and 20 received the fabric dressing only (controls). Patients using the fibrin sealant plus the dressing reported significantly less pain (mean score, 0.42 versus 1.60, p < 0.001) and significantly less incapacity (mean score, 0.48 versus 1.71, p < 0.001). Patients allocated to the fibrin sealant group recorded shorter lengths of stay and faster time to discontinuation of dressing, though statistical significance was not achieved. Conclusion: Patients whose split skin graft donor sites were dressed with fibrin sealant plus self-adhesive fabric dressing experienced significantly less pain and incapacity than patients with self-adhesive fabric dressings alone, allowing a more rapid return to normal activity. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, II.

Purinergic receptors are part of a signalling system for proliferation and differentiation in distinct cell lineages in human anagen hair follicles

We investigated the expression of P2X5, P2X7, P2Y1 and P2Y2 receptor subtypes in adult human anagen hair follicles and in relation to markers of proliferation [proliferating cell nuclear antigen (PCNA) and Ki-67], keratinocyte differentiation (involucrin) and apoptosis (anticaspase-3). Using immunohistochemistry, we showed that P2X5, P2Y1 and P2Y2 receptors were expressed in spatially distinct zones of the anagen hair follicle: P2Y1 receptors in the outer root sheath and bulb, P2X5 receptors in the inner and outer root sheaths and medulla and P2Y2 receptors in living cells at the edge of the cortex/medulla. P2X7 receptors were not expressed. Colocalisation experiments suggested different functional roles for these receptors: P2Y1 receptors were associated with bulb and outer root sheath keratinocyte proliferation, P2X5 receptors were associated with differentiation of cells of the medulla and inner root sheaths and P2Y2 receptors were associated with early differentiated cells in the cortex/medulla that contribute to the formation of the hair shaft. The therapeutic potential of purinergic agonists and antagonists for controlling hair growth is discussed.

The question mark ear in patients with mandibular hypoplasia.

Sir:The question mark ear is characterized by a cleft between the lower part of the helix and the earlobe, sometimes with a prominent upper pole of the pinna, and was first described in 1961.1 The term “question mark ear” was coined in 1970.2 Ear malformations and mandibular hypoplasia can occur tog

Heinrich von Pfalzpaint, Pioneer of Arm Flap Nasal Reconstruction in 1460, More Than a Century Before Tagliacozzi.

AbstractHeinrich von Pfalzpaint (circa 1415–1465) was a Bavarian military surgeon of the Teutonic Order who treated more than 4000 casualties during the siege of Marienberg Fortress (1454–1457). In 1460, he reported “How to create a new nose if it has been chopped off and the dogs have eaten it” in his treatise on wound care Bündt-Ertznei. He used opium-soaked sponges for anesthesia, described the surgical extraction of bullets and cleft lip repair. Pfalzpaint would have been the first author to describe nasal reconstruction in Europe if his treatise had not been lost. Only 5 copies of his manuscript existed. One was rediscovered and printed in 1868. Pfalzpaints technique for nasal reconstruction was performed in 2 stages using an undelayed skin flap from the upper arm, which was sutured to the nasal defect and the arm was bandaged to the head. After 8 to 10 days, he divided the pedicle; inset the flap; and fashioned the nasal dorsum, alae, and columella. Tagliacozzi described arm flap nasal reconstruction more than a century later in 1597. He used delayed skin flaps, with at least 6 operative stages over 4 months. Pfalzpaint was ahead of his time regarding his knowledge of wounds, insistence on surgical cleanliness, and his technically easier arm flap rhinoplasty, compared with Tagliacozzi. Pfalzpaint, who is rarely referenced in the literature, should be remembered as a great pioneer of reconstructive surgery in Europe.