Tristan de Chalain

Incidence of Oronasal Fistulae and Velopharyngeal Insufficiency After Cleft Palate Repair: An Audit of 211 Children Born Between 1990 and 2004

Objective: We present an audit of primary cleft palate surgery at our unit, including rates of oronasal fistula development, speech outcomes, and rates of velopharyngeal insufficiency requiring secondary surgery. Design: A retrospective study of patients with all cleft palate types, born between January 1990 and December 2004, who underwent primary palatoplasty at Middlemore Hospital, Auckland, New Zealand. Patients: The study included 211 patients, collectively operated on by five different surgeons. Results: The overall rate of true fistula development was 12.8% over a mean follow-up period of 4 years 10 months. The incidence of true fistulae that required surgical repair was 8.1%. Fistula rates were higher for more severe degrees of clefting but were not affected by gender or type of surgical repair. Overall, 31.8% of the study population had some degree of hypernasality following primary palatoplasty. Secondary surgery for velopharyngeal insufficiency was required in 13.3% of patients. Following surgical correction of velopharyngeal insufficiency, no patients were reported to have appreciable hypernasality and 21.7% were reported to have mild hypernasality, a result comparable to previously published audits. The requirement for secondary surgery was higher in patients with more severe clefts. Conclusion: Our results are comparable to other recent studies. We believe that highly coordinated cleft care helps ensure such outcomes. These data provide a benchmark against which we can measure future performance in our attempts to improve outcomes of cleft repair.

Current practice in autologous fat transplantation: suggested clinical guidelines based on a review of recent literature.

After more than a century of use, fat grafting is firmly entrenched in the skill set of plastic surgeons. While macrofat grafting is relatively predictable and reliable, microfat grafting by injection is still in the stages of technical evolution. Review of the current literature suggests that revascularization may take up to 21 days to reach the center of a microfat graft. We recommend harvesting by excision (or gentle aspiration), processing by short and gentle centrifuge to separate the layers, and reinjection of the lower layer via a fine cannula (for example, 17 gauge for the face) and a 1-mL syringe with multiple passes, injecting only a tiny amount with each pass as the needle is withdrawn, to obtain the most reliable clinical outcome.

Parry–Romberg syndrome: intracranial MRI appearances

AIM To gain further insight into the pathogenesis of Parry-Romberg syndrome, a sporadic disease of unknown aetiology characterized by progressive wasting of one side of the face. METHOD Cranial MRI was performed in 10 patients with Parry-Romberg syndrome. The central nervous system findings are correlated to clinical findings and a review of the literature. RESULTS Three patients with a history of migraine had abnormal brain findings confined to the cerebral hemisphere ipsilateral to the facial hemiatrophy. Two patients without CNS symptoms had intracranial changes, one ipsilateral, the other both ipsilateral and contralateral to the facial hemiatrophy, on MRI. These changes consisted of either intracerebral atrophy or white matter hyperintensity. Five patients without CNS symptoms had no pathological intracranial MRI appearances. CONCLUSION A significant number of patients with Parry-Romberg syndrome may have underlying brain involvement. These findings are consistent with previous reports.

Middle ear disease in children with cleft palate: Protocols for management

OBJECTIVE There is wide international variation in the protocols used for middle ear disease management in cleft palate patients. Ventilation tube (grommet) insertion may occur routinely at the time of palatoplasty or selectively on a separate occasion if symptomatic middle ear disease develops. The audiological and otologic outcomes of cleft palate patients were studied in a single institution over a timeframe in which both protocols were utilised. METHODS This was a retrospective study of 234 cleft palate patients who underwent palatoplasty from 1990 to 2005 at Middlemore Hospital, Auckland, New Zealand. Data on hearing loss, middle ear disease, and tympanic membrane abnormalities was collected from clinical notes. Audiological data was obtained from pure tone audiogram reports. RESULTS Forty-five patients had routine grommets inserted concurrent with palatoplasty and 189 patients were managed conservatively with selective grommet insertion if indicated. Grommets were subsequently required in 79 (41.8%) of these 189 patients. There was no difference in the incidence of persistent conductive hearing loss, but recurrent middle ear disease, tympanic membrane abnormalities, and the total number of grommet insertions were significantly higher in the routine grommet group. Poorer outcomes were noted in patients who had undergone a greater number of grommet insertions. CONCLUSION No significant deterioration in audiological outcomes and better otologic outcomes were found in cleft palate patients undergoing selective grommet insertion compared to routine grommet insertion. It is recommended that ventilation tube placement occur in patients selected on the basis of symptomatic infection or significant hearing loss.

Idiopathic muscular torticollis in children : the Cape Town experience

54 cases of Idiopathic Muscular Torticollis (IMT) referred for surgery over a 23-year period and 134 cases referred for physiotherapy over a 5-year period have been reviewed. Long-term cosmetic and functional results for 30% of the surgical cases are presented, with a mean follow-up time of 10.5 years. Demographic features, the role of physiotherapy, the timing of surgery and serial assessment are discussed and results are compared with similar studies from other centres. Locally, of the 134 cases referred primarily for physiotherapy, 36% defaulted from treatment, 60% enjoyed lasting benefit and 4% required subsequent surgical intervention. In the surgical cases, while early surgery appears preferable, delayed operation, even up to the ages of 4 or 5 years did not seem to prejudice long-term results, providing that the advent of facial hemihypoplasia did not precede surgery. Delay beyond this point, or roughly 6 or 7 years of age, would seem to prejudice function and/or cosmesis.

Our experiences managing a patient with mandibular duplication and cervical spinal fusion.

A patient with Klippel-Feil syndrome, which was associated with Pierre Robin sequence, bilateral mandibular duplication, and occipital clefts, is described. The management, surgery, and possible etiology are discussed in relation to the relevant literature.

Combination of unilateral polydactyly, syndactyly, and clinodactyly with occipitocervical encephalocele and vertebral fusion

This report describes a 6-year-old Mexican boy presenting with inter alia, hitherto unrepaired cervical encephalocele and associated unilateral syndactyly. There was also ipsilateral clinodactyly of the thumb and possible polydactyly of the foot. In addition, there was unilateral fusion of the first and second cervical vertebrae and a Chiari type III malformation. Motor and language skills were grossly normal for age, with the exception of mild left hemiparesis affecting the arm more than the leg. Medical history was significant for incidental drainage from the encephalocele as well as occasional high fevers and possible episodes of central nervous system infection. The clinical findings were not consistent with Meckel-Gruber or any other well-recognized syndrome. It is our contention that this case documents a previously unreported constellation of congenital anomalies and, as such, may suggest a teratological insult or a new syndrome. Surgical repair was carried out with meticulous excision of dysplastic neural tissue, relocation of neural tissue within the thecal sac, and coverage of the repair site with a trapezius muscle flap. Skin was closed directly. Postoperative recovery was largely uneventful, with the exception of a seroma in the donor muscle bed.

Sweet's syndrome triggered by scalding: A case study and review of the literature

Sweet’s syndrome, a neutrophilic dermatosis originally described by Dr. Robert Douglas Sweet in 1964, is characterized by fever, tender erythematous skin lesions (papules, nodules, and plaques), leucocytosis, and a diffuse neutrophilic infiltrate in the upper dermis [1]. Since it was first described, several hundred cases of Sweet’s syndrome have been reported [2]. Skin hypersensitivity or cutaneous pathology is a feature associated with Sweet’s syndrome in which skin lesions appear at sites of trauma, but only occasionally has it been described following thermal injury [3–4]. This case report details a rare case of delayed Sweet’s syndrome secondary to a burn, which involved multiple discrete anatomic sites including areas uninvolved with the original burn.

Our experiences managing a rare cranio-orbital cleft.

A unilateral upper lateral orbital cleft, fitting the description of the extremely rare Tessier cleft number 9 is reported. The management and surgery of this patient are discussed, and the relevant literature is reviewed. The usefulness of three-dimensional computed tomography reconstructions and plastic models in planning the surgery is discussed.