A Prospective Study to Examine Responsiveness and Minimally Important Differences (MIDs) for the CLEFT-Q Scales Following Three Cleft-Specific Operations.

The aim of this study was to examine internal responsiveness and estimate minimally important differences (MIDs) for CLEFT-Q scales.In this prospective cohort study, participants completed the CLEFT-Q appearance and health-related quality of life (HRQL) scales before and six months after cleft-related surgery.Seven cleft centres in Canada, USA and UK participated.Patients were ages 8-29 years with CL/P.Patients underwent rhinoplasty, orthognathic or cleft lip scar revision surgery.Internal responsiveness was examined using Cohen's d effect sizes (ESs) based on the following interpretation: 0.20-0.49 small, 0.50-0.79 moderate and ≥ 0.80 large. MIDs were estimated using two distribution-based approaches.Participants had a rhinoplasty (n = 31), orthognathic (n = 21) or cleft lip scar revision (n = 18) surgery. Most participants were males (56%) and aged 8-11 years (41%). Following rhinoplasty, ESs were larger for the nose (0.92, p = 0.001) and nostrils (0.94, p < 0.001) scales than for the face scale (0.51, p = 0.003). MIDs ranged between 6.2-10.4. For orthognathic surgery, larger ES was observed for the jaws scale (1.80, p < 0.001) compared with the teeth (1.16, p < 0.001), face (1.15, p = 0.001) and lips (0.94, p < 0.001) scales. MIDs ranged between 5.9-14.4. In the cleft lip scar revision sample, the largest ES was observed for the nose scale (0.76, p = 0.03), followed by lips (0.58, p = 0.009) and cleft lip scar (0.50, p = 0.043) scales. MIDs ranged between 6.4-12.3.CLEFT-Q detected change in key outcomes for three cleft-specific surgeries, providing evidence of its responsiveness. Estimated MIDs will aid in interpreting this PROM.

Lip symmetry following rotation advancement cleft lip repair in 5-year-old children treated by Ralph Millard and Ron Pigott.

Objective: To compare the symmetry of the lip following Rotation-Advancement cleft lip repair by Millard and Pigott and to investigate the effect on the symmetry of cleft side and gender by using different surgical protocols. Symmetry following cleft surgery was compared to that of non-cleft children. Design: Retrospective study of photographs of children aged 5 years. Setting: Three decades of post-operative photographs of children treated by Millard and Pigott. Patients: Eighty-nine children treated by Millard, 87 by Pigott and 91 non-cleft children. Interventions: Photographs were assessed using the Symnose Computer program, a rapid semi-objective quantitative assessment of lip symmetry. Main Outcome Measures: Asymmetry score for each surgeon, and non-cleft children. Results: There was no significant difference in the median lip % mismatch score of Millard, 36.65% and Pigott, 38.52%. Right-sided clefts showed better symmetry than left-sided clefts for Millard (p<.001). This was reversed for Pigott (P=.0121). There was a difference (P<.001) between the symmetry of the two cleft cohorts and the non-cleft children (asymmetry 19.9%), and between Millard's outcomes following different lip surgical protocols (P < .0001), but no difference between Pigott's outcomes using different palate surgical protocols (P = 0.59). Conclusions: Cleft lip repair by Millard and Pigott resulted in similar lip asymmetry (37% and 39% symmetry mismatch, respectively). Lip surgical protocol and cleft side may affect lip asymmetry. Palate surgery did not affect lip asymmetry. Following cleft surgery, children were more asymmetric than non-cleft children.

Sodium supplementation in neonates with pierre robin sequence significantly improves weight gain if urinary sodium is low.

OBJECTIVE: To investigate whether sodium supplementation increases weight gain in babies with grade 3 Pierre Robin sequence if their urinary sodium is low. DESIGN: Retrospective review of all medical, dietitian, and nursing notes of babies admitted from 2000 to 2007, inclusive. SETTING: Tertiary center for cleft care. PATIENTS: A total of 33 babies requiring nasogastric and nasopharangeal intubation for Pierre Robin sequence were identified. Those for whom weights could not be measured accurately (n  =  4) were excluded from further study. INTERVENTIONS: Urinary sodium was low and weight gain poor in 21 of the remaining 29 babies (72%), and oral sodium supplementation was given to establish and maintain a normal urinary sodium (10 to 20 mmol/L). MAIN OUTCOME MEASURE: Mean daily weight gain. RESULTS: The mean daily weight gain of the 18 babies with low urinary sodium admitted in the first few days of life improved from 20 to 34 g (p < .001) following oral sodium supplementation. This gave a mean difference of 14 g (lower quartile  =  6 g, upper quartile  =  25 g, 95% confidence interval  =  20, 90% confidence interval  =  10). The mean daily weight gain of the eight patients who did not require sodium supplementation was 33.6 g. CONCLUSIONS: Urinary sodium tests are simple and noninvasive and allow monitoring of urinary sodium. If low, adequate supplementation improved weight gain (p < .001), reversing failure to thrive in babies with severe Pierre Robin sequence. These findings have been unreported previously.

Multidisciplinary management of spinal lipoma.

Spinal lipomas (congenital lipomyelomeningoceles) can cause significant aesthetic deformity of the lower spine in addition to recognised neurological problems. Surgical management consists of untethering the cord in symptomatic cases, and debulking of the lipomatous component to improve the spinal contour. However, management remains controversial. Between May 1998 and November 2002, 23 cases of lumbosacral spinal lipoma were operated on by neurosurgeons at our hospital. Of these, eight (34%) patients were subsequently referred to plastic surgery for aesthetic revision, which was performed by a combined team of plastic surgeons and neurosurgeons. In addition, three new patients had their primary surgery as a planned procedure performed by the same combined team. The purpose of this paper is to present the method and results of combined plastic surgery and neurosurgery technique in these 11 cases of spinal lipoma. Follow up ranged between 12 months and 4 years (mean 20 months). One patient had wound break down and three patients developed seroma. Two patients had cerebrospinal fluid (CSF) leakage which required re-exploration and prolonged hospitalisation. The aesthetic results were very satisfactory in 10 out of the 11 patients. The frequency of secondary referral for aesthetic revision, together with the incidence of CSF leak, underlines the importance of managing spinal lipomas in a multidisciplinary team involving neurosurgeons and plastic surgeons. This series demonstrates that aesthetic results are encouraging but decreasing the CSF leakage rate remains a challenge. Further follow up will determine the role of early combined surgery in the management of spinal lipoma.

Home management of upper airway obstruction in Pierre Robin sequence using a nasopharyngeal airway.

OBJECTIVE: This unit has reported management of infants with Pierre Robin Sequence (PRS) and upper airway obstruction using nasopharyngeal airways and nutritional support until enough growth takes place for the infant to thrive. There was a mean hospital stay of 60 days. This long in-patient stay prompted review of our management protocols and consideration of treatment at home. This paper reports our experience of managing infants with PRS at home using a nasopharyngeal airway and nasogastric feeding tube and reviews whether such management reduces in-patient stay while remaining safe and effective. DESIGN: Retrospective review of cases referred over a 3.5-year period. Comparison is made with the unit's previously published results. PATIENTS: Thirteen PRS infants were referred to the West Midlands Regional Cleft service and required transfer to Birmingham Children's Hospital for specialist assessment and airway control. INTERVENTIONS: The parents of 12 infants underwent training to manage the airway and feeding tube. Treatment then continued at home. OUTCOME MEASURES: In-patient episode, rate of weight gain, and complication rate were used. RESULTS: The median hospital stay was 19.5 days compared to 54 days previously. The median rate of weight gain was 34 g/d. There were no complications or readmissions. CONCLUSION: This series demonstrates the revised management protocol followed has reduced in-patient stays and remained effective, with infants continuing to thrive after discharge home, and has a low complication rate.

Congenital desmoplastic trichoepithelioma.

Desmoplastic trichoepithelioma (DT) is a rare benign adnexal neoplasm considered to have follicular differentiation. It usually presents as an asymptomatic, firm, annular plaque with a raised border. The most common site of occurrence is the face, usually on the cheek. Females are more often affected than males and the age range of patients previously reported is 8-79 years. We present a case of congenital desmoplastic trichoepithelioma. A girl was born at term to a healthy mother after an uneventful pregnancy and was noted to have widespread erythematous plaques with milia-like lesions over the right scalp, face and neck, with some areas of atrophic scarring. Histology and immunohistochemistry of incisional biopsies of the lesions were consistent with a diagnosis of DT. To our knowledge, this is the first reported case of congenital DT.

Spontaneous regression of a rare tumour in a child: angiolymphoid hyperplasia with eosinophilia of the hand: case report and review of the literature.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign, idiopathic vasculoproliferative condition manifested by multiple or solitary subcutaneous nodules, usually in the head and neck region of young adults. It is very rare in the extremities especially in the hands. Although recurrence is common, surgical excision is still regarded as the treatment of choice. We report an 11-year-old girl with histologically confirmed ALHE on the dorsum of her left hand, which underwent complete spontaneous regression within 10 weeks. Angiolymphoid hyperplasia is a benign disease and it is important to recognise this condition so that early superficial lesions can be observed for 3-6 months pending spontaneous regression.

EMLA cream on the ears--is it effective? A prospective, randomised controlled trial of the efficacy of topical anaesthetic cream in reducing the pain of local anaesthetic infiltration for prominent ear correction.

A prospective, randomised, double blind trial was carried out to test whether or not the application of topical anaesthetic cream (EMLA cream) before infiltration of local anaesthetic would decrease the discomfort of correction of prominent ears under local anaesthetic. 23 patients were entered into the trial. They acted as their own controls, one ear having EMLA cream applied approximately 2 h prior to surgery, and the other Aqueous cream. Immediately after surgery, the patients were asked to complete two scales describing the amount of pain they had felt in each ear, both at the time of injection of local anaesthetic and during the surgery. They were also asked whether they thought the inconvenience associated with the use of the EMLA cream was worth any improvement they felt. The results showed that, compared to the placebo cream, EMLA cream significantly decreased the pain felt both at the time of injection (p < 0.005) and during the surgery (p < 0.01). However, only 62% of patients asked felt that the inconvenience associated with the use of EMLA cream was worth the benefits that it conveyed.

Rhinoplasty patients revisited.

National Health Service (NHS) rhinoplasty patients have been shown to differ from normal in their performance on psychological tests. In this study the same psychological parameters of two groups of private rhinoplasty patients are documented and compared to that of NHS patients. The two groups of private patients do not differ from each other either in content or in psychological performance. All three groups show consistent differences from normal in their psychological performance. Patients with no history of injury before their rhinoplasty, especially if treated in the NHS, are found to be more psychologically disturbed than other patients. It appears that the NHS is used particularly for treating those with a history of injury, and those with greatest psychological problems.

Postburn breast reconstruction: tissue expansion prior to contracture release.

Tissue expansion of the scarred chest following burns results in a poor breast mound shape with little projection or inframammary fold, since the expander, like normal developing breast tissue, is kept flat by the scarred skin envelope. We present a case that demonstrates that adequate projection of the breast and formation of an inframammary fold can be achieved by expansion if extensive release and skin grafting of contractures over the breast mound are performed after expansion. Maintained expansion will act as a stent reducing secondary contracture of the grafted-areas.

Are rhinoplasty patients potentially mad?

Rhinoplasty patients have long been considered to be psychologically unstable and therefore a "risky" group upon which to operate. Patients who had rhinoplastic operations more than 5 years ago were contacted by post and their psychological health assessed by the use of psychometric tests. The results show no evidence to support earlier suggestions that requests for rhinoplasty may be early symptoms of severe psychiatric disease. However, several points do emerge. Male patients show more symptoms of anxiety and depression than normal, and female patients who give no history of injury preceding their operation behave in a more extrovert and sociable manner than normal. Furthermore, patients of both sexes who give no history of injury before their operation, even though pleased with the operative results, are more self-conscious of their appearance than those who were injured prior to their rhinoplasty.