Palate lengthening by buccinator myomucosal flaps for velopharyngeal insufficiency.

OBJECTIVE : To assess the outcome of palate lengthening by myomucosal buccinator flaps for velopharyngeal insufficiency both in terms of speech and changes in palate length. DESIGN : Thirty-two consecutive patients who underwent the buccinator flap procedure were reviewed retrospectively. Palate length and the presence or absence of a velopharyngeal gap were assessed on pre- and postoperative videofluoroscopic recordings using a calibrated image analysis system. Hypernasality, nasal emission, nasal turbulence, and passive cleft type articulation errors were evaluated blindly by a speech-language pathologist external to the team using pre- and postoperative speech recordings. SETTING : Multidisciplinary cleft team based in a tertiary referral center. Results : In 81% of patients, speech outcome was such that no further velopharyngeal surgery was considered necessary at the time of follow-up. The buccinator flap procedure resulted in a mean palate lengthening of 7.5 mm (±5.5 SD). After the operation, there was a complete elimination of the velopharyngeal gap on lateral videofluoroscopy in 77% of patients. There were significant decreases in hypernasality ratings and passive cleft type articulation errors postoperatively. CONCLUSION: Palatal lengthening with myomucosal buccinator flaps in patients with velopharyngeal insufficiency is effective and safe. It has become one of our routinely practiced procedures for velopharyngeal insufficiency.

Increasing accuracy of antenatal ultrasound diagnosis of cleft lip with or without cleft palate, in cases referred to the North Thames London Region.

OBJECTIVES: To determine the accuracy of antenatal ultrasound diagnosis of cleft lip with or without cleft palate (CL +/- P) and isolated cleft palate (CP). METHODS: This was a retrospective review of 256 surviving cases referred in 2002-2003 for treatment of CL +/- P. RESULTS: We had referrals from 36 maternity units, 27 of which were in our local catchment area. There were 154 cases of CL +/- P, of which five had microform CL. Of the remaining 149, 88 (59%) were diagnosed on antenatal ultrasound examination. Among these 88 cases there were minor reporting errors in 22 (25%). These errors were in describing the side and type of the lip cleft in 10 cases, predicting if there was a CP in 10 cases, and recognizing the anomaly in two cases. There were 102 cases referred with isolated CP, of which 92 had overt CP and 10 submucous CP. None of these was diagnosed by antenatal ultrasound imaging. There was no significant difference in the accuracy of ultrasound diagnosis between district hospitals and teaching/tertiary units. CONCLUSIONS: There is a good awareness and ability to detect CL +/- P by obstetric units from which referrals are received. Inaccuracies in antenatal ultrasound reports occur frequently when attempting to determine the type of CL and when predicting if there is a CP. We recommend that families should continue to be referred to specialist centers for counseling immediately after antenatal diagnosis, and comprehensive advice should always be given about clefts of the lip and palate.

Surgery of the cleft lip and nose--the GOStA approach.

The technique for primary correction of the cleft lip and nose, as practised by The North Thames Cleft Lip and Palate Centre at Great Ormond Street Hospital/St Andrew's Centre (GOStA) has evolved over 28 years of personal experience. It is an amalgamation of techniques developed by many others with some personal modifications. Patients are routinely audited at 5, 10, 15 and 20 years and outcomes evaluated and compared with other centres where possible. Secondary surgery of lip and nose is based on the same principles of anatomical reconstruction used in the primary surgery.

Surgery of the cleft palate: repair using the operating microscope with radical muscle retropositioning--the GostA approach.

The aim of cleft palate surgery is to minimise damage which results in impaired maxillary growth, and to maximise outcome in terms of velopharyngeal function. Techniques developed by the author, building on work by many and employed by the North Thames Cleft Lip and Palate Centre at Great Ormond Street Hospital/St Andrew's Centre (GOStA) appears to offer one way of achieving these aims. However, the learning curve is long.

Long term hand function without long finger extensors: a clinical study.

This study examines the premise that, following severe injuries to the dorsum of the hand, reconstruction of the long extensor tendons of the fingers may not be necessary for many patients. The long-term hand function of nine patients who sustained such injuries and had operations to provide skin cover only, has been comprehensively assessed using objective tests and a detailed questionnaire. It is concluded that provision of skin cover followed by intensive early mobilisation may be an adequate management of these injuries.

Functional deficit following loss of continuity of the long extensors of the fingers: a method of assessment.

A method for assessing hand function following loss of the long finger extensors in Zones 6 and 7 (Verdan, 1968) is described. It relies on a number of mechanical and functional tests combined with a detailed questionnaire.

The open finger technique for the release of syndactyly.

A new technique of syndactyly release is described. The technique differs from the standard methods in that more digital flaps are used, and these are longer and are not defatted. A single stitch is applied to secure the tip of the flap and the defects between the flaps are not closed or grafted, hence the term "open" technique. Eight patients had 12 webs released using this operation. We compare this technique with 12 patients who had a total of 19 webs released using the standard technique. Patients were assessed for six parameters of operative success.

Atypical congenital dermoids of the face: a 25-year experience.

OBJECTIVE: To review the presentation and management of nasal and other facial dermoid cysts and sinuses (excluding external angular dermoids). We report on 28 patients with less common facial dermoids. They presented as a cyst, sinus or both and may be separated into four groups. Group 1 (18 cases) were in the midline on the nose and many (12) had extensions to the septum but only two to the skull base. Group 2 (two cases) were paramedian nasal lesions and one extended through the nasal bones. Group 3 were lesions around the medial orbital wall, and two of these had tracts extending into the orbit. Group 4 were a miscellaneous group of lesions on the cheek and lips. We observed that preoperative imaging, although useful and done mainly for medico-legal reasons, may not detect deep extensions and therefore surgeons should be prepared for a more involved procedure when removing atypical facial dermoids.

A randomized control trial investigating the effect of presurgical orthopedics on feeding in infants with cleft lip and/or palate.

OBJECTIVE: To investigate the controversial assertion that presurgical orthopedics (PSO) facilitate feeding in infants with cleft lip and palate. DESIGN: Randomized control trial of 34 infants with nonsyndromic complete unilateral cleft lip and palate and 16 with cleft of the soft and at least two thirds of the hard palate. Allocation to receive presurgical orthopedics or not used minimization for parity and gender. Other aspects of care were standardized. SETTING: The North Thames Regional Cleft Centre. MAIN OUTCOME MEASURES: Measurements were made at 3 months of age (presurgery) and at 12 months of age (postsurgery). Primary outcomes were anthropometry and oral motor skills. Objective measures of sucking also were collected at 3 months using the Great Ormond Street Measure of Infant Feeding. Twenty-one infants also had videofluoroscopic assessment. RESULTS: At 1 year, all infants had normal oral motor skills and no clear pattern of anthropometric differences emerged. For both cleft groups, infants randomized to presurgical orthopedics were, on average, shorter. The presurgical orthopedics infants were, on average, lighter in the unilateral cleft and lip palate group, but heavier in the isolated cleft palate group. Infants with complete unilateral cleft and lip palate randomized to presurgical orthopedics had lower average body mass index (mean difference PSO-No PSO: -0.45 (95% confidence interval [-1.78, 0.88]), this trend was reversed among infants with isolated cleft palates (mean difference PSO-No PSO: 1.98 [-0.95, 4.91]). None of the differences were statistically significant at either age. CONCLUSIONS: Presurgical orthopedics did not improve feeding efficiency or general body growth within the first year in either group of infants.

The nature of feeding in infants with unrepaired cleft lip and/or palate compared with healthy noncleft infants.

OBJECTIVE: Feeding difficulties are reported widely in infants with cleft lip and/ or palate. There is, however, a paucity of objective information about the feeding patterns of these infants. This study compared patterns of feeding in infants with unrepaired cleft lip and palate with healthy noncleft infants of a similar age. SETTING: North Thames Regional Cleft Centre. The noncleft cohort was recruited from West Middlesex University Hospital, a general hospital with similar demographics. PARTICIPANTS: Fifty newborn infants with nonsyndromic complete unilateral cleft lip and palate or a cleft of the soft and at least two thirds of the hard palate who were referred to the North Thames Regional Cleft Centre participated. Parents of 20 randomly selected, noncleft infants agreed to participate. MAIN OUTCOME MEASURES: Feeding patterns were rated using the Neonatal Oral Motor Assessment Scale. Additional objective information was collected using the Great Ormond Street Measurement of Infant Feeding (Masarei et al., 2001; Masarei, 2003). RESULTS: Infants with nonsyndromic complete unilateral cleft lip and palate or a cleft of the soft and at least two thirds of the hard palate had less efficient sucking patterns than their noncleft peers had. They used shorter sucks (mean difference, 0.30 second; p < .0005), a faster rate of sucking (mean difference, 34.20 sucks/second; p < .0005), higher suck-swallow ratios (mean difference, 1.87 sucks/swallow; p < .0005), and a greater proportion of intraoral positive pressure generation (mean difference, 45.97% positive pressure; p < .0005). CONCLUSIONS: This study demonstrated that the sucking patterns of infants with nonsyndromic complete unilateral cleft lip and palate or a cleft of the soft and at least two thirds of the hard palate differ from those of their noncleft peers.

A long-term follow-up of hypospadias patients.

A review of 60 patients aged 17 years or older, who underwent surgery to hypospadias in childhood, is presented and some impressions and conclusions drawn. Points discussed include psychological effects, micturition, sexual function, fertility, the effects of repeated operations, age of straightening, the site and size of the meatus, and dilatation of the reconstructed urethra. It is suggested that most emphasis should be given to the final result in the adult when hypospadias surgery is being assessed.

Airway obstruction due to a rudimentary premaxilla in holoprosencephaly.

OBJECTIVE: We present two infants with holoprosencephaly and clefts of the primary palate, who developed nasal airway obstruction due to a previously undocumented cause: a ball-valve-like action of a rudimentary premaxilla, which was extremely mobile on a soft tissue stalk. In such patients, the importance of intranasal examination to identify a rudimentary premaxilla, in order to preserve it, and if necessary, surgically stabilize it, is illustrated.

Duplication of the urethral meatus: case report of distal hypospadias and a glandular urethra.

An unusual example of congenital fistula of the distal urethra presenting in a 3-year-old boy is described. The likelihood of this deformity being a variant of hypospadias is explored and theories of the embryology of the anomaly are discussed.