Conservative Airway Management Successful in Majority of Infants With Pierre-Robin Sequence at Queensland Children's Hospital: A Retrospective Review.

PURPOSE: Pierre-Robin Sequence (PRS) is a congenital abnormality characterized by micrognathia, glossoptosis, and variable severity upper airway obstruction. Clear management algorithms are lacking, particularly the indications for surgical versus nonsurgical intervention. The authors reviewed the management of these children in Queensland. METHODS: All children diagnosed with PRS at Queensland Children's Hospital from April 2014 to October 2019 were identified (n=45), and their charts were retrospectively reviewed. Three management patterns emerged: prone/lateral positioning, nasopharyngeal airway (NPA) use, and surgery (tracheostomy or mandibular distraction). RESULTS: Most children (n=30; 67%) were managed successfully nonsurgically with an NPA (median age of insertion 0.25 mo, median duration 5.0 mo). Of these, 12 patients (40%) also required supplemental oxygen. The median age of NPA cessation was 5.5 months, with oxygen therapy ceasing at a median 8.25 months, upon which no further support was required. The remaining majority (n=13; 29%) of children were managed without an NPA, using positioning alone (10/13; 77%) or positioning combined with supplemental oxygen (1/13), CPAP (1/13), or both adjunct measures (1/13). Only 2 patients underwent surgical intervention. Feeding supplementation using nasogastric tube was necessary in 78% of patients for a median duration of 4 months. Cleft palate co-existed in all but one patient. CONCLUSION: Management of upper airway obstruction in PRS children is variable between units. Over a 5-year period, 96% of children with PRS were successfully managed without surgical intervention at the Queensland Children's Hospital. These findings contrast with some other literature and may suggest that more careful consideration of surgical intervention in PRS patients is prudent.

Patient Reported Outcome Following the Skoog Unilateral Cleft Lip Repair among Adults- a Long-Term Cohort Study and Comparison to a Non-cleft Population.

OBJECTIVE: The study primarily aimed to compare satisfaction with lip appearance among adults treated for unilateral cleft lip and palate (UCLP) with Skoog's primary lip repair procedure to those without clefts. The secondary aim was to determine whether satisfaction with lip appearance and the desire to change the lip/face appearance correlated with the number of secondary lip revisions performed. DESIGN: Long-term follow-up. PATIENTS/SETTINGS: All UCLP patients treated at the Uppsala University Hospital born between 1960- and 1987 (n = 109) were invited. At an average of 37 years following the primary lip repair, the participation rate was 76% (n = 83). A control group of adults without cleft (n = 67) completed the same study protocol for comparison. MAIN OUTCOME MEASURES: Satisfaction with appearance was measured with The Satisfaction with Appearance Questionnaire (SWA) and a modified version of the Body Cathexis -Scale was used to assess the desire to change the lip and facial appearance. RESULTS: UCLP patients were less satisfied with their lip, face, and overall appearance and reported a greater desire to change the appearance of their lips and face than non-cleft controls (p < 0.001). Dissatisfaction with lip appearance correlated to a greater willingness to change the appearance of the lip and face. No correlation was found between satisfaction with appearance and the number of the previously performed secondary lip revisions. CONCLUSION: Adults treated for UCLP are less satisfied with the appearance of their lips compared to the non-cleft population. The number of secondary revisions does not necessarily correlate to greater satisfaction with lip appearance.

Three-Dimensional Printed Polycaprolactone Mesh in Pediatric Cranial Vault Remodeling Surgery.

BACKGROUND: The surgical management of craniosynostosis has greatly evolved with improvements in both technology and understanding of the disease process. Some drawbacks remain regarding bone regeneration within the surgical bony gaps. Generally, bony gaps improve in the 12 to 24 months after surgery, but some gaps may remain for longer and cause deformity and/or require additional bony reconstruction. These considerations make tissue-engineered bone very attractive. Novel 3-dimensional printed bioresorbable mesh implants made of Polycaprolactone (PCL) can be used to fill the surgical bony defects. OBJECTIVES: The authors seek to investigate how the use of a 3-dimensional printed biodegradable PCL mesh applied to bony defects in cranial vault surgery affects bone healing. METHODS: Case series analysis of 8 pediatric patients who have undergone surgical intervention using PCL mesh implants for reconstruction of bony defects during craniosynostosis correction surgery. FINDINGS: Radiological evaluation of 3 patients at random time points between 9 and 12 months postoperative revealed persistent bony gaps in areas where PCL mesh was laid. One patient who underwent a subsequent cranial vault surgery at 9 months was found to have less bone regeneration in the defect area where PCL mesh was used when compared with an adjacent area where a particulate bone graft was used. CONCLUSIONS: Based on our experience, the use of PCL mesh on its own did not augment bone regeneration. It is possible that a greater amount of time or increased vascularization of the scaffold is required, which supports the concept of regenerative matching axial vascularization or the further addition of osteogenic factors to increase the rate of bone formation.

Nasal Septal Flaps for Repair of Large or Recurrent Palatal Fistula: Report of Technique and 4-Year Experience.

SUMMARY: Large or multiply recurrent oronasal fistulas following cleft palate repair present a challenging problem. Nasal septal mucoperichondrial flaps have been widely used for repair of skull base defects; however, their use in the repair of oronasal cleft palate fistulas has not previously been described. In this pilot study, the authors describe anterior palatal fistula repair using a nasal septal flap and review their experience with this technique over 4 years. Fourteen patients with anterior palatal fistulas not amenable to repair using local palatal flaps were included for analysis. The mean size of the fistula was 12 mm in maximum dimension. Flap healing with complete or near-complete closure of fistula was achieved in 13 patients (93%). Five of these patients had a small, slit-like residual fistula that was asymptomatic. Nasal septal flaps are a new technique for repair of large or recurrent palatal fistulas. The procedure is well-tolerated with minimal side effects, high success rate, and low incidence of recurrence. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Regenerative matching axial vascularisation of absorbable 3D-printed scaffold for large bone defects: A first in human series.

BACKGROUND: We describe the first clinical series of a novel bone replacement technique based on regenerative matching axial vascularisation (RMAV). This was used in four cases: a tibial defect after treatment of osteomyelitis; a calvarial defect after trauma and failed titanium cranioplasty; a paediatric tibial defect after neoadjuvant chemotherapy and resection of Ewing sarcoma; and a paediatric mandibular deficiency resulting from congenital hemifacial microsomia. METHOD: All patients underwent reconstruction with three-dimensional (3D)-printed medical-grade polycaprolactone and tricalcium phosphate (mPCL-TCP) scaffolds wrapped in vascularised free corticoperiosteal flaps. OUTCOME: Functional volumes of load-sharing regenerate bone have formed in all cases after a moderate duration of follow-up. At 36 cm, case 1 remains the longest segment of load bearing bone ever successfully reconstructed. This technique offers an alternative to existing methods of large volume bone defect reconstruction that may be safe, reliable, and give predictable outcomes in challenging situations. It achieves this by using a bioresorbable scaffold to support and direct the growth of regenerate bone, driven by RMAV. CONCLUSION: This technique may facilitate the reconstruction of bone defects previously thought unreconstructable, reduce the risk of long-term implant-related complications and achieve these outcomes in a hostile environment. These potential benefits must now be formally tested in prospective clinical trials.

Focal Idiopathic Calvarial Thinning: A Condition of Uncertain Prevalence and Significance.

ABSTRACT: Focal thinning of the calvarial bones unrelated to an underlying systemic disease is uncommon. Of such cases documented in the medical literature, most are bilateral parietal bone thinning, which tends to affect elderly females and results in bilateral symmetric, regularly shaped depressions of the skull. The authors describe 2 cases of unilateral, focal, irregularly shaped calvarial thinning in adolescent males that occurred without an obvious precipitating incident and were unrelated to systemic disease, a known syndrome or previous trauma. The nature and physical parameters of the deformities are demonstrated here and specific historic features such as age of onset and mode of obstetric delivery are explored. The clinical significance and potential pathogenesis of this finding is unclear, though these cases are relevant in highlighting a novel presentation that we henceforth term focal idiopathic calvarial thinning.

FACE-Q Craniofacial Module: Part 1 validation of CLEFT-Q scales for use in children and young adults with facial conditions.

BACKGROUND: The CLEFT-Q includes 12 independently functioning scales that measure appearance (face, nose, nostrils, teeth, lips, jaws), health-related quality of life (psychological, social, school, speech distress), and speech function, and an eating/drinking checklist. Previous qualitative research revealed that the CLEFT-Q has content validity in noncleft craniofacial conditions. This study aimed to examine the psychometric performance of the CLEFT-Q in an international sample of patients with a broad range of facial conditions. METHODS: Data were collected between October 2016 and December 2019 from 2132 patients aged 8 to 29 years with noncleft facial conditions. Rasch measurement theory (RMT) analysis was used to examine Differential Item Function (DIF) by comparing the original CLEFT-Q sample and the new FACE-Q craniofacial sample. Reliability and validity of the scales in a combined cleft and craniofacial sample (n=4743) were examined. RESULTS: DIF was found for 23 CLEFT-Q items when the datasets for the two samples were compared. When items with DIF were split by sample, correlations between the original and split person locations showed that DIF had negligible impact on scale scoring (correlations ≥0.995). In the combined sample, RMT analysis led to the retention of original content for ten CLEFT-Q scales, modification of the Teeth scale, and the addition of an Eating/Drinking scale. Data obtained fit with the Rasch model for 11 scales (exception School, p=0.04). Person Separation Index and Cronbach alpha values met the criteria. CONCLUSION: The scales described in this study can be used to measure outcomes in children and young adults with cleft and noncleft craniofacial conditions.

Safety and Efficacy Concerns of Autologous Fat Grafting for Velopharyngeal Insufficiency.

OBJECTIVE: Autologous fat grafting of the velopharynx has been well described for the treatment of velopharyngeal insufficiency (VPI), with most studies purporting it as a technique with low morbidity useful in the treatment of mild VPI. Prompted by 3 cases of obstructive sleep apnea (OSA) following fat grafting of the velopharynx, we undertook a review of the outcomes of this procedure at our unit. DESIGN: Retrospective case series. PARTICIPANTS: All patients who underwent autologous fat grafting for VPI at the Royal Children's Hospital Melbourne. MAIN OUTCOME MEASURES: Preoperative nasendoscopy findings, perceptual speech assessment results, and rates of revisional surgery and complications. RESULTS: Twenty-eight patients were included in the study. Three patients (11%) developed severe OSA requiring removal of the grafted fat. In a subanalysis of cleft patients, there was a reported improvement in hypernasality in 63% though only 25% had complete resolution of their hypernasality. Patients who had an improvement in speech were more likely to have a velopharyngeal gap of less than 0.5 cm2 on preoperative nasendoscopy. All 3 patients who developed OSA had syndromes associated with hypotonia. CONCLUSIONS: This study raises serious concerns over the safety and efficacy of fat grafting for VPI. Overall, there was a relatively high complication rate with generally poor speech results in our series of patients. Stringent criteria should be used to select candidates for fat grafting, namely, a velopharyngeal gap less than 0.5 cm2 and the absence of a syndrome associated with hypotonia.

Technique for Management of Aplasia Cutis Congenita of the Scalp With a Skin Allograft.

Aplasia cutis congenital is a rare condition presenting with localized or widespread congenital absence of skin, most commonly affecting the scalp. Its management remains controversial due to risks associated with both conservative and surgical approaches. The authors report a new technique to manage a large area of aplasia cutis congenital on a newborn's scalp, initially 5.5 cm × 6 cm, with a single skin allograft application. This is a simple procedure, which can be performed on a ward without need for anaesthetic and led to complete epithelialization of the skin defect.

Total care requirements of burn patients: implications for a disaster management plan.

The purpose of this study was to determine the operative and ward-based requirements of burn patients as a first step in the development of a National Health Emergency Multiple Complex Burn Action Plan. A retrospective review of 1043 patients admitted to the National Burn Centre at Middlemore Hospital, Auckland, New Zealand, from June 2006 to June 2009 was undertaken. Outcome measures included the number of operative procedures, operative time, length of inpatient stay, nursing hours, and allied health hours. A mean of 0.3 operating theater visits and 22.8 minutes of operating time was needed per percentage total body surface area (TBSA) burn. Length of inpatient stay equated to 1.1 days per percentage TBSA burn. There was an exponential relationship between operative requirements and burn surface area. Total operating theater time could be predicted from a formula based on burn surface area, mean depth, and type of burn. Operative time required was greatest in the first week and roughly halved each week after this, whereas nursing and allied health hours remained relatively constant. On the basis of operative requirements in the first week, patients with acute burn injuries totaling up to 129% TBSA could be treated at one time at the authors' institution. This study provides an objective trigger point for the activation of a disaster plan and enables us to predict operative and staffing requirements on a week by week basis, taking into account the existing workload. This information can be used to plan both the acute and protracted phase of a national response to a burn disaster.

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.

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.

Clinical and microstructural analysis of patients with hyper-reflective corneal endothelial nuclei imaged by in vivo confocal microscopy.

The purpose of this study was to determine the significance of hyper-reflective corneal endothelial nuclei imaged by in vivo confocal microscopy. A retrospective analysis was performed using a database of 505 patients that had undergone in vivo confocal microscopy of the cornea. All subjects with hyper-reflective endothelial nuclei were identified and these images were analysed to determine corneal endothelial cell density and morphology. The clinical notes of these patients were reviewed and corresponding data regarding corneal thickness was obtained from a related database of Orbscan II pachymetry. Hyper-reflective endothelial nuclei were identified in 41 eyes of 39 (7.7%) patients. Diagnoses included previous cataract surgery or penetrating keratoplasty, posterior polymorphous dystrophy, Fuchs' endothelial dystrophy and irido-corneal endothelial syndrome. No patients with clinically normal corneas exhibited bright endothelial nuclei. The mean endothelial cell density in this group was 1325+/-872 cells mm(-2) and endothelial density was below age-adjusted normal values in 69.2% of patients. Both cellular polymegathism (coefficient of variation of cell area 33.9+/-7.4%) and cellular pleomorphism were noted (51.8+/-9.0% hexagonal cells). The mean central corneal thickness was 582+/-52 microm. There was no significant difference in endothelial density and morphology compared to cases that had low endothelial density but did not exhibit bright nuclei. In conclusion, this study is the first to investigate the significance of bright endothelial nuclei detected by in vivo confocal microscopy. The strong association with corneal disease states suggests that the most likely explanation for this appearance is the alteration in cellular/nuclear morphology, composition or function.

In vivo confocal microstructural analysis of corneal endothelial changes in a patient on long-term chlorpromazine therapy.

BACKGROUND: Deposits in the cornea and lens are a known complication of long-term chlorpromazine therapy. METHOD: A 59-year-old woman had previously taken chlorpromazine for 20 years with doses up to 1,200 mg/day, with a mean dose of 400 mg/day. She presented with gradual onset of blurred vision in her left eye. Slit-lamp biomicroscopy revealed multiple fine creamy-white deposits on her corneal endothelium and anterior crystalline lens capsule bilaterally. RESULTS: In vivo confocal microscopy of the cornea identified irregular hyper-reflective deposits on the posterior surface of the endothelium. The deposits varied from 1 microm to 70 microm in diameter and had well-defined edges. Endothelial morphology was otherwise normal bilaterally. CONCLUSIONS: This is the first report of in vivo confocal imaging of deposits resulting from long-term chlorpromazine use. Microstructural analysis of the corneal endothelium reveals that there were no abnormalities in cellular morphology resulting from these deposits.