Radiographic outcome of secondary alveolar bone grafting in patients with alveolar clefts.

OBJECTIVE: To determine the outcome of secondary alveolar bone grafting (SABG) in a series of consecutive patients with clefts involving the alveolus. DESIGN AND SETTING: Retrospective cohort study of consecutive operations performed between June 2011 and September 2016 by a single surgeon at a single United Kingdom cleft center. PARTICIPANTS: A total of 160 patients with a cleft/s involving the alveolus, inclusive of syndromic patients and those with atypical facial clefts. INTERVENTIONS: A standard protocol involved an oral hygiene program, pre-surgical orthodontics where necessary and autologous bone grafting from the iliac crest. MAIN OUTCOME MEASURE(S): The Kindelan bone-fill index was used to evaluate success using occlusal radiographs. Weighted Cohen's kappa coefficient was used as a measure of intra- and inter-rater agreement. Fisher's exact test was used to examine the effects of type of cleft, pre-surgical orthodontics or age at time of SABG on radiographic outcome. RESULTS: There were 200 SABGs assessed. Mean age at time of SABG was 9.1 years old (SD 1.1) with 99% (n = 198) of grafts deemed successful. There were two failures where re-graft was performed successfully during the study period. A grade 1 outcome was achieved for 92.5% (n = 185) of grafts and this did not appear to be affected by type of cleft (P = .290), pre-surgical orthodontics (P = .380) or age at time of SABG (P = .081). CONCLUSIONS: The high success rate reported in this study supports the favorable outcomes of a high-volume cleft surgeon. These findings can be used for comparative audit with similar units providing cleft care.

A Closer Look at Delayed Primary Cleft Surgery and Unrepaired Cleft Lip and/or Palate in 5 UK Cleft Centers.

BACKGROUND: There may be many reasons for delays to primary cleft surgery. Our aim was to investigate the age of children undergoing primary cleft lip or primary cleft palate repair in 5 cleft centers within the United Kingdom. Identify the reasons for delayed primary cleft lip repair (beyond 6 months) and delayed primary palate repair (beyond 13 months). Identify children who had a cleft lip and/or palate (CL±P) that was intentionally unrepaired and the reasons for this. METHODS: A retrospective, multicenter review of patients born with a CL±P between December 1, 2012, and December 31, 2016. Three regional cleft centers, comprising of 5 cleft administrative units in the United Kingdom participated. RESULTS: In all, 1826 patients with CL±P were identified. Of them, 120 patients had delayed lip repair, outside the expected standard of 183 days. And, 178 patients in total had delayed palate repair, outside the expected standard of 396 days. Twenty (1%) patients had an unrepaired cleft palate. CONCLUSIONS: This large retrospective review highlights variations between centers regarding the timing of lip and palate surgery and details the reasons stated for delayed primary surgery. A small number of patients with an unrepaired cleft palate were identified. All had complex medical problems or comorbidities listed as a reason for the decision not to operate and 50% had a syndromic diagnosis. The number of patients receiving delayed surgery due to comorbidities, being underweight or prematurity, highlights the importance of the cleft specialist nurse and pediatrician within the cleft multidisciplinary team.

The Cleft Clinic Passport-Improving the Patient Experience.

Challenges in patient and public understanding of the cleft multidisciplinary team (MDT) clinic were highlighted in the Cleft Care UK report in 2012. An innovative personalized visual guide was designed to improve understanding of cleft MDT clinic and encourage child engagement at the Royal Manchester Children's Hospital. We demonstrate the use of Public and Patient Involvement to produce this novel visual educational tool in the Cleft MDT setting, which can form part of a personal health record.

Erythema Multiforme Major in a Dog Treated with Intravenous Human Immunoglobulin and Immunosuppressive Therapy.

An ∼12 yr old castrated mixed-breed dog was evaluated for a 7 wk progressive history of intermittent hyporexia, lethargy, and erosive dermatitis. Initial examination revealed disseminated papules and macules coalescing to irregularly shaped and serpiginous plaques with widespread erosion progressing to ulceration. Skin histopathology revealed transepidermal keratinocyte apoptosis with lymphocyte satellitosis and lymphocytic and histiocytic interface infiltrate. Histopathology combined with clinical signs and history were compatible with the diagnosis of erythema multiforme major. Treatment was initiated with multidrug immunosuppression. Following 36 hr with no improvement, intravenous human immunoglobulin (0.45 mg/kg IV) was administered resulting in notable improvement in the dog's attitude and appetite within 2 hr and the dog's skin lesions within 48 hr. Following discharge, the dog improved daily with near complete resolution of dermatologic disease achieved 1 mo postdischarge. All immunosuppressive medications were ultimately discontinued 5 mo following presentation. This is the first report of a dog with erythema multiforme major that has been successfully treated with a combination of intravenous immunoglobulin and immunosuppression.

Genitourinary malformations: an under-recognized feature of ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome.

The ectodermal dysplasia and cleft lip/palate (EEC) syndrome describes the association of ectrodactyly, ectodermal dysplasia and orofacial clefting. As with many autosomal dominant disorders, there is variability in expression and not all of these three core features are present in every individual with the condition. Moreover, there may be additional associated features, which are under-recognized. One of these is the presence of genitourinary anomalies, some of which cause significant morbidity. This report details a further two patients with EEC syndrome and genitourinary involvement, including flaccid megacystis with detrusor muscle failure, bilateral hydronephrosis and megaureter, requiring significant renal and urological involvement during their childhood. We go on to review the literature on the diagnosis and management of genitourinary malformations in EEC syndrome.

Alveolar Bone Grafting: Donor Site Review of 100 Consecutive Cases in Cleft Lip and Palate.

OBJECTIVE: Review of patients who underwent secondary alveolar bone grafting for total inpatient stay, postoperative complications, and postoperative analgesic requirements. DESIGN: Retrospective analysis of medical records. SETTING: Tertiary care center as part of a regional cleft lip and palate network. PATIENTS: All patients who underwent secondary alveolar bone grafting from the iliac crest. INTERVENTIONS: Local anesthetic was infiltrated overlying the anterior iliac crest. An incision was made to conform to the future skin crease and avoid muscle dissection. The cartilaginous cap was incised and raised, and cancellous bone was then harvested. The cavity was packed with hemostatic cellulose and closed in layers. All patients received postoperative antibiotics. All patients were prescribed regular paracetamol (acetaminophen) and ibuprofen if there were no contraindications. Oral morphine was available when requested. MAIN OUTCOME MEASURES: Length of stay, postoperative analgesic requirements, and postoperative donor site and oral complications. RESULTS: From 100 consecutive patients, 92 (92%) of the patients were discharged the day after surgery; one (1%) patient required four nights of monitoring for postoperative pyrexia of unknown origin. All patients received regular paracetamol, and the majority (86%) did not require oral morphine. Complications included seroma (4%), superficial donor site abscess (1%), postoperative pyrexia of unknown origin (2%), gingival bleeding (2%), and oral infection (2%). CONCLUSION: The findings suggest that donor site pain may be well controlled with simple, regular analgesia. Children tolerated this procedure well and were safely discharged the day after surgery. Alveolar bone grafting from the iliac crest was found to have low complication rates.

Fistula Rate after primary palatal repair with intravelarveloplasty: a retrospective three-year audit of six units (NorCleft) in the UK.

Our aim was to evaluate the rate of fistulation after one-stage palatal repair by intravelarveloplasty in the NorCleft Cleft Services (Scotland and Northern England), this being a primary outcome measure after repair of a cleft palate. We designed a retrospective, three-year clinical audit of six cleft units in the UK, and retrospectively reviewed the casenotes of babies with cleft palate born in 2006-2008 who were treated by intravelarveloplasty. We recorded type of cleft and procedure, including lateral relieving incisions, and our main outcome measure was the presence of a fistula behind the incisive foramen at 3 years of age, or a history of repair of a fistula. A total of 743 patients had cleft palates, but 69 (9%) were excluded (because they had not been operated on, or had not been reviewed by the age of 3 years, or their records were unavailable). A total of 626 patients had had a Sommerlad intravelarveloplasty repair, and 48 had had mixed procedures including Veau-Wardill-Kilner, Furlow, or two-stage repairs, and were not studied further. Eighty-seven (14%) who had had intravelarvoloplasty had a fistula behind the incisive foramen. There was no significant difference in age at time of repair between those who developed a fistula and those who did not (p=0.65). The fistula rate of 14% is comparable with that of Sommerlad. The fistula rate was higher in patients who had had lateral releasing incisions (58/275, 21%) or who had bilateral cleft palate (16/63, 25%). To our knowledge this is the largest review of the fistula rate in patients who had primary palatal repair using the intravelarveloplasty technique in the UK, and shows significant correlation between lateral releasing incisions and formation of a fistula, except in the unilateral cleft lip and palate group (p=0.12).