Bilateral reverse U incisions and separately placed columella relaxation incision for correction of bilateral cleft lip nose deformity

The reverse U incision method has been employed for the correction of the unilateral cleft lip nose deformity for more than twenty years with satisfactory results. It has some columella lengthening effect and thus has proved to be useful for the correction of nasal deformities of bilateral cleft lip. To augment its columella lengthening effect a columella relaxation incision is added to the bilateral reverse U incision for the correction of the bilateral cleft lip nose deformity. This results in a reconstructed columella of satisfactory length and width.     

The rhomboid flap for pilonidal disease

Introduction There have been many surgical techniques described for the treatment of pilonidal sinuses. Recurrent disease causes significant morbidity particularly with time from work. Aim To assess the rhomboid flap's role in promoting one‐stage primary healing in pilonidal disease and to evaluate the morbidity and recurrence. Methods Fifty‐three patients were prospectively recruited of which 27 had previous multiple abscess formation requiring surgical drainage from their pilonidal disease, although none had acute disease at the time of surgery. By using the transposition flap, we were able to obliterate the natal cleft and therefore the rolling action of the buttocks between the cleft in these patients and thereby remove one of the factors involved in pilonidal disease. Hospital stay, healing time, wound infection, wound breakdown and recurrence were noted. Results There were 47 males and 6 females with a median age of 28 years (range 16–64 years). Median follow‐up was 24 months (range 3–36 months). Post‐operative morbidity involved superficial wound infection in 7 (13%) which settled with out‐patient dressings. There were four recurrences (7%), two occurred between the flap and the anal canal, and the other two in the flap margin needing intervention. All the patients healed their wounds and the median healing time was 14 days. Conclusion As this condition affects a predominantly young population causing significant time off from work, we feel that the Rhomboid Flap is useful for difficult cases in that it allows early return to full activity and does not necessitate prolonged postoperative care.     

Autosomal recessive blepharoptosis,cleft lip/palate,dental anomalies,and ectrodactyly

Fluorescence in situ hybridization (FISH) with α-satellite DNA probes was used to study whole-arm chromosome translocation products in a family in which the propositus was shown to have a monosomy 18p/trisomy 20p imbalance. By this approach, we show that the chromosome 18 α-satellite DNA block is split into 2 smaller units, whereas the chromosome 20 breakpoint is not included within the α-satellite DNA region. We found no evidence to suggest that this split α-satellite DNA region has reduced or impaired the function of the centromere or that it contributed to the phenotype of the propositus. The FISH technique critically demonstrated the involvement of a whole-arm translocation in this case and provided accurate identification of breakpoints, which was not possible with standard banding techniques. © Wiley-Liss, Inc.     

Early parental adjustment to visible congenital disfigurement

Summary When a baby is born with a visible disfigurement, then parents need to adjust to the loss of the anticipated 'perfect' child and thus accept their baby. The impact of the birth on the parents is described in the context of a measure which identifies areas of potential difficulty. The two groups studied were parents of children with cleft palates and parents of children with congenital hand deficit. A wide range of adjustment was found. There was no significant difference between the two groups in terms of their overall adjustment, but there were individual differences in adjustment which did not relate to the severity or type of anomaly. The only significant variable found to relate to parental adjustment was perceived family support.     

腭裂儿童三位一体语音训练模式的构建

目的：评价家庭语音训练模式对腭裂术后患者语音康复的干预效果。方法：对16例患儿运用家庭-学校-医院三位一体的训练模式进行为期1年的家庭语音训练，通过语音清晰度评价疗效。结果：患儿语音清晰度平均提高36．06％，代偿性发音等腭裂音质明显改善。结论：家庭-学校-医院三位一体的训练模式对于校正腭裂术后患者的不良代偿性发音习惯有良好的效果。     

鼓室穿刺治疗腭裂渗出性中耳炎的临床评价

目的　探讨腭裂修复同期 ,鼓室穿刺对腭裂伴分泌性中耳炎患者中耳功能及听力损失的影响。方法　将 80例 ( 14 6耳 )患有分泌性中耳炎的腭裂患者 ,随机分为实验组 ( 78耳 ) :常规腭裂修复术 ,术毕作鼓室穿刺。对照组 ( 68耳 ) :单纯腭裂修复术。术后 6个月两组均进行鼓室图、脑干听觉诱发电位检查 ,并进行比较。结果　实验组术后无论与术前比较 ,还是与对照组术后比较 ,鼓室图、V波反应阈值及轻、中度听力损失 ,均有显著进步 (P <0 0 1) ;术后半年内实验组听力恢复早 ,而且治愈率高 ( 96 2 % ,75 /78) ,明显优于对照组 ( 3 8 2 % ,2 6/68)。结论　腭裂修复同期鼓室穿刺 ,不仅有助于改善伴有分泌性中耳炎腭裂患者的中耳功能 ,提高听力 ;而且可避免或减少粘连性中耳炎的发生     

The Van der Woude syndrome: a case report and review of the literature

The Van der Woude syndrome is a rare autosomal dominant developmental malformation usually associated with bilateral lower lip pits. These congenital lip pits appear clinically as a malformation in the vermilion border of the lip, with or without excretion. As a genetic defect has been identified as a microdeletion of chromosome bands 1q32-q41, genetic counselling of patients may be considered. A nonsense mutation in the interferon regulatory factor-6 (IRF-6) is discussed as a pathogenic relevant factor. Therapeutic intervention is generally not necessary, although surgical excision is especially indicated in patients with recurrent inflammation. Physicians should be aware of the Van der Woude syndrome because it has been reported to be associated with a variety of malformations or other congenital disorders.     

Anaesthesia for primary repair of cleft lip and cleft palate: a review of 244 procedures

The peri-operative course of 194 patients undergoing 244 procedures for primary repair of cleft lip and palate over an 8-year-period was studied. A marked increase in the extent of intra-operative monitoring was noted during this period. The Pierre Robin syndrome was the most common associated abnormality and was found in 17% of patients in this series. There were no deaths. A total of 101 procedures were undertaken in infants with cleft lip of whom 10% received an intra-operative blood transfusion. Post-operative opiate analgesics were administered following 97% of these procedures and profound respiratory depression was observed in three children. The use of lignocaine and noradrenaline did not significantly reduce the operative blood loss. A post-operative pyrexial illness was significantly associated with the presence of a positive pre-operative nasal and throat swab and this could be significantly reduced by pre-operative antibiotic treatment. A total of 143 children underwent repair of cleft palate and of these 16.8% received an operative blood transfusion. An elective tracheostomy was required in one patient because of unsuccessful attempts at endotracheal intubation. One patient developed a respiratory arrest after two doses of diamorphine peri-operatively. The use of lignocaine and noradrenaline significantly reduced the operative blood loss. The presence of a positive bacteriological nose or throat swab did not influence the development of a post-operative pyrexia which could however be significantly reduced by the use of pre-operative antibiotics.     

The abnormal anatomy of cleft lip and its correction

Summary The importance of the premaxillary position, size and shape in the development of the face and nose is discussed. The anthropological development of the premaxilla is studied. These factors are to be considered when cleft lip repairs are being planned and developed. Several cases are presented and discussed.