SPINA classification of cleft lip and palate: A suggestion for a complement

Spina's classification uses the incisive foramen as an anatomic reference to define groups I, II, and III. In some cases, the morphological manifestation of the cleft arises simultaneously pre- and postforamen, but without communicating. Considering that group I refers to isolated clefts of the primary palate and group III includes isolated clefts of the secondary palate, the authors suggest the classification group IIa for the association of these two occurrences in the same patient, thus associating two classifications. The original structure proposed by Spina is maintained and simply complemented and updated to Spina-A classification.     

Global and communicative development skills in preschool children with cleft lip and palate

Importance::Cleft lip and palate (CLP) is globally among the most common childhood malformations. This disorder impacts childhood development, including speech ...     

The effect of cleft lip and palate, and the timing of lip repair on mother-infant interactions and infant development

Background:  Children with cleft lip and palate are at risk for psychological problems. Difficulties in mother–child interactions may be relevant, and could be affected by the timing of lip repair.
Method:  We assessed cognitive development, behaviour problems, and attachment in 94 infants with cleft lip (with and without cleft palate) and 96 non-affected control infants at 18 months; mother–infant interactions were assessed at two, six and 12 months. Index infants received either 'early', neonatal, lip repair, or 'late' repair (3–4 months).
Results:  Index infants did not differ from controls on measures of behaviour problems or attachment, regardless of timing of lip repair; however, infants having late lip repair performed worse on the Bayley Scales of Mental Development; the cognitive development of early repair infants was not impaired. Difficulties in early mother–infant interactions mediated the effects of late lip repair on infant cognitive outcome.
Conclusions:  Early interaction difficulties between mothers and infants having late repair of cleft lip are associated with poor cognitive functioning at 18 months. Interventions to facilitate mother–infant interactions prior to surgical lip repair should be explored.     

Molecular contribution to cleft palate production in cleft lip mice

Cleft palate following cleft lip may include a developmental disorder during palatogenesis. CL/Fr mice fetuses, which develop cleft lip and palate spontaneously, have less capability for in vivo cell proliferation in palatal mesenchyme compared with CL/Fr normal fetuses. In order to know the changes of signaling molecules contributing to cleft palate morphogenesis following cleft lip, the mRNA expression profiles were compared in palatal shelves oriented vertically (before elevation) in CL/Fr fetuses with or without cleft lip. The changes in mRNA profile of cleft palate morphogenesis were presented in a microarray analysis, and genes were restricted to lists contributing to cleft palate development in CL/Fr fetuses with cleft lip. Four candidate genes (Ywhab, Nek2, Tacc1 and Frk) were linked in a gene network that associates with cell proliferation (cell cycle, MAPK, Wnt and Tgf beta pathways). Quantitative real‐time RT‐PCR highlighted the candidate genes that significantly changed in CL/Fr fetuses with cleft lip (Ywhab, Nek2 and Tacc1). The results of these molecular contributions will provide useful information for a better understanding of palatogenesis in cleft palate following cleft lip. Our data indicated the genetic contribution to cleft palate morphogenesis following cleft lip.     

A multiple malformations syndrome with cleft lip and palate and ankyloblepharon filiforme adnatum

A case of bilateral ankyloblepharon filiforma adnatum with cleft lip and palate, bilateral popliteal pterygia, bilateral complete syndactyly of 2nd and 3rd toes associated with hypoplastic nails, accessory nipple and partially descrended testes is presented. It is a rare case with unusual associations which are all potentially correctable surgically. Ankyloblepharon and cryptophthalmos must be distinguished from each other as syndromic diagnosis is different as also the prognosis for normal vision.     

Simplified method of feeding infants born with cleft palate with or without cleft lip.

Most infants born with a cleft palate with or without cleft lip are undergrown and have histories of difficult feeding. For the past two years, all of the infants born with isolated cleft palate with or without cleft lip, referred to the Boston Floating Cleft Palate Clinic, were fed using a simplified method. A standard nipple that was cross cut and a standard glass baby bottle were used. The infants were fed in the sitting position and burped frequently. Data indicating that nutrition in these infants is adequate as judged by weight gain was provided.     

Feeding considerations for infants with craniofacial malformations

Approximately 5% of children experience difficulty with the complex coordination of sucking, swallowing and breathing required for feeding. Infants with craniofacial malformations may have anatomic and neurologic contributions to feeding problems. Examples include cleft lip and/or palate, micrognathia, maxillary hypoplasia, and pharyngeal dysfunction. Interventions may facilitate weight gain and avoid failure-to-thrive in these infants. An interdisciplinary approach to address feeding challenges in children with craniofacial differences is necessary. Positional changes, latching maneuvers, specialized feeder nipples, squeezable bottles, and cup feeding can be implemented early. Surgical intervention, including gastrostomy tube placement, tongue lip adhesion, mandibular distraction osteogenesis and tracheostomy, may be required in more severe cases.     

Postoperative speech development based on cleft types in children with cleft palate

BACKGROUND: The purpose of this historical cohort study was to compare the speech development of children with three types of cleft palate while controlling for some confounding factors. METHODS: Subjects included 28 children with bilateral cleft lip and palate (BCLP), 74 with unilateral cleft lip and palate (UCLP), 33 with isolated cleft palate (ICP), and 168 normal control children. Children with cleft palate attended a speech clinic in Fukuoka, had attained adequate velopharyngeal function after palatoplasty carried out within 18 months after birth, and had no syndromic diseases, persistent hearing loss, mental retardation, or central nervous system problems. RESULTS: Children with BCLP began using two-word sentences later than other children (3 months, P<0.01). However, the difference based on sex was also statistically significant. Female patients started using two-word sentences earlier than male patients (2 months, P<0.01). Children with BCLP had lower verbal scores on the Wechsler Preschool and Primary Scale of Intelligence (WPPSI) than other children (12.4-14.9 points, P<0.01). There was no significant difference based on sex. CONCLUSIONS: The results of this research indicate the following: speech development of children with ICP and UCLP was similar to that of normal children; however, speech development of children with BCLP was delayed compared with other children with or without cleft palate.     

The effect of cleft lip on socio-emotional functioning in school-aged children

Background:  Children with cleft lip are known to be at raised risk for socio-emotional difficulties, but the nature of these problems and their causes are incompletely understood; longitudinal studies are required that include comprehensive assessment of child functioning, and consideration of developmental mechanisms.
Method:  Children with cleft lip (with and without cleft palate) ( N  =   93) and controls ( N  =   77), previously studied through infancy, were followed up at 7 years, and their socio-emotional functioning assessed using teacher and maternal reports, observations of social interactions, and child social representations (doll play). Direct and moderating effects of infant attachment and current parenting were investigated, as was the role of child communication difficulties and attractiveness.
Results:  Children with clefts had raised rates of teacher-reported social problems, and anxious and withdrawn-depressed behaviour; direct observations and child representations also revealed difficulties in social relationships. Child communication problems largely accounted for these effects, especially in children with cleft palate as well as cleft lip. Insecure attachment contributed to risk in both index and control groups, and a poorer current parenting environment exacerbated the difficulties of those with clefts.
Conclusions:  Children with clefts are at raised risk for socio-emotional difficulties in the school years; clinical interventions should focus on communication problems and supporting parenting; specific interventions around the transition to school may be required. More generally, the findings reflect the importance of communication skills for children's peer relations.     

Management of cleft lip and palate

Clefts of lip and palate are amongst the most common of congenital abnormalities. Although perhaps generally considered rather low on the list of disabling deformities, a more severe cleft (such as complete bilateral cleft lip and palate) can pose a major developmental and psychological challenge to a growing child by potentially affecting appearance, speech, hearing, maxillary growth and dentition. Increasing evidence suggests that the quality of outcome is very dependent on the skill and experience of surgeon, orthodontist, speech therapist and other specialist cleft team members¹.     

非综合征型唇腭裂易感基因位点研究进展

非综合征型唇腭裂是一种常见的出生缺陷,病因复杂,目前普遍认为是遗传因素和环境因素共同作用的结果.先天性唇腭裂易感基因是自全基因组测序以来的研究热点,筛选出的众多候选基因正不断被基因位点多态性检测、病例对照研究、Meta分析等方法验证,但结果迥异.该文就近年来研究较多的非综合征型唇腭裂易感基因以及环境因素与唇腭裂相互关系方面的研究进展展开综述.     

Management of cleft lip and palate

The complex nature of treatment for CL/P, a condition that requires a large multidisciplinary team treating patients from birth to maturity, has been outlined. Subjecting centres' outcomes to audit should precede heeding the current siren calls for paediatricians to refer children exclusively to a particular surgical speciality. A growing body of evidence has shown a close correlation between quality of outcome and the availability of high volume centralised care by dedicated teams, as has been proved and accepted for years in other fields such as surgery in infancy, childhood malignancies, and cystic fibrosis.