Maternal Representations and Parenting Style in Children Born With and Without an Orofacial Cleft.

OBJECTIVE: To investigate the evolution of maternal representations (ie, the way parents perceive their child in term of temperament, character, behaviors, etc) of children with a cleft at 3 major milestones: before/after reconstructive surgeries and at school age. Parenting style was also analyzed and compared with parents of children born without a cleft. DESIGN AND PARTICIPANTS: The sample was composed of 30 mothers of children with an orofacial cleft and 14 mothers of children without a cleft. Maternal representations were assessed when the child was 2 months (before surgery), 12 months (after surgery), and 5 years of age (when starting school) using semistructured interviews that were transcribed and coded according to the subscales of the Working Model of the Child Interview and the Parental Development Interview. At the 5-year appointment, mothers also completed a questionnaire about parenting style. RESULTS: Results showed no difference across groups (cleft/noncleft) in maternal representations at the 2-month, 12-month, and 5-year assessments. In the cleft group, significant differences were shown between 2 and 12 months in caregiving sensitivity, perceived infant difficulty, fear for the infant's safety, and parental pride, all factors being higher at 12 months. Those differences in parental representations over time were not found in the noncleft group. Additionally, mothers of the cleft group were significantly more authoritarian than mothers of children without a cleft. CONCLUSION: The absence of differences across cleft and noncleft groups suggests that having a child with a cleft does not affect maternal representations and emotions between 2 months and 5 years of the child's age. However, parenting style seems to be influenced by the presence of a cleft in the present sample.

ATTACHMENT IN INFANTS WITH CLEFT LIP AND/OR PALATE: MARGINAL SECURITY AND ITS CHANGES OVER TIME.

This study examines the attachment quality and how this changed over time among infants who had cleft lip and palate (CLP), by conducting a prospective longitudinal study addressing the effects of this type of perinatal event on the parent-infant relationship and the emotional development of the infants. At 12 months of age, the Strange Situation Paradigm (SSP; M. Ainsworth, M.C. Blehar, E. Waters, & T. Wall, 1978) was administered to a sample of 38 CLP infants (born between 2003 and 2010) and 17 healthy controls. At 4 years of age, the Attachment Story Completion Task (ASCT; I. Bretherton, D. Ridgeway, & J. Cassidy, 1990) was administered to 32 individuals from the CLP sample and 14 from the control group. As reported in the literature, CLP infants display secure attachment behaviors as frequently as do control infants (55%). However, a more detailed analysis of the attachment scales revealed that CLP infants show more avoidance and less proximity seeking. In addition, a closer examination of the subcategories of attachment styles revealed that most CLP infants (71%) displayed distal attachment strategies such as the B1/B2 or A1/A2 subcategories. At 4 years old, CLP infants clearly displayed more deactivation and less security than did the control sample. Moreover, when detailing the evolution of attachment individually, almost 60% of the CLP children showing distal strategies at 12 months became deactivated or disorganized when they reached 4 years. Indeed, subtle differences in attachment behaviors at 12 months old-which can be considered marginally secure at that age-may reveal attachment vulnerabilities, which seem to be more apparent over the course of development.

Rhinopharyngeal autologous fat injection for treatment of velopharyngeal insufficiency in patients with cleft palate.

Patients with cleft palate are prone to velopharyngeal insufficiency. In minor cases or when hypernasal speech does not resolve after velopharyngoplasty, an augmentation pharyngoplasty with autologous fat can be proposed. The aim of the present study is to evaluate the short-term (within 2 months) and long-term efficiency (during the 24 months following the procedure) of our procedure in the setting of velopharyngeal insufficiency related to a cleft palate. Twenty-two patients with cleft palate related velopharyngeal insufficiency were included in this retrospective study. All patients were operated following the same technique, in the same institution. The pre- and postoperative evaluations included a nasometry, a subjective evaluation using the Borel-Maisonny score, and a nasofibroscopy to assess the degree of velopharyngeal closure. Scores of Borel-Maisonny and nasometry were compared before, shortly after the procedure (within 2 months) and long term after the procedure (within 24 months). Forty-one procedures in 22 patients with a cleft palate performed in our institution between October 2004 and January 2012 were included in the study. Nine patients had a previous velopharyngoplasty with persistent rhinolalia despite intensive speech therapy. In 14 patients the procedure was repeated because of recurrent hypernasal speech after the first injection. The average number of procedures per patient was 1.8. Postoperative nasometry and Borel-Maisonny scores were statistically significantly improved and remained stable until the end of the follow-up (median 42 months postoperative) in most patients. Complications were rare and minor. Autologous fat injection is a simple procedure for treatment of minor velopharyngeal insufficiencies in patients with cleft palate, with good long-term results and few complications.

[Vascular anomalies]. / Anomalies vasculaires: exemple du syndrome de Klippel-Trenaunay.

Vascular anomalies are rare conditions that could be observed at all ages. They are classified, according to their histology, in vascular tumors or vascular malformations. The general practitioner plays a significant role in diagnosis and patient management, diagnosis being suspected on clinical history. In case of vascular anomaly, ultrasound-Doppler assessment is helpful to characterize morphologic and hemodynamic changes of the lesion and permits to monitor the evolution and to detect complications. Further investigations are often necessary prior to multidisciplinary management. In this article, a brief overview of vascular anomalies, their multidisciplinary management and the exemple of Klippel-Trenaunay syndrome are presented.

Factors influencing maternal mental health after the birth of a child with a cleft in benin and in Switzerland.

OBJECTIVE: The main objective of the study is to identify practical and cultural factors influencing the mental health of mothers of children with an orofacial cleft in Benin and to compare it with a sample of Swiss mothers in the same conditions. METHOD: Thirty-six mothers of children with an orofacial cleft in Benin and 40 mothers of children with an orofacial cleft in Switzerland were interviewed about practical and emotional aspects concerning their child and their own lives. Then, they completed the Perinatal Postraumatic Stress Questionnaire and the Beck Depression Inventory. RESULTS: Mothers in Benin had significantly higher posttraumatic stress and depression symptoms compared with mothers in Switzerland. Depression symptoms were higher in Beninese mothers coming from urban areas, in Beninese mothers with few or no other children, and in Beninese mothers whose child was operated on at a more advanced age. DISCUSSION: This study stressed the importance of cultural differences in perceptions of orofacial clefts in order to provide appropriate care to patients and their families. In particular, wide campaigns of information should help parents to understand the cleft origin and the medical staff in small dispensaries to provide adequate support and care. This may diminish anxiety concerning the child's short- and long-term prognosis. Creation of a Beninese parental support group for children with clefts and their families could be another way to provide information and support where multidisciplinary care is not available.

Early mother-child interaction and later quality of attachment in infants with an orofacial cleft compared to infants without cleft.

Objective : The main objective of this study was to assess mother-child patterns of interaction in relation to later quality of attachment in a group of children with an orofacial cleft compared with children without cleft. Design : Families were contacted when the child was 2 months old for a direct assessment of mother-child interaction and then at 12 months for a direct assessment of the child's attachment. Data concerning socioeconomical information and posttraumatic stress symptoms in mothers were collected at the first appointment. Participants : Forty families of children with a cleft and 45 families of children without cleft were included in the study. Families were recruited at birth in the University Hospital of Lausanne. Results : Results showed that children with a cleft were more difficult and less cooperative during interaction at 2 months of age with their mother compared with children without a cleft. No significant differences were found in mothers or in dyadic interactive styles. Concerning the child's attachment at 12 months old, no differences were found in attachment security. However, secure children with a cleft were significantly more avoidant with their mother during the reunion episodes than secure children without cleft. Conclusion : Despite the facial disfigurement and the stress engendered by treatment during the first months of the infant's life, children with cleft and their mothers are doing as well as families without cleft with regard to the mothers' mental health, mother-child relationships, and later quality of attachment. A potential contribution for this absence of difference may be the pluridisciplinary support that families of children with cleft benefit from in Lausanne.

Early Mother-Child Interaction and Later Quality of Attachment in Infants With an Orofacial Cleft Compared to Infants Without Cleft.

Objective : The main objective of this study was to assess mother-child patterns of interaction in relation to later quality of attachment in a group of children with an orofacial cleft compared with children without cleft. Design : Families were contacted when the child was 2 months old for a direct assessment of mother-child interaction and then at 12 months for a direct assessment of the child's attachment. Data concerning socioeconomical information and posttraumatic stress symptoms in mothers were collected at the first appointment. Participants : Forty families of children with a cleft and 45 families of children without cleft were included in the study. Families were recruited at birth in the University Hospital of Lausanne. Results : Results showed that children with a cleft were more difficult and less cooperative during interaction at 2 months of age with their mother compared with children without a cleft. No significant differences were found in mothers or in dyadic interactive styles. Concerning the child's attachment at 12 months old, no differences were found in attachment security. However, secure children with a cleft were significantly more avoidant with their mother during the reunion episodes than secure children without cleft. Conclusion : Despite the facial disfigurement and the stress engendered by treatment during the first months of the infant's life, children with cleft and their mothers are doing as well as families without cleft with regard to the mothers' mental health, mother-child relationships, and later quality of attachment. A potential contribution for this absence of difference may be the pluridisciplinary support that families of children with cleft benefit from in Lausanne.

Success and failure for children born with facial clefts in Africa: a 15-year follow-up.

BACKGROUND: This study reviews the 15 year program of our Department of Pediatric Surgery for the treatment and follow-up of children born with a cleft in Benin and Togo. METHODS: We analyzed files of children born in Africa with a cleft. They were referred to us through a nongovernmental organization (NGO) between 1993 and 2008 and assessed in Africa by local pediatricians before and after surgery. Operations were performed by our team. RESULTS: Two hundred files were reviewed: 60 cases of unilateral cleft lip, seven of bilateral cleft lip, 44 of unilateral cleft lip palate (UCLP), 29 of bilateral cleft lip palate (BCLP), 53 of cleft palate (CP), three of bilateral oro-ocular cleft, one of unilateral and two of median clefts (Binder), and one of commissural cleft. Sixty-nine (35 %) of these cases were not operated in Africa: 25 (12.5 %) had not shown up, 28 (15 %) were considered unfit for surgery (Down's syndrome, HIV-positive, malnutrition, cardiac malformation), and 16 (7.5 %) were transferred to Switzerland. Palatal fistula occurred in 20 % of UCLP, 30 % of BCLP, and 16 % of CP. Evaluation of speech after palate surgery gave less than 50 % of socially acceptable speech. CONCLUSIONS: Our partnership with a NGO and a local team makes it possible to treat and subsequently follow children born with a cleft in West Africa. Surgery is performed under good conditions. If aesthetic results are a success, functional results after palate surgery need further improvement to promote integration in school and social life.

Impact of a cleft lip and/or palate on maternal stress and attachment representations.

OBJECTIVE: The announcement, prenatally or at birth, of a cleft lip and/or palate represents a challenge for the parents. The purpose of this study is to identify parental working internal models of the child (parental representations of the child and relationship in the context of attachment theory) and posttraumatic stress disorder symptoms in mothers of infants born with a cleft. METHOD: The study compares mothers with a child born with a cleft (n  =  22) and mothers with a healthy infant (n  =  36). RESULTS: The study shows that mothers of infants with a cleft more often experience insecure parental working internal models of the child and more posttraumatic stress symptoms than mothers of the control group. It is interesting that the severity or complexity of the cleft is not related to parental representations and posttraumatic stress disorder symptoms. The maternal emotional involvement, as expressed in maternal attachment representations, is higher in mothers of children with a cleft who had especially high posttraumatic stress disorder symptoms, as compared with mothers of children with a cleft having fewer posttraumatic stress disorder symptoms. DISCUSSION: Mothers of children with a cleft may benefit from supportive therapy regarding parent-child attachment, even when they express low posttraumatic stress disorder symptoms.

Treatment of velopharyngeal insufficiency by autologous fat injection.

Velopharyngeal insufficiency (VPI) is a structural or functional trouble, which causes hypernasal speech. Velopharyngeal flaps, speech therapy and augmentation pharyngoplasty, using different implants, have all been used to address this trouble. We hereby present our results following rhinopharyngeal autologous fat injection in 18 patients with mild velopharyngeal insufficiency (12 soft palate clefts, 4 functional VPI, 2 myopathy). 28 injections were carried out between 2004 and 2007. The degree of hypernasal speech was evaluated pre- and postoperatively by a speech therapist and an ENT specialist and quantified by an acoustic nasometry (Kay Elemetrics). All patients were exhaustively treated with preoperative speech therapy (average, 8 years). The mean value of the nasalance score was 37% preoperatively and 23% postoperatively (p = 0.015). The hypernasality was reduced postoperatively in all patients (1-3 degrees of the Borel-Maisonny score). There were no major complications, two minor complications (one hematoma, one cervical pain). The autologous fat injection is a simple, safe, minimally invasive procedure. It proves to be efficient in cases of mild velopharyngeal insufficiency or after a suboptimal velopharyngoplasty.

Influence of the primary cleft palate closure on the future need for orthognathic surgery in unilateral cleft lip and palate patients.

The aim of the study was to determine the influence of the dissection of the palate during primary surgery and the type of orthognathic surgery needed in cases of unilateral total cleft. The review concerns 58 children born with a complete unilateral cleft lip and palate and treated between 1994 and 2008 at the appropriate age for orthognathic surgery. This is a retrospective mixed-longitudinal study. Patients with syndromes or associated anomalies were excluded. All children were treated by the same orthodontist and by the same surgical team. Children are divided into 2 groups: the first group includes children who had conventional primary cleft palate repair during their first year of life, with extensive mucoperiosteal undermining. The second group includes children operated on according to the Malek surgical protocol. The soft palate is closed at the age of 3 months, and the hard palate at 6 months with minimal mucoperiosteal undermining. Lateral cephalograms at ages 9 and 16 years and surgical records were compared. The need for orthognathic surgery was more frequent in the first than in the second group (60% vs 47.8%). Concerning the type of orthognathic surgery performed, 2- or 3-piece Le Fort I or bimaxillary osteotomies were also less required in the first group. Palate surgery following the Malek procedure results in an improved and simplified craniofacial outcome. With a minimal undermining of palatal mucosa, we managed to reduce the amount of patients who required an orthognathic procedure. When this procedure was indicated, the surgical intervention was also greatly simplified.

Targeting burn prevention in the paediatric population : a prospective study of children's burns in the Lausanne area.

QUESTION UNDER STUDY: Domestic accidents are an important problem in paediatric medicine. This study was designed to gain a better understanding of burn mechanisms and target prevention. METHODS: Children treated for burn lesions in the Department of Paediatric Surgery between August 2004 and August 2005 were included in this prospective study. The burn mechanisms, the children's ages and the circumstances in which children were burned as well as their home environment variables were analyzed. RESULTS: The current study included eighty-nine patients, aged between 2 months and 15 years. Seventy-eight percent were less than 5 years old. More than half were boys. Hot liquid scalding was the most frequent mechanism. There does not seem to be an increased risk in the immigrant population or in low economic status families. In most cases, an adult person was present at time of injury. CONCLUSIONS: If we were to describe the highest "at risk" candidate for a burn in our region, it would be a boy aged 15 months to 5 years who is burned by a cup of hot liquid on his hand, at home, around mealtime, in the presence of one or both parents. Reduced attention in the safe domestic setting is probably responsible.

Biliary atresia: Swiss national study, 1994-2004.

OBJECTIVES: To determine the epidemiology of biliary atresia (BA) in Switzerland, the outcome of the children from diagnosis, and the prognostic factors. PATIENTS AND METHODS: The records of all patients with BA born in Switzerland between January 1994 and December 2004 were analyzed. Survival rates were calculated with the Kaplan-Meier method, and prognostic factors evaluated with the log rank test. Median follow up was 58 months (range, 5-124). RESULTS: BA was diagnosed in 48 children. Incidence was 1 in 17,800 live births (95% confidence interval 1/13,900-1/24,800), without significant regional, annual, or seasonal variation. Forty-three children underwent a Kasai portoenterostomy (PE) in 5 different Swiss pediatric surgery units. Median age at Kasai PE was 68 days (range, 30-126). Four-year survival with native liver after Kasai PE was 37.4%. Liver transplantation (LT) was needed in 31 in 48 children with BA, including 5 patients without previous Kasai PE. Four patients (8%, all born before 2001) died while waiting for LT, and 29 LT were performed in 27 patients (28 in Geneva and 1 in Paris). All of the transplanted patients are alive. Four-year overall BA patient survival was 91.7%. Four-year survival with native liver was 75% in patients who underwent Kasai PE before 46 days, 33% in patients operated on between 46 and 75 days, and 11% in patients operated on after 75 days (P = 0.02). CONCLUSIONS: Overall survival of patients with BA in Switzerland compares favorably with current international standards, whereas results of the Kasai operation could be improved to reduce the need for LTs in infancy and early childhood.

Tissue engineered fetal skin constructs for paediatric burns.

Autologous skin-grafting is the gold standard for treatment of deep second and third degree burns. Available bioengineered skin products also necessitate this two-step surgical procedure. Therefore, we developed fetal skin constructs to improve healing of such degree burns. A bank of fetal skin cells was developed from one organ donation (4 cm2 of skin allowing the preparation of several million three-dimensional skin constructs, 9x12 cm, on native horse collagen). Successive fetal constructs were applied to eight patients at every change of dressing during 1-3 weeks in an outpatient setting. Complete closure was rapid (mean 15.3 days [SD 5.5]) with little hypertrophy of new skin and no retraction seen. This simple technique provided complete treatment without auto-grafting, showing that fetal skin cells might have great potential to treat burns and eventually acute and chronic wounds of other types.

Development, characterization, and use of a fetal skin cell bank for tissue engineering in wound healing.

Wound healing in fetal skin is characterized by the absence of scar tissue formation, which is not dependent on the intrauterine environment and amniotic fluid. Fetal cells have the capacity of extraordinary expansion and we describe herein the development of a fetal skin cell bank where from one organ donation (2-4 cm2) it is possible to produce several hundred million fetal skin constructs of 9 x 12 cm2. Fetal cells grow three to four times more rapidly than older skin cells cultured in the same manner and these banked fetal cells are very resistant against physical and oxidative stress when compared to adult skin cells under the same culture conditions. They are up to three times more resistant to UVA radiation and two times more resistant towards hydrogen peroxide treatment. This mechanism may be of major importance for fetal cells when they are delivered to hostile wound environments. For fetal cell delivery to patients, cells were associated with a collagen matrix to form a three-dimensional construct in order to analyze the capacity of these cells for treating various wounds. We have seen that fetal cells can modify the repair response of skin wounds by accelerating the repair process and reducing scarring in severe bums and wounds of various nature in children. Hundreds of thousands of patients could potentially be treated for acute and chronic wounds from one standardized and controlled cell bank.

Multimodality imaging evaluation of the pediatric neck: techniques and spectrum of findings.

Neck masses are a common finding in children and can present a difficult diagnostic challenge. These masses may represent a variety of conditions having a congenital, acquired inflammatory, neoplastic, or vascular origin. The fascial spaces and compartments of the neck provide an approach to differential diagnosis, and extensive knowledge of the anatomy and contents of each cervical compartment is mandatory in the diagnosis of pediatric neck lesions. Several imaging techniques, including radiography, gray-scale and Doppler ultrasonography, conventional and three-dimensional computed tomography, magnetic resonance (MR) imaging, and MR angiography, have been proposed for the evaluation of such lesions, and each has its own advantages and limitations. The imaging findings in 120 children who had been referred or treated for cervical lesions were retrospectively reviewed, and a systematic multimodality imaging approach to pediatric neck lesions based on the involvement of anatomic compartments of the cervical region was developed to increase diagnostic efficiency. Careful attention to clinical history and physical examination findings, along with knowledge of the embryologic features and anatomy of the cervical region and a multimodality imaging approach, is very helpful in the diagnosis and management of pediatric neck lesions.

Grommets and speech at three and six years in children born with total cleft or cleft palate.

OBJECTIVE: Grommets may be considered as the treatment of choice for otitis media with effusion (OME) in children born with a cleft. But the timing and precise indications to use them are not well established. The aim of the study is to compare the results of hearing and speech controls at three and six year-old in children born with total cleft or cleft palate in the presence or not of grommets. METHODS: This retrospective study concerns non syndromic children born between 1994 and 2006 and operated for a unilateral cleft lip palate (UCLP) or a cleft palate (CP) alone, by one surgeon with the same schedule of operations (Malek procedure). We compared the results of clinical observation, tympanometry, audiometry and nasometry at three and six year-old. The Borel-Maisonny classification was used to evaluate the velar insufficiency. None of the children had preventive grommets. The Fisher Exact Test was used for statistical analysis with p<0.05 considered as significant. RESULTS: Seventy-seven patients were analyzed in both groups. Abnormal hearing status was statistically more frequent in children with UCLP compared to children with CP, at three and six years (respectively, 80-64%, p<0.03 and 78-60%, p<0.02), with the use of grommets at six years in 43% of cases in both groups. Improvement of hearing status between three and six year-old was present in 5% of children with UCLP and 9% with CP, without the use of grommets. CONCLUSION: The use of grommets between three and six year-old was not associated to any improvement of hearing status or speech results children with UCLP or with CP, with a low risk of tympanosclerosis. These results favor the use of grommets before the age of three, taking into account the risk of long term tympanosclerosis.

Prenatal diagnosis of facial clefts: evaluation of a specialised counselling.

QUESTIONS UNDER STUDY/PRINCIPLES: Prenatal diagnosis of cleft lip and palate has raised many questions concerning the best way to inform the parents and offer appropriate support during the remaining pregnancy to help prepare a positive birth experience. Prenatal counselling differs according to who is responsible, and to that person's knowledge of the practical aspects of care and the impact of the malformation on the child's quality of life. The aim of the study was to evaluate our current techniques and modify them when appropriate. METHODS: 29 couples having experienced prenatal diagnosis were asked to respond retrospectively to a questionnaire. Personal obstetric and family history, timing and quality of information provided and their impressions at birth were considered. RESULTS: The parents used several sources of information: the obstetrician, the counsel by the cleft team surgeon, the parents support groups and the Internet. 93% of the parents felt well prepared psychologically for the birth of their child and concerning the practical aspects of care. 54% felt relieved that their child was less affected than imagined. 96% considered prenatal diagnosis a benefit. CONCLUSION: Basic information should be provided soon after ultrasound confirmation, followed by progressively more detailed technical discussions over the remaining pregnancy.

Phonetic and otological results after early palate closure in 18 consecutive children presenting with cleft lip and palate.

OBJECTIVE: First, to analyze the speech and hearing results at 3.5 years of age when early palate closure has been performed. Second to assess at 7 years of age the need for speech therapy and/or additional surgery in the form of cranial-based pharyngeal flap for obtaining normal speech. METHODS: Retrospective study in a tertiary teaching hospital concerning 18 consecutive cases presenting cleft lip and palate with no associated abnormalities. Interventions included early palatine closure (velum at 3 months, hard palate and lip at 6 months). Speech therapy was initiated at 3.5 years of age when needed. Cranial-based pharyngeal flap was performed when normal nasal emission was not obtained by speech therapy at 7 years of age. Phonetic and otological assessment were performed in all cases. RESULTS: Good to excellent speech in the majority (95%) of children, with only 3/18 undergoing pharyngoplasty to obtain type I or I/II speech by the age of 7 (range: 6.5-8.0). 6/18 children had drain insertion, and 2/18 had hearing loss of 20-40 dB in all frequencies. CONCLUSION: Most children (95%) start school with good or excellent speech. However, the high incidence of middle ear problems shows that more effective screening and treatment are warranted.

Propranolol in infantile haemangioma: simplifying pretreatment monitoring.

BACKGROUND: Infantile haemangiomas (IHs) are very common vascular tumours. Propranolol is at present the first-line treatment for problematic and complicated haemangioma. In accordance with a Swiss protocol, children are monitored for 2 days at the start of the treatment to detect possible side effects of this drug. Our study advocates a simplification of the pretreatment monitoring process. METHODS: All children with a problematic and complicated haemangioma treated with propranolol between September 2009 and September 2012 were included in the study. All patients were hospitalised under constant nurse supervision for 48 hours at the start of the treatment and subjected to cardiac and blood measurements. The dosage of propranolol was 1 mg/kg/day on the first day and 2 mg/kg/day from the second day. Demographic data, clinical features, treatment outcome and complications were analysed. RESULTS: Twenty-nine infants were included in our study. Of these, 86.2% responded immediately to the treatment. There were no severe adverse reactions. Six patients presented transient side effects such as bradycardia, hypotension after the first dose and hypoglycaemia later. No side effects occurred after the second dose. Treatment was never interrupted. CONCLUSION: Propranolol (a ß-blocker) is a safe treatment for problematic IH. Side effects may occur after the first dose. A strict 48 hour monitoring in hospital is expensive and may be unnecessary as long as the contraindications for the drug are respected.