Judith Hohlfeld

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.

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.

Speech prognosis and need of pharyngeal flap for non syndromic vs syndromic Pierre Robin Sequence

BACKGROUND The aim of this retrospective study was to evaluate speech outcome and need of a pharyngeal flap in children born with nonsyndromic Pierre Robin Sequence (nsPRS) vs syndromic Pierre Robin Sequence (sPRS). METHODS Pierre Robin Sequence was diagnosed when the triad microretrognathia, glossoptosis, and cleft palate were present. Children were classified at birth in 3 categories depending on respiratory and feeding problems. The Borel-Maisonny classification was used to score the velopharyngeal insufficiency. RESULTS The study was based on 38 children followed from 1985 to 2006. For the 25 nsPRS, 9 (36%) pharyngeal flaps were performed with improvements of the phonatory score in the 3 categories. For the 13 sPRS, 3 (23%) pharyngeal flaps were performed with an improvement of the phonatory scores in the 3 children. There was no statistical difference between the nsPRS and sPRS groups (P = .3) even if we compared the children in the 3 categories (P = .2). CONCLUSIONS Children born with nsPRS did not have a better prognosis of speech outcome than children born with sPRS. Respiratory and feeding problems at birth did not seem to be correlated with speech outcome. This is important when informing parents on the prognosis of long-term therapy.

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.

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.

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 childs 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.

Complete bilateral facial cleft (Tessier 4) with corneal staphyloma: a rare association

The oro-ocular cleft no. 4 according to the Tessier classification is one of the rarest facial cleft, and to this day, few cases have been reported in the literature. We describe the case of a 9-month-old girl with a complete bilateral facial cleft. On the right cornea protruded a hard lesion, a corneal staphyloma. We describe the 3 primary surgical steps used to restore the possibility of satisfactory feeding, to promote language acquisition, and to protect vision in the nonaffected eye. The psychological and social aspects of severe facial deformities in developing countries are also tackled.

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 β-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.

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: Marginal Security in Infants With Cleft

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.