The relation between clinically diagnosed and parent-reported feeding difficulties in children with and without clefts.

A cleft lip and/or palate (CL/P) is one of the most common craniofacial malformations, occurring worldwide in about one in 600-1000 newborn infants. CL/P is known to influence the feeding process negatively, causing feeding difficulties in 25-73% of all children with CL/P. Because there is a risk for serious complications in these children regarding feeding difficulties, there is often a need for intensive medical counseling and treatment. At this moment, adequate diagnosis and measurement remain a challenge and often lead to a delayed referral for professional help. Since parents play a big part in reporting feeding difficulties, it is important to help objectify parents' experiences, as well as the use of a frontline screening instrument for routine check-ups during medical appointments. The aim of this study is to investigate the relationship between parent perspective and standardized observation by medical professionals on feeding difficulties in 60 children with and without clefts at the age of 17 months. We focus on the information from parents and health professionals by comparing the Observation List Spoon Feeding and the Schedule for Oral Motor Assessment with the validated Dutch translation of the Montreal Children's Hospital Feeding Scale.  Conclusion: There is a need for timely and adequate diagnosis and referral when it comes to feeding difficulties in children with CL/P. This study underscores the importance of combining both parental observations and measurements of oral motor skills by healthcare professionals to enable this. What is Known: • Early identification of feeding difficulties can prevent adversely affected growth and development. • Clefts increase the probability of feeding difficulties; however, the diagnostic trajectory is unclear. • The Observation List Spoon Feeding (OSF) and Schedule for Oral Motor Assessment (SOMA) are validated to measure oral motor skills. The Montreal Children's Hospital Feeding Scale Dutch version (MCH-FSD) has been validated for the parental perception of infant feeding difficulties. What is New: • Parents of children with CL/P experience relatively few feeding problems in their child on average. • Oral motor skills for spoon feeding are associated with oral motor skills for solid foods in children with CL/P. • The extent of the cleft is associated with experiencing more feeding difficulties in children with CL/P.

Human adult, pediatric and microtia auricular cartilage harbor fibronectin-adhering progenitor cells with regenerative ear reconstruction potential.

Remaining challenges in auricular cartilage tissue engineering include acquiring sufficient amounts of regeneration-competent cells and subsequent production of high-quality neocartilage. Progenitor cells are a resident subpopulation of native cartilage, displaying a high proliferative and cartilage-forming capacity, yet their potential for regenerative medicine is vastly understudied. In this study, human auricular cartilage progenitor cells were newly identified in healthy cartilage and, importantly, in microtia-impaired chondral remnants. Their cartilage repair potential was assessed via in vitro 3D culture upon encapsulation in a gelatin-based hydrogel, and subsequent biochemical, mechanical, and histological analyses. Auricular cartilage progenitor cells demonstrate a potent ability to proliferate without losing their multipotent differentiation ability and to produce cartilage-like matrix in 3D culture. As these cells can be easily obtained through a non-deforming biopsy of the healthy ear or from the otherwise redundant microtia remnant, they can provide an important solution for long-existing challenges in auricular cartilage tissue engineering.

"Auricular reconstruction: A sociocultural, surgical and scientific perspective".

The functional and sociocultural role of the auricle has been prevalent in art, literature and history for millennia. It is no surprise, therefore, that auricular anomalies can be associated with affective disorders and impaired academic performance in children. The challenge of auricular reconstruction has captured the attention of surgical innovators for millennia with the earliest records of auricular reconstruction documented in the Edwin Smith Surgical Papyrus dating back to 3000 BCE. Since the 19th century, however, the interest in the ambition partial and total auricular reconstruction witnessed a rebirth, with refinements in frame construction, projection and skin coverage improving exponentially over the last two centuries. The gold standard auricular reconstruction practices today have their roots in these historical milestones, and form a solid foundation for the introduction of technological advancements such as 3D bioprinting and composite tissue allotransplantation into future auricular reconstruction practice. The aim of this review is to outline the sociocultural role of the auricle, the history and evolution of auricular reconstruction surgery and to provide an insight into potential future avenues of restoring auricular form and function.

Parental Attitude Toward the Prenatal Diagnosis of Oral Cleft: A Prospective Cohort Study.

OBJECTIVES: The prenatal diagnosis of oral clefts (OCs) by ultrasound can pose an ethical dilemma. The purpose of this study was to obtain insight into the psychosocial and moral considerations of prospective parents concerning OCs, the burden of OCs and parents' attitude toward possible termination of pregnancy (TOP) in order to improve counseling in the future. DESIGN: Between August 2011 and August 2014, a prospective cohort questionnaire study was administered. SETTING: Prenatal care clinic of the Wilhelmina's Children hospital, a Tertiary Care Centre. PARTICIPANTS: Parents expecting a child with an OC. MAIN OUTCOME MEASURES: Expectation of OCs in general and attitudes toward the impact and expected burden of the OC of their child. Furthermore, parents were asked if they had considered TOP. RESULTS: Most of the parents described an OC as a cosmetic disability (50.6%) or as "just a little different" (29.4%). These parents expected that the OC would not affect their own happiness and have only minor influence on the happiness of their future child. Health professionals had a considerable influence on parental opinion. A minority (6.4%, 5/85) of the respondents considered TOP, and none of the responders chose to terminate the pregnancy. CONCLUSIONS: This study suggests that future parents have very few negative perceptions of OC after prenatal counseling. Caregivers should be aware that their counseling is important for decision-making of parents.

Long-Term Follow-Up Study of Young Adults Treated for Unilateral Complete Cleft Lip, Alveolus, and Palate by a Treatment Protocol Including Two-Stage Palatoplasty: Speech Outcomes.

BACKGROUND: No consensus exists on the optimal treatment protocol for orofacial clefts or the optimal timing of cleft palate closure. This study investigated factors influencing speech outcomes after two-stage palate repair in adults with a non-syndromal complete unilateral cleft lip and palate (UCLP). METHODS: This was a retrospective analysis of adult patients with a UCLP who underwent two-stage palate closure and were treated at our tertiary cleft centre. Patients ≥17 years of age were invited for a final speech assessment. Their medical history was obtained from their medical files, and speech outcomes were assessed by a speech pathologist during the follow-up consultation. RESULTS: Forty-eight patients were included in the analysis, with a mean age of 21 years (standard deviation, 3.4 years). Their mean age at the time of hard and soft palate closure was 3 years and 8.0 months, respectively. In 40% of the patients, a pharyngoplasty was performed. On a 5-point intelligibility scale, 84.4% received a score of 1 or 2; meaning that their speech was intelligible. We observed a significant correlation between intelligibility scores and the incidence of articulation errors (P<0.001). In total, 36% showed mild to moderate hypernasality during the speech assessment, and 11%-17% of the patients exhibited increased nasalance scores, assessed through nasometry. CONCLUSIONS: The present study describes long-term speech outcomes after two-stage palatoplasty with hard palate closure at a mean age of 3 years old. We observed moderate long-term intelligibility scores, a relatively high incidence of persistent hypernasality, and a high pharyngoplasty incidence.

Growth and prevalence of feeding difficulties in children with Robin sequence: a retrospective cohort study.

OBJECTIVES: In addition to breathing problems, patients with Robin sequence (RS) often encounter feeding difficulties (FD). Data regarding the occurrence of FD and possible influencing factors are scarce. The study aim was to elucidate these factors to improve treatment strategies. MATERIAL AND METHODS: A retrospective comparative cohort study was conducted, consisting of 69 infants diagnosed with both RS and a cleft palate and 64 isolated cleft palate only (iCPO) infants. Data regarding FD, growth, and airway intervention were collected during the first 2 years of life. A systematic review of the literature was conducted to identify reported FD in RS patients. RESULTS: RS patients had more FD (91 %) than iCPO patients (72 %; p = 0.004). Also, nasogastric (NG)-tube feeding was necessary more frequently and for a longer period (both p < 0.001). Growth was lower in RS than iCPO infants (p = 0.008) and was not affected by the kind of airway management (conservative/surgical; p = 0.178), cleft palate grade (p = 0.308), or associated disorders (p = 0.785). By contrast, surgical intervention subtype did significantly affect growth. Mean reported FD for RS in the literature is 80 % (range = 47-100 %), and 55 % (range = 11-100 %) of infants need NG-tube feeding. CONCLUSIONS: FD is present in a large proportion of infants with RS, which indicates the need for early recognition and proper treatment to ensure optimal growth. Growth during the first 2 years of life is significantly lower in RS patients than iCPO patients, which indicates the need for careful attention and long-term follow-up. CLINICAL RELEVANCE: This study indicates the need for early recognition and proper treatment of FD in RS to ensure optimal growth. In addition, growth needs careful attention and long-term follow-up.

Mechanisms of ear trauma and reconstructive techniques in 105 consecutive patients.

Acquired auricular deformities may diminish facial esthetics and cause psychological distress. The aim of this article is to provide an overview of the type of injuries and applied reconstructive techniques in a large academic hospital in The Netherlands. A retrospective chart review was conducted for the last 105 patients who underwent auricular reconstruction for an acquired deformity. Data concerning gender, affected side, cause of injury, anatomical region, the previous and further surgeries, type of cartilage, and skin cover used were collected and analyzed. 105 patients were included. Acquired auricular deformities were mainly caused by bite injuries (22 %), traffic accidents (17 %), burns (9.5 %), and post-otoplasty complications (9.5 %). The upper third of the auricle was most often injured (41 %), followed by the entire auricle (19 %). 70 % of cases required reconstruction with costal cartilage. The most common form of cutaneous cover was a postauricular skin flap (40 % of cases). This study gives a complete overview of causes and treatment of acquired auricular deformities. The results are comparable with the results of similar studies found in literature. Bite wounds are the leading cause of acquired auricular injuries. The upper third is most commonly affected. In the largest percentage of reconstructions, costal cartilage and a postauricular flap were used to correct the deformity.

Combining regenerative medicine strategies to provide durable reconstructive options: auricular cartilage tissue engineering.

Recent advances in regenerative medicine place us in a unique position to improve the quality of engineered tissue. We use auricular cartilage as an exemplar to illustrate how the use of tissue-specific adult stem cells, assembly through additive manufacturing and improved understanding of postnatal tissue maturation will allow us to more accurately replicate native tissue anisotropy. This review highlights the limitations of autologous auricular reconstruction, including donor site morbidity, technical considerations and long-term complications. Current tissue-engineered auricular constructs implanted into immune-competent animal models have been observed to undergo inflammation, fibrosis, foreign body reaction, calcification and degradation. Combining biomimetic regenerative medicine strategies will allow us to improve tissue-engineered auricular cartilage with respect to biochemical composition and functionality, as well as microstructural organization and overall shape. Creating functional and durable tissue has the potential to shift the paradigm in reconstructive surgery by obviating the need for donor sites.

Long-Term Results of Mandibular Distraction Osteogenesis with a Resorbable Device in Infants with Robin Sequence: Effects on Developing Molars and Mandibular Growth.

BACKGROUND: Mandibular distraction osteogenesis with a unidirectional resorbable device is an effective treatment option for severe upper airway obstruction in infants with Robin sequence. Long-term effects, especially with regard to tooth development and mandibular outgrowth, are not known. METHODS: Robin sequence infants with a follow-up of greater than or equal to 5 years were included. Baseline characteristics were extracted from medical records. Panoramic and lateral cephalometric radiographs were analyzed and patients were recalled for physical examination. RESULTS: Ten infants underwent mandibular distraction osteogenesis at a mean age of 3.7 months (median, 19 months; range, 11 days to 27 months). Mean length of follow-up was 6.8 years (range, 5.0 to 7.9 years). Ten Robin sequence infants without mandibular distraction osteogenesis (mean length of follow-up, 7.4 years; range, 6.7 to 8.9 years) were the controls. Shape anomalies, positional changes, and root malformations of molars were seen significantly more often than in the control group (p = 0.007, p = 0.009, and p = 0.043, respectively). Mandibular length was shorter (p = 0.030), but mandibular ramus height was comparable (p = 0.838) with that of the non-mandibular distraction osteogenesis group. Compared with healthy controls, all Robin sequence infants had a significantly shorter mandible. CONCLUSIONS: Mandibular distraction osteogenesis with a resorbable system reveals overall good short- and long-term results, but the effects on developing molars and mandibular outgrowth likely necessitate secondary procedures. This factor should be considered when deciding on treatment options and counseling of parents. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Accurate Measurements of the Skin Surface Area of the Healthy Auricle and Skin Deficiency in Microtia Patients.

BACKGROUND: The limited cranial skin covering auricular implants is an important yet underrated factor in auricular reconstruction for both reconstruction surgery and tissue engineering strategies. We report exact measurements on skin deficiency in microtia patients and propose an accessible preoperative method for these measurements. METHODS: Plaster ear models (n = 11; male:female = 2:1) of lobular-type microtia patients admitted to the University Medical Center Utrecht in The Netherlands were scanned using a micro-computed tomographic scanner or a cone-beam computed tomographic scanner. The resulting images were converted into mesh models from which the surface area could be calculated. RESULTS: The mean total skin area of an adult-size healthy ear was 47.3 cm2, with 49.0 cm2 in men and 44.3 cm2 in women. Microtia ears averaged 14.5 cm2, with 15.6 cm2 in men and 12.6 cm2 in women. The amount of skin deficiency was 25.4 cm2, with 26.7 cm2 in men and 23.1 cm2 in women. CONCLUSIONS: This study proposes a novel method to provide quantitative data on the skin surface area of the healthy adult auricle and the amount of skin deficiency in microtia patients. We demonstrate that the microtia ear has less than 50% of skin available compared with healthy ears. Limited skin availability in microtia patients can lead to healing problems after auricular reconstruction and poses a significant challenge in the development of tissue-engineered cartilage implants. The results of this study could be used to evaluate outcomes and investigate new techniques with regard to tissue-engineered auricular constructs.

Treatment of infantile hemangiomas: therapeutic options in regard to side effects and adverse events - a review of the literature.

INTRODUCTION: While options for treatment strategies for infantile hemangiomas (IH) are numerous, evidence-based information about agents, optimal dosage, adverse effects, treatment modality, pretreatment and treatment strategies remain limited. AREAS COVERED: To evaluate side effects and adverse events of medical treatment in children with infantile hemangioma, a comprehensive review of the literature was performed to provide information for daily practice. In total 254 studies were retrieved from medical databases and comprised 10,022 patients divided into 5 different treatment groups. Information about working mechanism, side effects and adverse events of therapies used as a single agent for IH are discussed and evaluated according to information from pharmacotherapeutic databases. Randomized controlled trials have only scarcely been performed for the many therapeutic options reported for IH. Short- and long-term side effects and adverse events, have not been systematically studied. Subsequently information about the medical treatment options and pharmacotheraputic databases for therapy in children with IH are incomplete. EXPERT OPINION: From the many therapeutic options, propranolol is the first-line approach for IH, predominantly based on clinical observation, efficacy and tolerability in the short-term. The unsolved ravels of possible short and long-term adverse events of propranolol used during early developmental stages of children need thorough review.

Replantation versus Prosthetic Fitting in Traumatic Arm Amputations: A Systematic Review.

BACKGROUND: Traumatic arm amputations can be treated with replantation or surgical formalization of the stump with or without subsequent prosthetic fitting. In the literature, many authors suggest the superiority of replantation. This systematic review compared available literature to analyze whether replantation is functionally and psychologically more profitable than formalization and prosthetic fitting in patients with traumatic arm amputation. METHODS: Functional outcome and satisfaction levels were recorded of patients with amputation levels below elbow, through elbow, and above elbow. RESULTS: Functional outcomes of 301 replantation patients and 172 prosthesis patients were obtained. In the replantation group, good or excellent functional scores were reported in 39% of above elbow, 55% of through elbow, and 50% of below elbow amputation cases. Nearly 100% of patients were satisfied with the replanted limb. In the prosthesis group, full use of the prosthesis was attained in 48% of above elbow and in 89% of below elbow amputation patients. Here, 29% of patients elected not to use the prosthesis for reasons including pain and functional superfluity. In both replantation patients and prosthesis wearers, a below elbow amputation yielded better functional results than higher amputation levels. CONCLUSIONS: Replantation of a traumatically amputated arm leads to good function and higher satisfaction rates than a prosthesis, regardless of the objective functional outcome. Sensation and psychological well-being seem the two major advantages of replantation over a prosthesis. The current review of the available literature shows that in carefully selected cases replantation could be the preferred option of treatment.

A practical prenatal ultrasound classification system for common oral clefts.

OBJECTIVE: Our aim is to introduce and validate a new oral cleft classification system based on prenatal ultrasound for use by professionals in daily practice. METHODS: During a 3-year period (2011-2014), all cases of prenatal oral cleft diagnosed by ultrasound were retrospectively reviewed. A new prenatal ultrasound classification system was introduced. For the purpose of validation, prenatal ultrasound images of oral cleft types were described according to the new classification system and were compared with postnatal findings by reviewing medical records. RESULTS: A total of 103 fetuses with oral cleft were identified by ultrasound. The mean gestation time at detection was 20.4 weeks (95% confidence intervals: 20.0-20.7). The association between oral cleft and other anomalies varied by cleft type; types 2b/3b and 4 were most frequently associated with other anomalies. The measure of agreement between the prenatal and postnatal findings showed a Kappa value of 0.63 (95% confidence intervals: 0.52-0.75), demonstrating the accuracy of this new classification system. CONCLUSION: A new prenatal oral cleft classification system is presented. This system appears to be accurate, and it shows the variation in the risk of associated anomalies for each cleft type. We expect that ultrasonographers will be able to use the new classification in daily practice.

Nerve conduction studies after decompression in painful diabetic polyneuropathy.

PURPOSE: To investigate the influence of nerve decompression at potential entrapment sites in the lower extremity in painful diabetic polyneuropathy on nerve conduction study variables. METHODS: Forty-two patients with painful diabetic polyneuropathy were included in this prospective randomized controlled trial. Preoperative nerve conduction studies were performed bilaterally. Each patient underwent unilateral surgical decompression of the tibial nerve and common, superficial, and deep peroneal nerves. The contralateral side was used as a control: within-patient comparison. One year postoperatively, the nerve conduction studies were repeated. Univariate paired sample T-tests and a multivariate analysis of variance were performed to compare data. RESULTS: In univariate analysis of the peroneal nerve, the distal compound muscle action potential amplitude measured at the extensor digitorum brevis muscle of the intervention legs decreased significantly, as did the area drop measured at the extensor digitorum brevis muscle of the control legs. The distal motor latency measured at the extensor digitorum brevis muscle of the intervention legs increased significantly, as did the distal compound muscle action potential amplitude measured at the anterior tibial muscle of the control legs. For the tibial nerve, the distal compound muscle action potential duration decreased significantly in the control legs. The multivariate analysis showed no significance overall. CONCLUSIONS: Decompression of nerves of the lower extremity in patients with painful diabetic polyneuropathy has no beneficial effect on nerve conduction study variables 12 months after surgery.

Professional opinion on oral cleft during pregnancy: a comparison between Israel and The Netherlands.

OBJECTIVE: The aim of this study was to assess the opinion of obstetric care providers who perform prenatal ultrasounds to screen for anomalies and who advise women about their options, including termination of pregnancy, when an oral cleft is detected. We compared providers' opinions about pregnancy termination for isolated oral cleft in The Netherlands, where the number of terminations is low, and in Israel, where the number is high. METHODS: Online questionnaires were used. The questions assessed the providers' views regarding the estimated burden of treatment, the functioning ability, and the level of happiness of children with an oral cleft and their parents. Additionally, we assessed providers' opinions on pregnancy termination for isolated oral cleft. RESULTS: In The Netherlands, more professionals considered oral cleft a disability (rate differences 17.8%, 95% confidence interval: 0.5-33.1%) than in Israel. In the Netherlands, 10.6% of respondents (compared with 11.1% in Israel) thought that an isolated cleft was a reason for terminations of pregnancy (TOP) (rate differences 0.6%, 95% confidence interval: -12% to 10.9%). CONCLUSIONS: Prenatal care providers in The Netherlands and Israel do not differ in their opinions about the severity of oral cleft and the acceptability of TOP for an isolated oral cleft. This study shows that prenatal care providers' attitudes do therefore not explain the dramatic difference between these countries in the number of TOP for isolated oral cleft.

A pragmatic approach to infants with Robin sequence: a retrospective cohort study and presence of a treatment algorithm.

OBJECTIVES: Initial approaches to and treatments of infants with Robin sequence (RS) is diverse and inconsistent. The care of these sometimes critically ill infants involves many different medical specialties, which can make the decision process complex and difficult. To optimize the care of infants with RS, we present our institution's approach and a review of the current literature. MATERIAL AND METHODS: A retrospective cohort study was conducted among 75 infants diagnosed with RS and managed at our institution in the 1996-2012 period. Additionally, the conducted treatment regimen in this paper was discussed with recent literature describing the approach of infants with RS. RESULTS: Forty-four infants (59%) were found to have been treated conservatively. A significant larger proportion of nonisolated RS infants than isolated RS infants needed surgical intervention (53 vs. 25%, p = .014). A mandibular distraction was conducted in 24% (n = 18) of cases, a tracheotomy in 9% (n = 7), and a tongue-lip adhesion in 8% (n = 6). Seventy-seven percent of all infants had received temporary nasogastric tube feeding. The literature review of 31 studies showed that initial examinations and the indications to perform a surgical intervention varied and were often not clearly described. CONCLUSIONS: RS is a heterogenic group with a wide spectrum of associated anomalies. As a result, the decisional process is challenging, and a multidisciplinary approach to treatment is desirable. Current treatment options in literature vary, and a more uniform approach is recommended. CLINICAL RELEVANCE: We provide a comprehensive and pragmatic approach to the analysis and treatment of infants with RS, which could serve as useful guidance in other clinics.