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Objectives: The submucous cleft palate (SMCP) is considered to be the most subtle type of cleft palate. Early detection is important to allow on time intervention by speech therapy and/or surgical repair before the children already develop compensatory speech mechanisms. The purpose of this study was to investigate at what time children with a SMCP present, to determine when children are operated, and to analyze the postoperative outcomes for in SMCP children. Patient and methods: Medical records from 766 individuals registered in the cleft registry in the Wilhelmina's Children's' Hospital, Utrecht, were retrospectively reviewed. Inclusion criteria were children diagnosed with SMCP. The following data were collected: age at diagnosis, physical examination, age at surgery, surgical technique, speech therapy pre- and post-surgery, otitis media, secondary cleft surgery, family history, syndromes, and other anomalies. Results: In total, 56 SMCP children were identified. The mean age of diagnosis was 44.0 months (range 0–150, SD = 37.0). In 48 children (85.7%), surgical intervention was performed (Furlow plasty, intravelar veloplasty, pharyngoplasty, or Furlow combined with buccal flap). Conclusion: This retrospective study reconfirms that SMCP often presents late, even in a country with a modern healthcare system and adequate follow-up of all newborns by the so-called youth doctors in "children's healthcare centers" up to the age of 4 years old. Almost 86% of patients ultimately needed palate surgery when SMCP was suspected. Clinical relevance: Any child presenting with repeated episodes of otitis media, nasal regurgitation, or speech difficulties should have prompt consideration for SMCP as diagnosis. [ABSTRACT FROM AUTHOR]

Objectives: Clinicians agree that children with isolated cleft lip have fewer cleft-associated problems than children with cleft lip and palate. Unfortunately, for isolated cleft lip children, the risk of cleft-associated problems is unknown and maybe underestimated. Often, these children do not get the required follow-up by a multidisciplinary team and thereby not the known benefits in supporting their development. This study examines the incidence of cleft-related speech problems and ear problems in children with isolated cleft lip. Materials and methods: A prospective study was performed on all children born with an isolated cleft lip and treated at the Wilhelmina Children's Hospital in Utrecht between January 2007 and April 2014. Data were collected for sex, date of birth, genetics, cleft lip type, date of cleft lip repair, type of repair, speech/language problems, and ear problems. Results: This study included 75 patients (59% male). The mean age of the children at the moment of speech examination was 32.5 months (SD 6.1). Eighteen of the 75 children (24%) needed speech and language therapy; however, only one child (1.3%) had a cleft-related speech problem. Sixteen of the 75 patients (21%) reported a history of one or more episodes of acute otitis media (AOM)/otitis media with effusion (OME) during the first 6 years. Conclusion/clinical relevance: This is the first prospective study analyzing the incidence of cleft-related speech problems in children with an isolated cleft lip. These children do not have a higher risk of cleft-related speech problems or AOM/OME when compared to the general population. However, children with an isolated cleft do have a higher incidence of speech therapy. [ABSTRACT FROM AUTHOR]

Objectives: Throat packs are commonly used to prevent ingestion or aspiration of blood and other debris during cleft lip/palate surgery. However, dislodgement or (partial) retainment after extubation could have serious consequences. The aim of the present study was to investigate the effect of omitting pharyngeal packing during cleft lip/palate surgery on the incidence of early postoperative complications in children.Materials and methods: A retrospective study was performed on all children who underwent cleft lip/palate surgery at the Wilhelmina Children’s Hospital. This study compared the period January 2010 through December 2012 when pharyngeal packing was applied according to local protocol (group A) with the period January 2013 till December 2015 when pharyngeal packing was no longer applied after removal from the protocol (group B). Data were collected for sex, age at operation, cleft lip/palate type, type of repair, lateral incisions, length of hospital stay, and complications in the first 6 weeks after surgery. Early complications included wound dehiscence, postoperative bleeding, infection, fever, upper respiratory tract infection (URTI), and lower respiratory tract infection (LRTI).Results: This study included 489 cleft lip/palate operations (group A n = 246, group B n = 243). A total of 39 (15.9%) early complications were recorded in group A and a total of 40 (16.5%) in group B. There were no significant differences (P = 0.902) in complications between the two groups; however, there was a significant difference (P < 0.001) in length of hospital stay between the two groups (group A 3.6 days vs group B 3.2 days).Conclusion: Omitting routine placement of throat packs in cleft lip/palate surgery was not associated with an increased early postoperative complication rate. Therefore, the traditional, routine placement of a throat pack during cleft lip/palate surgery can be questioned.Clinical relevance: The traditional, routine placement of a throat pack during cleft lip/palate surgery can be questioned. [ABSTRACT FROM AUTHOR]

Objective: To develop a reliable and easy-to-use method to assess the nasolabial appearance of 18-year-old patients with unilateral cleft lip and palate (CLP). Design: Retrospective analysis of nasolabial aesthetics using a 5-point ordinal scale and newly developed photographic reference scale: the Cleft Aesthetic Rating Scale (CARS). Three cleft surgeons and 20 medical students scored the nasolabial appearance on standardized frontal photographs. Setting: VU University Medical Center, Amsterdam. Patients: Inclusion criteria: 18-year-old patients, unilateral cleft lip and palate, available photograph of the frontal view. Exclusion criteria: history of facial trauma, congenital syndromes affecting facial appearance. Eighty photographs were available for scoring. Main Outcome Measures: The interobserver and intraobserver reliability of the CARS for 18-year-old patients when used by cleft surgeons and medical students. Results: The interobserver reliability for the nose and lip together was 0.64 for the cleft surgeons and 0.61 for the medical students. There was an intraobserver reliability of 0.75 and 0.78 from the surgeons and students, respectively, on the nose and lip together. No significant difference was found between the cleft surgeons and medical students in the way they scored the nose (P = 0.22) and lip (P = 0.72). Conclusions: The Cleft Aesthetic Rating Scale for 18-year-old patients has a substantial overall estimated reliability when the average score is taken from three or more cleft surgeons or medical students assessing the nasolabial aesthetics of CLP patients. [ABSTRACT FROM AUTHOR]

Unlabelled: Cleft palate only (CPO) is a common congenital malformation, and most patients are diagnosed within the first weeks after birth. Late diagnosis of the cleft palate (CP) could initially result in feeding and growth impairment, and subsequently speech and hearing problems later in life. The purpose of this study is to retrospectively investigate (1) at which age CPO is diagnosed and (2) how the presence of syndromes and other factors relate to the age at diagnosis. The mean age of all children at our centre with CPO included between 1997 and 2014 at diagnosis (n = 271) was 1 year and 4 months. In all, 24.8% (n = 67) was older than 12 months when diagnosed, and 37.3% (n = 101) of all children had been diagnosed >30 days. These findings remain valid when a cut-off point of 14 days is used (44.3% late). Moreover, the grade of the cleft was a determining factor for successful diagnosis; submucous clefts were detected much later on average (89.3% > 30 days; p = .000). Similar results were found using Kaplan-Meier survival analyses.Conclusion: CPO is often diagnosed late. Patients diagnosed ≤30 days after birth more often presented with an associated disorder. Early diagnoses became more frequent as the severity of the cleft increased (grades 1-4). Professionals should perform more thorough intra-oral investigations, including manual palpations and visual inspections of the palate; they should be made more aware of the frequent accompanying symptoms.What Is Known: The presence of cleft palate only (CPO) is known to negatively affect feeding, hearing, speech and (social) development. Submucous clefts are often underdiagnosed due to their difficulty to detect. As far as we know the literature shows that symptomatic submucous CPs are often diagnosed at an average age of 4.9 years.What Is New: 37.3% respectively of all children with CPO were diagnosed relatively late (>30 days after birth), 24.8% was older than 12 months when diagnosed. Mean age of all children with CPO was 1 year and 4 months. We conclude that midwives and pediatricians should perform more through intra-oral investigations of all new-borns, including both a manual palpation, als well a visual inspection of the palate. [ABSTRACT FROM AUTHOR]

Objective The aim of this study was to assess the accuracy of prenatal transabdominal ultrasound in determining the oral cleft type. Methods A retrospective cohort study was performed on all consecutive cases of orofacial cleft diagnosed by prenatal ultrasound examination in the Wilhelmina Children's Hospital, a tertiary referral hospital, between January 2002 and December 2012. Prenatal findings were compared with postnatal diagnoses. Results A total of 134 patients were included. The mean gestational age at ultrasound examination was 24 weeks + 5 days. Prenatal diagnosis was in accordance with postnatal findings in 76.9% of the cases (103/134) with regard to the cleft type. Underestimation of the cleft occurred in 19.4% (26/134), whereas in 3.7% (5/134), the extent was overestimated. In distinguishing bilateral from unilateral clefts, no errors were made. Conclusion Prenatal ultrasound is accurate in assessing the types of orofacial clefts in a large majority of the cases and is completely accurate in distinguishing between unilateral and bilateral clefts. This study indicates that it is a reliable technique to assess the cleft type, which is important for counseling future parents. Although, the clinician should be aware of the fact that a cleft palate is easily missed, and subsequently, underestimation of cleft extend is frequent. [ABSTRACT FROM AUTHOR]

ABSTRACT Objective The aim of the study was to measure the degree of premaxillary protrusion in fetuses with orofacial clefts of various severities. Methods The maxilla-nasion-mandible (MNM) angle was measured retrospectively on by multiplanar corrected volumes. Sixty-two fetuses with orofacial clefts and known outcome volumes of the fetal head were available. Results In 48 of the 62 cases, the MNM angle could be measured [mean gestational age 23 (range, 18-30) weeks]. The mean MNM angle was normal in all nine cases with cleft lip and intact alveolar ridge (15.2°; range, 12.5°- 16.9°). In 24 cases with unilateral cleft lip with or without cleft palate (UCL/P), the mean MNM angle was 20.0° (range, 13.3-26.2°), being above the 95th percentile in 79% ( n = 19) and normal in 21% ( n = 5). In 14 bilateral cleft lip and palate (BCL/P) cases, the mean MNM angle was 26.5° (range, 19.2°-33.7°) and above the 95th percentile in all cases. There was no difference in MNM angle between isolated clefts and clefts associated with other anomalies. In one case with a Tessier 4 cleft, the MNM angle was above the 95th percentile (25.2°). Conclusion The premaxilla tends to protrude in both BCL/P as UCL/P cases. The degree of protrusion varies greatly, especially in the BCL/P group. © 2013 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Objective: To describe the effect of time after velopharyngoplasty on outcome and to search for preoperative prognostic factors for residual hypernasality in patients with 22q11.2 deletion syndrome. Design: Retrospective chart review. Setting: Tertiary hospital. Patients: Patients with 22q11.2 deletion syndrome and velopharyngeal dysfunction who underwent a primary (modified) Honig velopharyngoplasty between 1989 and 2009. Main Outcome Measures: Clinically obtained perceptual and instrumental measurements of resonance, nasalance, and understandability before and after velopharyngoplasty. Results: Data were available for 44 of 54 patients (81% follow-up), with a mean follow-up time of 7.0 years (range, 1.0 to 19.4 years). During follow-up, 24 (55%) patients attained normal resonance and 20 (45%) had residual hypernasality or underwent revision surgery. Mean postoperative nasalance and understandability scores were closer to the norm than mean preoperative scores were (2.0 versus 5.5 standard deviations for the normal passage, 1.3 versus 8.1 standard deviations for the nonnasal passage, and score 2.3 versus 4.1 understandability). Serial measurements revealed that hypernasality only resolved an average of 5 years after surgery, and three patients whose resonance initially normalized later relapsed to hypernasality. Gender, age at surgery, lateral pharyngeal wall adduction, velar elevation, presence of a palatal defect, previous intravelar veloplasty, nasalance, understandability, adenoidectomy, hearing loss, and IQ were not able to predict poor outcome following primary velopharyngoplasty (all p > .05). Conclusions: In this chart review of patients with 22q11.2 deletion syndrome and velopharyngeal dysfunction, residual hypernasality persisted in many patients after velopharyngoplasty. None of the preoperative factors that were studied had prognostic value for the outcome. [ABSTRACT FROM AUTHOR]

Objectives To evaluate the sensitivity and specificity of ultrasound for detecting prenatal facial clefts in low-risk and high-risk populations. Methods This study prospectively followed up a non-selected population, namely all pregnant women who underwent routine second-trimester prenatal ultrasound screening in the Utrecht region during the 2-year period from January 2007 to December 2008. Results A total of 35 924 low-risk and 2836 high-risk pregnant women underwent ultrasound screening. Orofacial clefts were present in 62 cases, an incidence of 1:624. The distribution of clefts was as follows: 18 (29%) cleft lip, 25 (40%) cleft lip with cleft palate, 17 (27%) cleft palate only, one median cleft and one atypical cleft. Of these, 38 (61%) were unilateral and 23 (37%) were bilateral. Thirty-nine per cent (24/62) had associated anomalies, with most chromosomal defects found in the cleft lip with cleft palate and cleft palate only groups. Cleft lip with or without cleft palate was detected prenatally in 38/43 cases, a sensitivity of 88%. No case of cleft palate only was detected prenatally. There were three false-positive cases, of which two were fetuses with multiple congenital deformities. Conclusions Ultrasound screening has a high sensitivity for the detection of cleft lip with and without cleft palate in high-risk and low-risk pregnancies in our region, where well-trained sonographers carry out primary screening. The key to a high sensitivity of prenatal ultrasound is likely to be a combination of excellent training of sonographers, referral to specialized centers when a cleft is suspected, routine visualization of the fetal face and advances in ultrasound techniques. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Objective: To review the clinical outcomes of our cleft palate team in terms of fistula rate and to determine whether gender, syndrome, cleft type, age at repair, operating surgeon, type of cleft repair, and cleft width influence the risk of fistula occurrence. Cleft palate repair was done in one- or two-stages. Design: A retrospective analysis was performed of medical records of 275 patients who underwent palate repair and who were born between 1988 and 1997. All consecutive cleft (lip and) palate patients (including syndromes) were incorporated. Multivariate analysis was performed to look for predictors of the formation of fistulas. Results: One hundred fifty-four and 121 patients underwent two-stage and one-stage repair, respectively. The median duration of follow-up was 9 years. The overall incidence of the formation of a fistula was 21%, with a recurrence rate of 9% after fistula repair. Three independent predictors of fistulization were found: palate repair in two stages, younger age at the second stage of a twostage repair (≤3.0 years), and greater cleft width (≥13 mm). A fistula occurred in 27% of two-stage repairs versus 14% of one-stage repairs. Conclusions: This study found a relatively high incidence of fistula formation after cleft palate repair. Although the study populations were not randomized, this study suggests that a two-stage closure has a higher rate of fistula formation when compared with a one-stage closure. This study demonstrates that cleft width at the time of cleft palate repair plays a crucial role in the development of fistulas. [ABSTRACT FROM AUTHOR]

Objective: To gather evidence surrounding the confusion in the classification of Robin sequence and inform those who have the power to make the changes in defining this symptom complex. Method: A questionnaire was sent to all participating cleft palate teams (N = 204) of the American Cleft Palate-Craniofacial Association. The questionnaire identified the precise, different characteristics for diagnosing Robin sequence and evaluated whether the difference between a retrognathia and micrognathia influenced the diagnosis process. We subsequently also investigated whether the cleft type (i.e., U-shaped versus V-shaped) had any influence in the decision-making process. A PubMed literature review of the 50 most recent manuscripts about Robin sequence was evaluated also. Results: Seventy-three questionnaires were received. This 35% response rate revealed 14 different definitions of Robin sequence. A PubMed literature review of 50 consecutive manuscripts revealed 15 different descriptions. Conclusion: This study confirms that nosologic confusion is widespread with regard to defining Robin sequence. This has implications for evaluating Robin sequence, giving advice about the prognosis and genetic counseling, and refining treatment options. [ABSTRACT FROM AUTHOR]