Hemangiol in infantile haemangioma: A paediatric post-marketing surveillance drug study.

AIM: Infantile haemangioma (IH) is the most common benign tumour in children. Since 2014, propranolol has become the first-choice therapy and currently Hemangiol is the only approved drug for complicated haemangioma. This post-marketing study reports the use of Hemangiol for IH in paediatric practice. METHOD AND RESULTS: From January 2014 to November 2018, 94 children (median age 4 [0; 21] months; 75% female) treated with Hemangiol for proliferative IH were enrolled in the study. The systematic paediatric cardiology consultation never contraindicated beta-blockers. Two Hemangiol initiation protocols were used: a conventional ambulatory 3-week titration phase protocol (n = 76, 80.9%), and a rapid initiation protocol with a 48-hour dose escalation in conventional hospitalization for severe proliferative or ulcerated IH (n = 18, 19.1%). In both protocols, the haemodynamic tolerance was good. The mean maintenance dose of Hemangiol was 2.7 ± 0.8 mg/kg/day, with a median treatment duration of 7 [1.5; 19] months. Adverse events (AEs) have been found in 25 (26,6%) patients, including 8 (8.5%) patients with serious AEs (uncontrolled bronchial hyperreactivity, n = 5; serious hypoglycaemia, n = 3). Some patients had one or more AEs, a total of 24 nonserious AEs was reported in 19 patients (sleep disturbances, n = 9; respiratory disorders, n = 5; digestive disorders, n = 6). No cardiac adverse event was reported. CONCLUSION: This post-marketing surveillance drug study supports the good tolerance of Hemangiol in children with IH. A rapid initiation protocol is of interest when treatment is urgent. The pretherapeutic paediatric cardiology consultation should not be systematic but only indicated for specific patients. CLINICALTRIALS.GOV: NCT04105517.

Early needle aspiration of large infant cephalohematoma: a safe procedure to avoid esthetic complications.

Cephalohematoma is a common pathology in newborns. Observation is the primary treatment for most patients with small uncomplicated cephalohematoma. Conversely, a large cephalohematoma can lead to calcification with unesthetic local deformation or deformational plagiocephaly. The objective of the study was to evaluate the iatrogenic risk associated with early puncture under local anesthesia and oral sucrose. This is a retrospective study of 67 consecutive newborns followed at Montpellier University Hospital, France, between 2010 and 2017. Large cephalohematoma was defined on the basis of the bump projection. Due to the uncertainty of the spontaneous resorption and the risk of calcification after 4 weeks which render the needle aspiration ineffective, puncture was performed between 2 and 4 weeks of life after coagulation evaluation and ultrasound of the skull and scalp. Puncture was performed in 43 boys (64%) and 24 (36%) girls between day 15 and day 30 after birth. The cephalohematoma maximal projection measured by ultrasound ranged from 9 to 13 mm (Q1,Q4) with a median value of 12 mm. No puncture-related complication was recorded during the intervention and at the 1-month follow-up visit.Conclusion: In newborns with large and persistent unesthetic cephalohematoma, puncture under local anesthesia with oral sucrose can be safely proposed between day 15 and day 30 after birth.What is Known:• Infant cephalohematoma is a frequent pathology of newborns, consisting of a traumatic subperiosteal hematoma of the skull. Most cephalohematomas are small and require no treatment because they spontaneously disappear within the first month.• Large and non-resorptive cephalohematomas may have significant esthetic and functional consequences.What is New:• Early puncture under local anesthesia is a safe, effective, and rapid procedure, decreasing the risk of persistent skull deformities.• Puncture can be proposed for newborns with a large (high projection and/or high angle connection) persistent anesthetic cephalohematoma, between day 15 and day 30, before spontaneous calcification.

Prenatal ultrasound diagnosis of cleft palate without cleft lip, the new ultrasound semiology.

OBJECTIVES: The aim of this study was to define the prenatal ultrasound semiology of cleft palate without cleft lip using 3D visualization of the fetal palate. METHODS: A prospective longitudinal study was performed in our University Hospital Center from 2011 to 2018. The fetal secondary palate was studied in 3D, starting with 2D axial transverse ultrasound view. We defined a cleft palate as a disruption of the horizontal plate of the palatine bone of the secondary palate. Prenatal findings were correlated to anatomic postnatal examinations performed by a paediatric plastic surgeon. RESULTS: Forty-three cases of cleft palate without cleft lip were prenatally diagnosed, of whom 34 were associated with malformations. We defined four types of disruptive appearances: isolated nonvisualization of the posterior nasal spine; partial-disruption or cleft velum; complete disappearance or V-shaped cleft palate; and complete disappearance or U-shaped cleft palate. The adjusted kappa coefficient, between prenatal and postnatal evaluation, was 0.88 (95% CI: 0.79-0.97), corresponding to an excellent agreement. CONCLUSIONS: Using a strictly axial transverse ultrasound view, visualization of the secondary fetal palate enables to diagnose a cleft palate without cleft lip. This method offers a prenatal anatomic classification of cleft palate with a high level of concordance to postnatal findings.

The scalp hair collar and tuft signs: A retrospective multicenter study of 78 patients with a systematic review of the literature.

BACKGROUND: Hair collar sign (HCS) and hair tuft of the scalp (HTS) are cutaneous signs of an underlying neuroectodermal defect, but most available data are based on case reports. OBJECTIVE: We sought to define the clinical spectrum of HCS and HTS, clarify the risk for underlying neurovascular anomalies, and provide imaging recommendations. METHODS: A 10-year multicenter retrospective and prospective analysis of clinical, radiologic, and histopathologic features of HCS and HTS in pediatric patients was performed. RESULTS: Of the 78 patients included in the study, 56 underwent cranial and brain imaging. Twenty-three of the 56 patients (41%) had abnormal findings, including the following: (1) cranial/bone defect (30.4%), with direct communication with the central nervous system in 28.6%; (2) venous malformations (25%); or (3) central nervous system abnormalities (12.5%). Meningeal heterotopia in 34.6% (9/26) was the most common neuroectodermal association. Sinus pericranii, paraganglioma, and combined nevus were also identified. LIMITATIONS: The partial retrospective design and predominant recruitment from the dermatology department are limitations of this study. CONCLUSIONS: Infants with HCS or HTS are at high risk for underlying neurovascular anomalies. Magnetic resonance imaging scans should be performed in order to refer the infant to the appropriate specialist for management.

Reticular infantile hemangiomas with minimal or arrested growth associated with lipoatrophy.

BACKGROUND: Reticular infantile hemangioma (RIH) with minimal or arrested growth (MAG) is an underrecognized variant of infantile hemangioma (IH). OBJECTIVE: We describe a new clinical subtype of RIH-MAG associated with lipoatrophy. METHODS: The medical charts and serial clinical photographs and imaging studies of 53 children given a diagnosis between 2004 and 2013 labeled as "abortive," "minimal or arrested growth," "reticular," or "congenital" hemangiomas were reviewed in the departments of dermatology and infantile plastic surgery of 2 French university hospital centers. RESULTS: Seven children were identified with RIH-MAG associated with lipoatrophy. There were 3 main patterns: (1) a focal cutaneous depression; (2) semicircular lipoatrophy with a horizontal bandlike depression; and (3) segmental soft-tissue atrophy. One case of segmental RIH-MAG of the back of the foot was associated with genital abnormalities reminiscent of perineal hemangioma, external genitalia malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tags (PELVIS) syndrome. LIMITATIONS: The limitations were the retrospective study design, the small number of cases, and selection bias based on recall and photography. CONCLUSION: RIH-MAG with lipoatrophy is an uncommon variant of IH that may be confused with other vascular anomalies, particularly capillary malformation, focal and atrophic cutis marmorata congenita telangiectatica, and rapidly involuting congenital hemangioma with lipoatrophy as a sequela.

Bilateral suprazygomatic maxillary nerve block for cleft palate repair in children: a prospective, randomized, double-blind study versus placebo.

BACKGROUND: The authors investigated the efficacy of bilateral suprazygomatic maxillary nerve block (SMB) for postoperative pain relief in infants undergoing cleft palate repair. METHODS: In this prospective, double-blind, single-site, randomized, and parallel-arm controlled trial, 60 children were assigned to undergo bilateral SMB with general anesthesia with either 0.15 ml/kg of 0.2% ropivacaine (Ropi group) or 0.15 ml/kg of isotonic saline (Saline group) on each side. The primary endpoint was total postoperative morphine consumption at 48 h. Pain scores and respiratory- and SMB-related complications were noted. RESULTS: The overall dose of intravenous morphine after 48 h (mean [95% CI]) was lower in the Ropi group compared with that in the Saline group (104.3 [68.9 to 139.6] vs. 205.2 [130.7 to 279.7] µg/kg; P = 0.033). Continuous morphine infusion was less frequent in the Ropi group compared with that in the Saline group (1 patient [3.6%] vs. 9 patients [31%]; P = 0.006). Three patients in the Saline group had an episode of oxygen desaturation requiring oxygen therapy. There were no technical failures or immediate complications of the SMB. Intraoperative hemodynamic parameters, doses of sufentanil, pain scores, and postoperative hydroxyzine requirements were not different between the two groups. CONCLUSION: Bilateral SMB is an easy regional anesthesia technique that reduces total morphine consumption at 48 h after cleft palate repair in children and the use of continuous infusion of morphine and may decrease postoperative respiratory complications.

New neonatal classification of unilateral cleft lip and palate part 2: to predict permanent lateral incisor agenesis and maxillary growth.

Objectives : To bring a neonatal classification system of unilateral cleft lip and palate and to correlate this classification with the distribution of the permanent lateral incisor and maxillary growth. Design : Retrospective with longitudinal follow-up. Setting : Tertiary. Patients : A total of 112 individuals with treated unilateral cleft lip and palate and 30 controls. Main Outcome Measures : Unilateral cleft lip and palate neonatal casts were classified anatomically in four categories, in which Class 1 corresponds to a maxillary arch with a narrow alveolar cleft; Class 2 corresponds to a balanced form; Class 3 corresponds to a wide cleft and short maxilla; and Class 4 corresponds to a wide cleft and long maxilla. The classification was correlated with the distribution of the permanent lateral incisor. Maxillary growth was evaluated using a cephalometric analysis after the age of 10 years. Results : Clinical classification of unilateral cleft lip and palate found 10 cases of Class 1 (8.9%), 34 cases of Class 2 (30.4%), 46 cases of Class 3 (41.1%), and 22 cases of Class 4 (19.6%). The permanent lateral incisor was most often present in narrower clefts (Classes 1 and 2); whereas, large clefts (Classes 3 and 4) were relatively more frequently associated with an agenesis of the permanent lateral incisor (P = .019). Maxillary growth impairment was most severe in Class 3, with a mean sella-nasion-A point angle at 71.9° ± 4.6° (P < .001). Conclusions : Using the cleft width, arch form, and shape of the nasal septum, unilateral cleft lip and palate can be classified into four different classes at birth, which can all give information about permanent lateral incisor agenesis and maxillary growth.

New neonatal classification of unilateral cleft lip and palate-part 1: to predict primary lateral incisor agenesis and inherent tissue hypoplasia.

OBJECTIVES: To bring a neonatal classification system of unilateral cleft lip and palate (UCLP) and to correlate this classification with the distribution of the primary lateral incisor. DESIGN: Retrospective with longitudinal follow-up. Setting : Tertiary. Patients : One hundred twenty-one patients with treated UCLP. Thirteen plaster casts were used as controls. MAIN OUTCOME MEASURES: The UCLP patients were classified anatomically into four categories: class 1 corresponds to a maxillary arch with a narrow alveolar cleft, class 2 corresponds to a balanced form, class 3 corresponds to a wide cleft and short maxilla, and class 4 corresponds to a wide cleft and long maxilla. Clinical validity was evaluated with a concordance analysis (intra- and interexaminer). This anatomical classification was also corroborated with an automatic classification determined by morphometric parameters measured on neonatal maxillary plaster casts. The class was finally correlated with the distribution of the primary lateral incisor. RESULTS: Clinical classification of UCLP found 12 cases of class 1 (9.9%), 36 cases of class 2 (29.8%), 47 cases of class 3 (38.8%), and 26 cases of class 4 (21.5%). The clinical classification was validated with a good intra- and interexaminer concordance analysis (κ > .6). The automatic classification was close to the clinical classification in 84%. The correlation was ideal in class 1 (100%), almost perfect in class 4 (92%), but lower for class 2 (74%) and class 3 (70%). The primary lateral incisor was usually duplicated in class 1, whereas class 2 and class 4 were correlated with a primary lateral incisor located on the lateral palatal segment. Class 3 was associated with an agenesis of the primary lateral incisor (P < .001). CONCLUSIONS: UCLP can be classified into four different classes at birth, which can all give information about the inherent tissue hypoplasia and the distribution of the primary lateral incisor.

Surgical treatment of arteriovenous malformations of the buttock.

For symptomatic buttock arteriovenous malformations (AVMs), embolization techniques and surgical resection have been suggested as treatment options. Our aim was to evaluate the feasibility and long-term results after a single surgical resection. Twelve patients had undergone surgical resection without preoperative embolization. Of the 12 patients, 11 had had incomplete procedures, 9 of whom had undergone arterial embolization 1 to 3 years previously. All the patients were symptomatic. Computed tomography scans showed AVMs located in the cellular spaces with preservation of the gluteal muscle. The median follow-up time was 80 months. On the last follow-up computed tomography scan, 67% had had no residual AVM. The use of preoperative embolization, especially with nonresorbable embolic material (Onyx; Medtronic, Dublin, Ireland), makes AVM resection and imaging follow-up more difficult because of artifacts and should be avoided.

Classification and pathogenic models of unintentional postural cranial deformities in infants: plagiocephalies and brachycephalies.

Unintentional postural deformities of the skull have increased in a pseudoepidemic manner in the last 15 years. Although dorsal decubitus and prenatal risk factors can play a role in the genesis of such deformities, we think that a crucial determinant is a postnatal defect of cervical mobility responsible for the infant's posture (ie, positional preference) when supine. Indeed, muscular factors, which limit the range of head and neck movements, have been underestimated in the genesis of skull deformities. Here, we have retrospectively analyzed data from 181 infants with unintentional skull deformities and propose a classification of these deformities into 3 types based on their pathogenic model and clinical appearance: fronto-occipital plagiocephalies due to severe muscle hypertonia in which the myogenic component is the first implicated, occipital plagiocephalies with muscle imbalance due to neurogenic muscle hypertonia, and posterior brachycephalies with neurogenic muscle hypertonia of the suboccipital muscles due to trauma to the occipitovertebral junction. Future studies on the size and density of specific muscles or group of muscles should help us to better understand their involvement in the pathogenesis of postural deformities. Our findings also highlight the importance of carefully assessing cervical mobility during the first week of life to detect possible limitations and to prescribe (if needed) an adapted rehabilitation. Rehabilitation should be associated with postural measures put in place when infants sleep supine to prevent the appearance of skull deformations.

Comparison between combined cortical and cancellous bone graft and cancellous bone graft in alveolar cleft: Retrospective study of complications during the first six months post-surgery.

BACKGROUND: In patients with alveolar cleft, alveolar bone graft is usually performed before the permanent maxillary canine or incisor eruption and using cancellous bone harvested from the iliac crest. The authors sought to compare the early complication rate in patients who received cancellous bone graft alone and cortical bone graft in addition to cancellous bone to reconstruct the nasal floor. METHODS: For this observational, retrospective, monocentric study, patients with alveolar cleft who underwent autologous alveolar bone graft and gingivoperiosteoplasty at Montpellier Hospital were divided into two groups: group A, who had cortical and cancellous bone graft; and group B, who had only cancellous bone graft. Both groups were followed for 6 months after surgery, and complications were recorded. RESULTS: In total, 319 grafts (group A, 163; group, 156) were performed from June 1999 to May 2016. The major complication rates were 8% and 5.8% in group A and B, respectively, and were not significantly different (p = 0.52). Similarly, the rates of minor complications and of donor site morbidity were not different between groups. CONCLUSIONS: This preliminary study shows that adding a cortical bone graft for the nasal floor reconstruction does not increase the risk of complications in 6 months following the graft. Long-term benefits such as alveolar height and a piriform aperture symmetrisation will have to be analysed to study the contribution of the technique.

Syndromic sebaceous nevus: current findings.

BACKGROUND: Sebaceous nevus is a congenital malformation of the skin that usually occurs on the scalp or face. Syndromic forms do rarely exist with associated cerebral and ocular malformations. The skin lesions are pale at birth and become irregular by puberty. In the adult patient, tumors (usually benign) develop from sebaceous nevus. Their surgical excision during childhood can give a better result in terms of the definitive scar. OBJECTIVES: The aim of this study is to analyze our cases of syndromic sebaceous hamartoma, perform a review of the existing literature, and propose guidelines for the therapeutic plan. METHODS: This is a retrospective study reviewing the cases of syndromic sebaceous nevus treated in the Department of Orthopedic Plastic Pediatric Surgery in Montpellier, France, and the Department of Pediatric Surgery in Lausanne, Switzerland, between 1994 and 2016. RESULTS: The files of six patients with syndromic sebaceous nevus were analyzed. The average age at the first consultation was 4 months. The location was craniofacial in all cases. Cerebral radiological imaging was performed on all patients; two showed abnormal findings. Four patients underwent ophthalmic examination, which all revealed abnormalities. Three patients had other associated malformations. Three patients presented with epilepsy or learning difficulties in the course of follow-up. CONCLUSION: All patients presenting with extensive sebaceous nevus of the craniofacial region should benefit from cerebral imagery and ophthalmic examination since there is a very high probability of associated abnormalities. The developmental problems encountered could not be definitively associated with the skin malformations.

Early secondary bone grafting in children with alveolar cleft does not modify the risk of maxillary permanent canine impaction at the age of 10 years.

OBJECTIVE: The risk of maxillary permanent canine retention is elevated in children with cleft lip and palate (CLP). This study compared this risk in 10 ± 1 year-old patients with unilateral CLP (UCLP) who underwent or not early maxillary bone grafting at the age of 6 ± 1 years. METHODS: Retrospective, controlled monocentric and observational study with the following inclusion criteria: patients with UCLP, isolated cleft, pre-surgery orthodontic maxillary expansion, same surgical protocol, same surgical team. The risk of maxillary canine retention was evaluated using a dental panoramic radiograph, on the basis of the canine root development stage and of the angle (> or <45°) between the main axis of the maxillary canine and a virtual reference axis parallel to the medial sagittal axis. RESULTS: The impaction risk at the age of 10 was of 14.29% (4/28) for the group who underwent early bone grafting and 21.88% (7/32) for the group without early grafting (p = 0.45). Permanent lateral incisor agenesis and the patient's sex did not change this risk. CONCLUSIONS: Early alveolar bone grafting does not significantly change the risk of permanent maxillary canine retention at 10. Long-term followup of a larger population is needed to confirm this result.

PELVIS Syndrome.

BACKGROUND: Large perineal hemangiomas can be associated with congenital anomalies, including anorectal, urinary tract, spine, and external genitalia malformations. OBSERVATIONS: We describe 2 infants with large sacral and perineal hemangiomas, with severe malformation of the external genitalia with ambiguous genitalia in one case and urinary tract malformations and imperforate anus in the other case. These cases are discussed, along with 9 previously reported cases with similar findings. CONCLUSIONS: Large perineal hemangiomas may constitute a distinctive group of associated anorectal, neurologic, renal or urinary tract, and genital defects. We propose the acronym PELVIS syndrome to emphasize the characteristic findings of this syndrome: perineal hemangioma, external genitalia malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tag.

Median cleft of the upper lip: A new classification to guide treatment decisions.

Median cleft of the upper lip (MCL) is a specific and rare entity on the spectrum of facial clefts. MCL have different clinical expressions and can be either isolated or part of multiple malformations. Confusion still exists regarding the explanation and classification of MCL; some cases have been reported in the literature, but no studies carried out a complete review of the literature. This study reviewed cases of MCL in 2 French units and conducted a systematic review of the literature, in order to derive a new classification. Fourteen patients with MCL in the 2 units and 195 cases in the literature were reviewed. They involved complete (42%), incomplete (49%), and minor forms (9%). Epidemiological and clinical data were collected, from which a classification was derived, based on the type of cleft and its belonging to other syndrome(s). Three main groups were distinguished, namely, isolated MCL, MCL within craniofacial malformations, and MCL with extrafacial malformations. Each group and subgroup was associated with a prognosis and led to specific management. This study reviewed all of the various forms of MCL and their associated anomalies, in order to have a global view of MCL and to derive a useful classification scheme to guide management of care.

Computer-assisted midface reconstruction in Treacher Collins syndrome part 2: soft tissue reconstruction.

INTRODUCTION: Treacher Collins syndrome (TCS) midfacial involvement associate a skeletal hypoplasia centred on the zygoma to a hypoplasia of all surrounding soft tissues layers and an inferolateral lower lid pseudocoloboma. TCS soft tissue hypoplasia, which has not been well studied, continues to bring challenges in both the indication of surgical treatment and the prediction of their results. MATERIAL AND METHOD: From a standard magnetic resonance imaging (MRI) acquisition, we studied qualitatively and quantitatively the prezygomatic fat compartments and the buccal fat pad of two individuals with TCS whose age were 10 and 14 years. In parallel, we studied 20 controls at the same age to obtain a morphometric database of reference and compare our results. TCS soft tissue involvement was correlated to the results of our prior skeletal involvement study. RESULTS: The midfacial fat compartments in TCS are severely hypoplastic, especially in the superficial and lateral compartments of the face (all P's < 0.001). No significant correlation existed between the soft tissue and the skeletal involvement. CONCLUSIONS: To our knowledge, this is the first published study of TCS midfacial fat compartments. Their hypoplasia is an important part of the syndrome's facial deformity. The knowledge of their anatomy, organization and volumetric variation is essential. Their re-establishment is key in the early treatment phases of this syndrome. Using the preoperative data, the morphometric database of reference, and surgical simulation, an appropriate surgical technique, going from an autologous fat graft to a free flap, can then be chose.

Computer-assisted midface reconstruction in Treacher Collins syndrome part 1: skeletal reconstruction.

INTRODUCTION: Treacher Collins syndrome (TCS) is a severe and complex craniofacial malformation affecting the facial skeleton and soft tissues. The palate as well as the external and middle ear are also affected, but his prognosis is mainly related to neonatal airway management. Methods of zygomatico-orbital reconstruction are numerous and currently use primarily autologous bone, lyophilized cartilage, alloplastic implants, or even free flaps. This work developed a reliable "customized" method of zygomatico-orbital bony reconstruction using a generic reference model tailored to each patient. METHODS: From a standard computed tomography (CT) acquisition, we studied qualitatively and quantitatively the skeleton of four individuals with TCS whose age was between 6 and 20 years. In parallel, we studied 40 controls at the same age to obtain a morphometric database of reference. Surgical simulation was carried out using validated software used in craniofacial surgery. RESULTS: The zygomatic hypoplasia was very important quantitatively and morphologically in all TCS individuals. Orbital involvement was mainly morphological, with volumes comparable to the controls of the same age. The control database was used to create three-dimensional computer models to be used in the manufacture of cutting guides for autologous cranial bone grafts or alloplastic implants perfectly adapted to each patient's morphology. Presurgical simulation was also used to fabricate custom positioning guides permitting a simple and reliable surgical procedure. CONCLUSIONS: The use of a virtual database allowed us to design a reliable and reproducible skeletal reconstruction method for this rare and complex syndrome. The use of presurgical simulation tools seem essential in this type of craniofacial malformation to increase the reliability of these uncommon and complex surgical procedures, and to ensure stable results over time.

Effects of growth on maxillary distraction osteogenesis in cleft lip and palate.

PURPOSE: The objective was to analyze the effects of growth on the long-term result of maxillary distraction osteogenesis (DO) in cleft lip and palate (CLP). PATIENTS AND METHODS: Retrospective study of 24 CLP cases with long-term follow-up operated for maxillary DO using the Polley and Figueroa technique: 10 patients were distracted during growth, while 14 patients were operated after their growth spurt. Preoperative (T0), 6-12 months postoperative (T1), and ≥4 years postoperative (T2) cephalometric radiographs were evaluated. A classical cephalometric analysis was used to assess the treatment stability, and a Procrustes superimposition method was performed to assess local changes in the maxilla and the mandible. RESULTS: At T0, the mean age was of 11.9 ± 1.4 years for growing patient, and 17.9 ± 3.5 years for patient treated after their growth spurt (P < 0.001). Between T0 and T1, a greater increase of the SNA was shown in growing patients (P = 0.036), but the relapse was more important between T1 and T2, with a significant decrease of the SNA (P = 0.002) and ANB (P = 0.032) compared to the patients treated after their growth spurt. Although not significant, growing patients showed greater rotations of their palatal plane and mandibular plane. CONCLUSIONS: Maxillary DO in CLP does not correct the growth deficit inherent to the pathology. Overcorrection of at least 20% is advised during growth.

Speech outcomes of early palatal repair with or without intravelar veloplasty in children with complete unilateral cleft lip and palate.

We compared the early speech outcomes of 40 consecutive children with complete unilateral cleft lip and palate (UCLP) who had been treated according to different 2-stage protocols: the Malek protocol (soft palate closure without intravelar veloplasty at 3 months; lip and hard palate repair at 6 months) (n=20), and the Talmant protocol (cheilorhinoplasty and soft palate repair with intravelar veloplasty at 6 months; hard palate closure at 18 months) (n=20). We compared the speech assessments obtained at a mean (SD) age of 3.3 (0.35) years after treatment by the same surgeon. The main outcome measures evaluated were acquisition and intelligibility of speech, velopharyngeal insufficiency, and incidence of complications. A delay in speech articulation of one year or more was seen more often in patients treated by the Malek protocol (11/20) than in those treated according to the Talmant protocol (3/20, p=0.019). Good intelligibility was noted in 15/20 in the Talmant group compared with 6/20 in the Malek group (p=0.010). Assessment with an aerophonoscope showed that nasal air emission was most pronounced in patients in the Malek group (p=0.007). Velopharyngeal insufficiency was present in 11/20 in the Malek group, and in 3/20 in the Talmant group (p=0.019). No patients in the Talmant group had an oronasal fistula (p<0.001). All other outcomes were similar. Despite later closure of the soft and hard palate, early speech outcomes were better in the Talmant group because intravelar veloplasty was successful and there were no fistulas after closure of the hard palate in 2 layers.