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Surgical modifications for microform cleft lip repairs.

Microform cleft lips are very rare forms of cleft lips, and the timing and methods of surgical correction remain controversial. We reviewed our surgical methods used between 1991 and 2003. There were eight such cases, with patients ranging in age from 3 months to 9 years, who underwent primary repairs, and their outcomes were evaluated. Two different surgical procedures were used, either a conventional linear incision, lower small triangular flap insertion, and orbicularis oris muscle reconstruction, or the two small z-flap approach in which the central philtral muscles are repositioned over the affected philtrum by way of two small z-flaps. Six cases underwent the former procedure, and two cases received the latter. The two small z-flap method was used in milder and more subtle skin furrow cases. There was at least 18 months of follow-up with no noticeable sex distinction or timing of the surgery compared with other cleft lip types. Orbicularis oris muscle reconstruction by way of the intra-oral approach together with vermillion repairs using z-plasty demonstrated satisfactory postoperative results such as nostril symmetry, disappearance of the linear furrow and vermillion notches, and reduced prominence in comparison with conventional small triangular flap repairs, which are used for regular primary cleft repairs at our institute. Small z-flap and muscle repositioning cases, both surgically repaired in infancy, were successfully corrected without noticeable scars in most white lips. Our surgical modification for microform cleft lip repairs is recommended for infant patients to avoid cicatrices in most white lips, especially in milder cases.

Child↗

Lip image segmentation using fuzzy clustering incorporating an elliptic shape function.

Recently, lip image analysis has received much attention because its visual information is shown to provide improvement for speech recognition and speaker authentication. Lip image segmentation plays an important role in lip image analysis. In this paper, a new fuzzy clustering method for lip image segmentation is presented. This clustering method takes both the color information and the spatial distance into account while most of the current clustering methods only deal with the former. In this method, a new dissimilarity measure, which integrates the color dissimilarity and the spatial distance in terms of an elliptic shape function, is introduced. Because of the presence of the elliptic shape function, the new measure is able to differentiate the pixels having similar color information but located in different regions. A new iterative algorithm for the determination of the membership and centroid for each class is derived, which is shown to provide good differentiation between the lip region and the nonlip region. Experimental results show that the new algorithm yields better membership distribution and lip shape than the standard fuzzy c-means algorithm and four other methods investigated in the paper.

Algorithms↗

Prevalence of cleft lip and palate in a hospital-based population in the Sudan.

UNLABELLED: Cleft lip and palate represents one of the most common developmental deformities seen in oral surgery clinics. It is usually associated with problems which include not only cosmetic and dental abnormalities, but also speech, hearing and facial growth difficulties. OBJECTIVES: The purpose of the present study was to determine the prevalence of cleft lip and palate in births taking place in hospitals in the Sudan. METHODS: The records of 15,890 Sudanese new-borns delivered at three hospitals during the period from 1997 to 2000 were examined. RESULTS: Thirteen cases of cleft lip and palate were found, demonstrating a prevalence of 0.9 per 1000. There were more girls than boys, with a male:female ratio of 3:10. Fifty-four per cent of the cases had cleft lip with cleft palate, 30% had only cleft palate and the remaining 16% had cleft lip alone. CONCLUSIONS: The present study was confined to hospital-based births in one city, and the true incidence of cleft lip and/or palate in the Sudan is not yet known. Findings differ from reports from other countries in terms of suggesting a higher incidence in girls.

Cleft Lip↗

Donor-site morbidity in buccal mucosa urethroplasty: lower lip or inner cheek?

OBJECTIVE: To evaluate donor-site complications of buccal mucosa urethroplasty and whether there is a difference in morbidity between harvesting the mucosa graft from the inner cheek or the lower lip. PATIENTS AND METHODS: Twenty-four consecutive patients with recurrent urethral strictures were treated with buccal mucosa urethroplasty in our department between September 2002 and April 2004. In 12 patients the graft was harvested from the lower lip or cheek and lower lip (group 1), and in 12 patients from the cheek (group 2). The mean (range) age of patients was 51 (26-66) years in group 1 and 53 (32-75) years in group 2. The mean (range) graft length was 6.2 (2-16) cm in group 1 and 5.7 (2-13) cm in group 2. All patients were followed up using a mailed questionnaire that asked about pain, numbness, difficulties in mouth opening or ingestion, and satisfaction, monthly for the first 3 months and then every 6 months. The mean (range) follow-up was 12.5 (6-23) months. RESULTS: There were no bleeding complications or disturbances in wound healing. All of the patients reported numbness in the area of the mental and buccal nerves, and graft-site tenderness after surgery. In group 1, the pain lasted for a mean (range) of 5.9 (0.5-22) months, compared to 1 (0.1-7) months in group 2 (P = 0.022). Perioral numbness lasted for a mean (range) of 10.3 (0.5-23) months in group 1 and 0.85 (0.1-3) months (P = 0.0027) in group 2. There were no statistically significant differences in problems with mouth opening or food intake between the two groups, but the patients in group 1 seemed to be less satisfied (6/12 patients satisfied) than those in group 2 (11/12 patients satisfied). CONCLUSIONS: Buccal mucosa graft harvesting from the lower lip and the inner cheek are both feasible, but harvesting from the lower lip resulted in a significantly greater long-term morbidity, which resulted in a lower proportion of satisfied patients. This seems to be due to a long-lasting neuropathy of the mental nerve. We therefore have changed our technique entirely from lower lip to inner cheek graft harvesting, whenever possible.

Adult↗

Second primary cancers in patients with carcinoma of the lip.

The occurrence of second primary cancers in patients with lip cancer was evaluated in order to test certain etiologic hypotheses. All cases of lip cancer reported to the Finnish Cancer Registry in 1953-74 (3303 men, 320 women) were followed up for a second (or third) primary cancer through the files of the Registry either to death or to 31 December 1974 (a total of 25 510 person-years). The expected numbers of cases were calculated on the basis of the incidence rates specific for sex, age, time and residence (urban or rural) in Finland. A higher than expected risk of cancer was found among both urban and rural male lip cancer patients; among women the observed number of new primary cancers did not differ from that expected. A significant excess risk among males was noted for cancers of the lung and larynx (rural patients) and for non-melanomatous skin cancer in locations other than the head and neck (urban patients). The association of cancers of the lip, lung and larynx found earlier on a geographic level supports the hypothesis that tobacco smoking is a common risk factor in these cancers. The differences in the relative risks between urban and rural patients, however, suggest that the risk factors in lip cancer in urban areas might be partially different from those prevalent in the rural population. The results do not support the hypothesis that sunlight is an important risk factor in lip cancer.

Adult↗

Potentially malignant and malignant lesions of the lip. Role of silver staining nucleolar organizer regions, proliferating cell nuclear antigen, p53, and c-myc in differentiation and prognosis.

The cellular changes leading to carcinoma of the lip are still not completely understood. This study was carried out on 44 malignant and potentially malignant lesions of the lower lip [30 squamous cell carcinomas (SCC), 7 actinic cheilitis, 3 leukoplakias, and 4 nodal metastases from lower lip SCC]. Silver-stained nucleolar organizer regions (AgNORs) and the immunohistochemical expression of proliferating cell nuclear antigen (PCNA), p53, and c-myc were evaluated on formalin-fixed, paraffin-embedded sections. The results indicate that the size and numbers of AgNORs and the percentage of PCNA-positive cells are sensitive parameters for discriminating between potentially malignant lesions and SCC, and for the prognostic sub-typing of lower lip SCC. Furthermore, while p53 positivity was found more frequently in high-grade carcinomas, p53-positive cellular clones were also found in some potentially malignant lesions, a finding probably related to ultraviolet-related cellular damage. These p53-positive lesions could be considered at higher risk of progression to malignancy than the p53-negative ones, although there is no evidence for this as yet. c-myc positivity was found only in some high-grade carcinomas and metastases, and appeared correlated with the later phases of lip carcinogenesis. The combined evaluation of the proliferation status, together with the changes in p53 and c-myc oncoproteins, might constitute useful markers for the prognostic evaluation of potentially malignant, as well as malignant, lesions of the lip.

Adolescent↗

Trading relations between tongue-body raising and lip rounding in production of the vowel /u/: a pilot "motor equivalence" study.

Articulatory and acoustic data were used to explore the following hypothesis for the vowel /u/: The objective of articulatory movements is an acoustic goal; varying and reciprocal contributions of different articulators may help to constrain acoustic variation in achieving the goal. Previous articulatory studies of similar hypotheses, expressed entirely in articulatory terms, have been confounded by interdependencies of the variables being studied (e.g., lip and mandible displacements). One case in which this problem may be minimized is that of lip rounding and tongue-body raising (formation of a velo-palatal constriction) for the vowel /u/. Lip rounding and tongue-body raising should have similar acoustic effects for /u/, mainly to lower F2. In multiple repetitions, reciprocal contributions of lip rounding and tongue-body raising could help limit F2 variability for /u/; thus this experiment looked for complementary covariation (negative correlations) in measures of these two parameters. An electro-magnetic midsagittal articulometer (EMMA) was used to track movements of midsagittal points on the tongue body, upper and lower lips, and mandible for large numbers of repetitions of utterances containing /u/. (Interpretation of the data was aided by results from area-function-to-formant modeling.) Three of four subjects showed weak negative correlations, tentatively supporting the hypothesis; a fourth showed the opposite pattern: positive correlations of lip rounding and tongue raising. The results are discussed with respect to ideas about motor equivalence, the nature of speech motor programming, and potential improvements to the paradigm.

Female↗

Acoustical behavior of brass player's lips.

The controversial problem of whether a brass player's lip movements are best modeled as outward striking or as upward striking is approached by measuring the phase difference between mouthpiece pressure and lip displacement. In analogy with the phase of the input impedance to a resonant system, the negative/positive value of this phase difference suggests an outward-striking/upward-striking oscillation. On the basis of this speculation, the experimental results revealed the following: (1) Large lip-driven pipes support both outward-striking and upward-striking oscillations because of the lip's high flexibility in the absence of a mouthpiece; (2) mouthpiece-nonresonant tube systems almost support the outward-striking oscillation over a wide frequency range; (3) mouthpiece-resonant tube systems totally support the outward-striking oscillation; (4) the French horn and trumpet exhibit the outward-striking oscillation for the lowest mode and the upward-striking oscillation for higher modes. The oscillation transition in brass instruments, which corresponds well to the change of mouthpiece pressure waveform from a low-pitch, nonsinusoidal one to a higher-pitch, more sinusoidal one, depends mainly on the nonlinear acoustic impedance of the lip opening. If its magnitude is negligible, the cavity coupling between the mouthpiece cup and player's mouth yields a capacitive input impedance that favors the outward-striking oscillation; if its magnitude is significant, the cavity decoupling is likely to yield an inertive input impedance that favors the upward-striking oscillation. Since both the time-varying Bernoulli pressure at the lip opening and the area receiving this pressure are generally apt to increase as the blowing pressure increases, the upward-striking oscillation tends to dominate higher mode tones in brass instruments.

Humans↗

Electrophysiological and behavioral analysis of lip touch as a component of the food stimulus in the snail Lymnaea.

Electrophysiological and video recording methods were used to investigate the function of lip touch in feeding ingestion behavior of the pond snail Lymnaea stagnalis. Although this stimulus was used successfully as a conditioning stimulus (CS) in appetitive learning experiments, the detailed role of lip touch as a component of the sensory stimulus provided by food in unconditioned feeding behavior was never ascertained. Synaptic responses to lip touch in identified feeding motoneurons, central pattern generator interneurons, and modulatory interneurons were recorded by intracellular electrodes in a semi-intact preparation. We showed that touch evoked a complex but characteristic sequence of synaptic inputs on each neuron type. Touch never simply activated feeding cycles but provided different types of synaptic input, determined by the feeding phase in which the neuron was normally active in the rhythmic feeding cycle. The tactile stimulus evoked mainly inhibitory synaptic inputs in protraction-phase neurons and excitation in rasp-phase neurons. Swallow-phase neurons were also excited after some delay, suggesting that touch first reinforces the rasp then swallow phase. Video analysis of freely feeding animals demonstrated that during normal ingestion of a solid food flake the food is drawn across the lips throughout the rasp phase and swallow phase and therefore provides a tactile stimulus during both these retraction phases of the feeding cycle. The tactile component of the food stimulus is strongest during the rasp phase when the lips are actively pressed onto the substrate that is being moved across them by the radula. By using a semi-intact preparation we demonstrated that application of touch to the lips during the rasp phase of a sucrose-driven fictive feeding rhythm increases both the regularity and frequency of rasp-phase motoneuron firing compared with sucrose applied alone.

Animals↗

Histologic features and clinical outcomes of melanomas of lip, haired skin, and nail bed locations of dogs.

Outcomes, signalments, and the relationship of histologic features with the outcome of melanomas located in lip, nail bed, and haired skin of dogs were reviewed. These melanomas were diagnosed as benign or malignant based on histologic features. Melanomas of the lip arose from mucous membrane in most cases. 32 dogs with lip melanomas that had histologic features of malignancy, 22 died because of the tumor within 1 year and 10 were tumor free for at least 1 year following removal. Of 10 dogs with melanomas with benign histologic features on the mucous membrane of the lip, 9 were tumor free for at least 1 year. Of 4 dogs with benign appearing tumors of the haired skin of the lip, 3 were tumor free for at least 1 year. Melanomas with histologic features of malignancy occurred in many locations of haired skin, and 11 of 24 dogs were tumor free for at least 1 year. All nail bed melanomas had histologic features of malignancy and all invaded the third phalanx, but 6 of 14 dogs were tumor free for at least 1 year after amputation of the digit. Among these dogs, the 1-year survival rates for tumors classified as malignant by histologic features were 31% [corrected] for lip, 46% for haired skin, and 43% for nail bed. However, the clinical outcome of an individual malignant tumor could not be predicted accurately by any specific histologic features.

Animals↗

Description of the methodology used in an ongoing pediatric care interventional study of children born with cleft lip and palate in South America [NCT00097149].

BACKGROUND: The contribution of birth defects, including cleft lip and palate, to neonatal and infant mortality and morbidity is substantial. As other mortality and morbidity causes including infections, hygiene, prematurity, and nutrition are eradicated in less developed countries, the burden of birth defects will increase proportionally. METHODS/DESIGN: We are using cleft lip and palate as a sentinel birth defect to evaluate its burden on neonatal and infant health and to assess the effectiveness of systematic pediatric care during the first month and first two years of life in decreasing this burden. The neonatal intervention, consisting of weekly pediatric evaluation and referral to appropriate care, is delivered to about 696 infants born with cleft lip and/or palate in 47 hospitals in South America. Neonatal mortality in this group will be compared to that in a retrospective control group of about 464 infants born with cleft lip and/or palate in the same hospitals. The subgroup of infants with isolated clefts of both the lip and palate (about 264) is also randomized into two groups, intervened and non-intervened, and further followed up over 2 years. Intervened cases are evaluated by pediatricians every three months and referred for appropriate care. The intervened and non-intervened cases will be compared over study outcomes to evaluate the intervention effectiveness. Non-intervened cases are matched and compared to healthy controls to assess the burden of cleft lip and palate. Outcomes include child's neurological and physical development and family social and economic conditions. DISCUSSION: Large-scale clinical trials to improve infant health in developing countries are commonly suggested, making it important to share the methods used in ongoing studies with other investigators implementing similar research. We describe here the content of our ongoing pediatric care study in South America. We hope that this may help researchers targeting this area to plan their studies more effectively and encourage the development of similar research efforts to target other birth defects or infant outcomes such as prematurity and low birth weight.

Cleft Lip↗

Conversational skills of children with cleft lip and palate: a replication and extension.

OBJECTIVE: To replicate and to extend a previous study examining the conversational skills of children with cleft lip and palate. PARTICIPANTS: Thirty-four children (33 to 44 months) participated: 17 children with cleft lip and palate and 17 noncleft children. METHODS: The children were observed during an interaction with caregivers in their homes. Samples of caregiver-child interactions were coded as assertive or responsive, for type of conversational act, and for discourse level categories. Profiles of conversational activity were determined for each child based on the coding. Correlations were performed to examine the relationship between assertiveness and speech variables (articulation and resonance) for the children with cleft lip and palate. RESULTS: Group comparisons revealed that the children with cleft lip and palate produced fewer assertive utterances, were less likely to respond adequately to comments by caregivers, and produced more topic maintaining and fewer topic extending utterances than did their noncleft peers during conversational interactions. Examination of individual child data indicated that 35% of the children with cleft lip and palate exhibited conversational profiles characterized by either low assertiveness or low responsiveness. Finally, a significant positive correlation was noted between conversational assertiveness and speech production skills. CONCLUSION: These findings suggested that the children with cleft lip and palate were less conversationally assertive than their noncleft peers. Further, there appeared to be a relationship between speech production skills and conversational skills, suggesting that poor speech may be impacting language performance for these children.

Assertiveness↗

Population-based study on the practice of breastfeeding in children born with cleft lip and palate.

OBJECTIVE: To investigate the practice of breastfeeding and related difficulties in children born in Porto Alegre, Brazil, with cleft lip, cleft palate, and cleft lip with cleft palate. DESIGN: Cohort, observational, and population-based study. POPULATION AND SAMPLE: Mothers of all children born in 2001 and 2002 with cleft lip and palate in the city of Porto Alegre, Brazil (n = 31), were interviewed in their homes. From the information collected, the frequencies of breastfeeding and exclusive breastfeeding in the first year of life were estimated and the median duration was computed according to the type of cleft. A descriptive analysis was used to study the difficulties. MAIN INDICATORS: Initiation rate, pattern, duration, and difficulties of breastfeeding and exclusive breastfeeding. RESULTS: The initiation rate of breastfeeding was 100% and its median duration was 42.5 days. Exclusive breastfeeding was initiated by 67.7% of the sample and maintained for 15 days (median). The breastfeeding duration was significantly higher in the presence of cleft lip, being equal or even superior to (in the case of exclusive breastfeeding) the median of Porto Alegre's general population. The most frequently mentioned difficulties were weak suction, difficulty attaching to the breast, and breast milk escaping through the nostrils. CONCLUSIONS: In spite of the diverse difficulties reported and the lack of professional support after discharge from the maternity wards, the initiation rate and the duration of breastfeeding of children with cleft lip and palate found in this study reinforce the theory that this malformation, especially cleft lip, is compatible with successful breastfeeding.

Adult↗

Dental age in children with a complete unilateral cleft lip and palate.

OBJECTIVE: To assess dental age in children with a complete unilateral cleft lip and palate and to compare this with a noncleft control group. DESIGN: Two-group, mixed-longitudinal cohort study. SETTING: Cleft group from an academic center for cleft lip and palate treatment. Noncleft control group from the same population. PATIENTS: Participants included 70 Caucasian children with a full complement of teeth and a complete unilateral cleft lip and palate (45 boys and 25 girls) from the Cleft Palate Craniofacial Center at the Radboud University Nijmegen Medical Center, Nijmegen, the Netherlands. The control group (90 boys and 91 girls) was taken from the Nijmegen Growth Study. MAIN OUTCOME MEASURE: Dental age was assessed on orthopantomograms. In the unilateral cleft lip and palate group, linear interpolation in individual age curves was applied to obtain the dental age at 5, 9.5, and 14 years of age. For these ages, a comparison was made with the noncleft control group. RESULTS: Boys and girls with a unilateral cleft lip and palate showed a significant delay in dental age, as compared with their noncleft peers at all three ages. This delay was more pronounced in boys than in girls. The gender effect was significant at chronological ages 5 and 14 years. CONCLUSIONS: Children with a complete unilateral cleft lip and palate have a delay in dental age, compared with noncleft children.

Adolescent↗

Comparison of craniofacial and dentoalveolar morphologies of three Japanese monozygotic twin pairs with cleft lip and/or palate discordancy.

OBJECTIVE: In this study, we analyzed the craniofacial and dentoalveolar morphologies of three pairs of monozygotic twins discordant for cleft lip and/or palate (CL/P) in order to evaluate the effects of environmental factors on growth and development. DESIGN: Craniofacial and dentoalveolar morphologies revealed by analyses of cephalograms and dental casts were compared for each pair of twins discordant for CL/P. SUBJECTS: In case 1, the subjects were 10-year-old male twins, one of whom had a repaired unilateral cleft lip and alveolus and one of whom had an unrepaired unilateral cleft lip. In case 2, the subjects were 13-year-old female twins, one of whom had a repaired bilateral cleft lip and palate and one of whom had an unrepaired unilateral cleft lip. In case 3, the subjects were 9-year-old female twins, one of whom had a repaired unilateral cleft lip and palate and one of whom had no cleft. RESULTS: Cephalometric analysis disclosed distinguished intrapair differences in the maxillary and mandibular morphologies in cases 2 and 3. However, the tooth axes of the incisors in operated subjects were consistently influenced in all three cases. Dental cast analysis indicated that the shapes and sizes of the alveolar and dental arches in the operated subjects were affected in case 2 and, more severely, in case 3, while they looked fairly similar in case 1. CONCLUSIONS: Cephalometric and dental cast analyses demonstrated characteristic intrapair differences between the twins discordant for CL/P according to each cleft type. These morphological differences indicate that surgical closure of clefts may have considerable effects on craniofacial and dentoalveolar growth and development in CL/P patients.

Adolescent↗

Location and distribution of epithelial pearls and tooth buds in human fetuses with cleft lip and palate.

OBJECTIVE: The purpose of this study was to establish the location and distribution of epithelial pearls and tooth buds in cleft palate fetuses, relative to the time of palate fusion. DESIGN: The facial skeletal structures, dental laminae, tooth buds, and epithelial pearls were examined in seven spontaneously aborted human fetuses, of which five had unilateral or bilateral cleft lip and palate or cleft palate. The sectioned fetuses were reconstructed by 3D-computer technology. RESULTS: Epithelial pearls were found in four of the investigated cases, of which one was a control specimen. They were located at the margins of the palatal shelves. In the cleft lip and palate cases, the cleft was found in the premaxilla between the first and second incisor tooth. The premaxilla was found to be hypoplastic in both bilateral cleft lip and palate cases and was totally absent in the unilateral cleft lip and palate case. The maxilla was hypoplastic in one case with unilateral cleft lip and palate. In all other specimens, it was developed symmetrically. CONCLUSIONS: The results indicate that cleft lip and palate development may occur after the fusion of the frontonasal prominence with the maxillary prominence and the palatal shelves, as well as a nonfusion of the palatal shelves in the secondary palate.

Cleft Lip↗

Comparative study between children with and without cleft lip and cleft palate, part 1: cephalometric analysis.

OBJECTIVE: This study was conducted to compare craniofacial relationships, position, and curvature of the cervical spine between children with cleft lip and cleft palate who had been operated on and children without clefts. METHOD: This study was performed in 28 children with mixed dentition. They were divided into two groups. The study group included 14 children with unilateral operated cleft lip and cleft palate, ranging in age from 6 to 12 years, who clinically presented with a short upper lip, abnormal lip seal, and inhibition of sagittal development of the midface that was radiographically assessed. The control group included 14 children without clefts, ranging in age from 8 to 11 years. All of them had normal lip seal, nasal breathing, and a clinically normal body posture. DESIGN: A lateral craniocervical radiograph in a self-balanced natural head position in an erect posture, and without using a head holder, was taken for each child of both groups, with the mandible in maximum intercuspation and lips in habitual posture. The true vertical was marked on all the films. Specific angular and linear dimensions were used to assess the craniocervical relationships, as were the position of the cervical spine, its curvature, or both. RESULTS AND CONCLUSIONS: The study group presented a significant increase in the extension of the head on the neck, forward position of the cervical spine, and a decrease in the curvature of the cervical spine in comparison with the children without clefts. These results are more relevant considering that the study group also presented higher significant values of lower facial height than children without clefts.

Cephalometry↗

Major hematological diseases associated with cleft lip and palate.

OBJECTIVE: Cleft lip and palate is among the most common congenital anomalies. Its association with major blood disorders has rarely been reported. The purpose of this study was to report two patients who had major blood diseases associated with cleft lip and palate. PATIENTS AND RESULTS: From June 1995 to December 1997, there were 2700 patients with cleft lip, cleft palate, or both who received treatment at Chang Gung Memorial Hospital. Two of them were found to have major hematological disorders. In both cases, the disorder was detected by preoperative blood cell counts and white cell differentiation. Case 1 was a 21-year-old woman patient with repaired right cleft lip. She was admitted for alveolar bone grafting and closure of oronasal fistula. Abnormal presentation of blast cells was found, and subsequent bone marrow study confirmed acute lymphocytic leukemia. Case 2 was a 26-year-old man with left secondary cleft lip nasal deformity scheduled to receive staged reconstructive operations. An elevated platelet count was found and subsequently confirmed to represent essential thrombocytosis. In both cases, reconstructive operations for the cleft-related deformities were performed. CONCLUSIONS: Association of major hematological disorders and cleft lip, palate, or both is rare and is reported herein.

Adult↗