Distichiasis and cleft palate

A patient with the unusual coexistence of distichiasis and cleft palate is described. Distichiasis is a rare congenital eyelid anomaly in which accessory eyelashes are present in the meibomian gland orifices. Its association with other systemic abnormalities is reviewed; the distinctions among distichiasis, trichiasis, entropion, and epiblepharon are outlined; and methods of treatment for distichiasis are described.     

D-penicillamine-induced cleft palate in mice

The teratogenic potential of the lathyrogen, D-penicillamine (DP), was assessed in pregnant mice, especially with respect to its ability to produce cleft palate. The dosage and the duration of treatment as they relate to the induction of cleft palate were also studied. Two different doses of DP were administered orally for either 5 or 4 consecutive days during the critical period of palatal closure. D-penicillamine (DP) at a dose level which does not have any apparent maternal toxic effects produced cleft palate in the offspring, and this teratogenic effect depended more upon the duration of treatment than the dosage administered. Inhibitory effects on the formation of bone matrix were observed at the base of the palatal shelf. It is suggested that DP is potentially an osteolathyrogenic agent. The mechanism of induction of cleft palate in DP-treated mice was explored by histological studies using light microscopy. Delayed elevation of the palatal shelves was observed and is considered to be the cause of the induction of cleft palate. No other external malformations could be detected in DP-treated fetuses.     

Sex-linked inheritance of cleft palate

Summary A family pedigree is reported in which males in four generations are affected with midline isolated cleft palate. This is the second report of cleft palate inherited as a sex-linked recessive trait, and it emphasizes the importance of careful pedigree construction when counseling families of children afflicted with this disorder.     

Short stay after cleft palate surgery

Although algorithms for the repair of soft and hard palatal clefts continue to be debated, the appropriate length of postoperative stay has not yet been defined. Recent reports of cleft palate repair advocate a 2- to 5-day hospitalization. The plastic surgery service at St. Joseph Hospital frequently uses same-day admission with 23-hour observation postoperatively, with no increase in complications from the reported 2- to 5-day stay.The authors inspected the records for all the cleft palate patients undergoing cleft repair at St. Joseph Hospital Cleft Clinic from August of 1988 through June of 1998. After excluding syndromic patients and secondary or revision surgical cases, 79 patients remained in the study. These 79 patients underwent 104 procedures; all procedures were performed by a single surgeon (E.D.C.) with resident assistance. Short-term morbidity, length of stay, and operation performed were studied. All patients were admitted the day of surgery.Mean age at the time of operation was 13.2 months, with a range of 6 months to 20 years. The length of operation averaged 1 hour and 37 minutes; 94 percent of patients stayed 24 hours or less postoperatively, and 97 percent stayed 36 hours or less. The longest stay was 72 hours, which was related to delay in resuming adequate oral intake. The overall complication rate was 3.8 percent for this cohort, which included two partial palatal dehiscences and two small fistulas. No blood transfusions were needed, and no infections were noted postoperatively. No patients required readmission postoperatively for bleeding, respiratory compromise, or inadequate oral intake.The authors do not advocate a 1-night stay for all cleft palate cases. However, they do think it is safe for a healthy group of patients undergoing routine cleft palate surgery. The decision to discharge a patient early must always be left to the treating physician.     

Binderoid complete cleft lip/palate

A small subset of infants with complete cleft lip/palate look different because they have nasolabiomaxillary hypoplasia and orbital hypotelorism. The authors' purpose was to define the clinical and radiographic features of these patients and to comment on operative management, classification, and terminology. The authors reviewed 695 patients with all forms of incomplete and complete cleft lip/palate and identified 15 patients with nasolabiomaxillary hypoplasia and orbital hypotelorism. All 15 patients had complete labial clefting (5 percent of 320 patients with complete cleft lip/palate), equally divided between bilateral and unilateral forms. The female-to-male ratio was 2:1. Of the seven infants with unilateral complete cleft lip/palate, one had an intact secondary palate and all had a hypoplastic septum, small alar cartilages, narrow basilar columella, underdeveloped contralateral philtral ridge, ill-defined Cupid's bow, thin vermilion-mucosa on both sides of the cleft, and a diminutive premaxilla. Of the eight infants with bilateral complete cleft lip, one had an intact secondary palate. The features were the same as in patients with unilateral cleft, but with a more severely hypoplastic nasal tip, conical columella, tiny prolabium, underdeveloped lateral labial elements, and small/mobile premaxilla. Central midfacial hypoplasia and hypotelorism did not change during childhood and adolescence. Intermedial canthal measurements remained 1.5 SD below normal age-matched controls. Skeletal analysis (mean age, 10 years; range, 4 months to 19 years) documented maxillary retrusion (mean sagittal maxillomandibular discrepancy, 13.7 mm; range, 3 to 17 mm), absent anterior nasal spine, and a class III relationship. The mean sella nasion A point (S-N-A) angle of 74 degrees (range, 65 to 79 degrees) and sella nasion B point (S-N-B) angle of 81 degrees (range, 71 to 90 degrees) were significantly different from age-matched norms ( = 0.0007 and = 0.004, respectively). The ipsilateral central and lateral incisors were absent in all children with unilateral cleft, whereas a single-toothed premaxilla was typically found in the bilateral patients. Several modifications were necessary during primary nasolabial repair because of the diminutive bony and soft-tissue elements. All adolescent patients had Le Fort I maxillary advancement and construction of an adult nasal framework with costochondral or cranial graft. Other often-used procedures were bony augmentation of the anterior maxilla; cartilage grafts to the nasal tip and columella; and dermal grafting to the median tubercle, philtral ridge, and basal columella. Infants with complete unilateral or bilateral cleft lip/palate in association with nasolabiomaxillary hypoplasia and orbital hypotelorism do not belong on the holoprosencephalic spectrum because they have normal head circumference, stature, and intelligence, nor should they be referred to as having Binder anomaly. The authors propose the term cleft lip/palate for these children. Early recognition of this entity is important for counseling parents and because alterations in standard operative methods and orthodontic protocols are necessary.     

Genetics of cleft lip and cleft palate in China.

During the past 10 years, 60 cases of cleft lip with or without cleft palate [CL(P)] were recorded among 45,072 newborns at Shanghai International Peace Maternity and Infant Hospital, China. The incidence was 1.33 per 1,000 births. The family histories of 163 CL(P) patients were analyzed. The incidences of CL(P) in the first-, second-, and third-degree relatives of CL(P) patients were 11/246 (4.47%), 10/1,032 (0.97%), and 6/1,727 (0.35%), respectively. Of the 163 probands, three had a history of consanguinity of the parents (1.8%), in contrast to 0.77% in the general population. These data are suggestive of multifactorial inheritance. The heritability of CL(P) in our study calculated by Falconer's formula was 77.6%.     

A technique for cleft palate repair

The author has developed a technique of palate repair that combines minimal hard palate dissection with radical retropositioning of the velar musculature and tensor tenotomy. The repair is performed under the operating microscope. Results are reported for 442 primary palate repairs performed between 1978 and 1992 inclusive, with follow-up of at least 10 years. In 80 percent of these palate repairs, repair was carried out through incisions at the margins of the cleft and without any mucoperiosteal flap elevation or lateral incisions. Secondary velopharyngeal rates have decreased from 10.2 to 4.9 to 4.6 percent in successive 5-year periods within this 15-year period. Evidence from independent assessment of speech results in palate re-repair and submucous cleft palate repair suggests that this more radical muscle dissection improves velar function.