Comparing the treatment protocols for cleft lip and palate requires a study of facial growth and development. Serial orthodontic study models aid in delineating the effects of surgery on maxillary mandibular relationships. In 1978, a new protocol, POPLA (presurgical orthopedics followed by periosteoplasty and lip adhesion), was developed and put into practice. This article compares the results obtained using POPLA (group I) with those using the previous method (lip adhesion alone) (group II) for cases of unilateral and bilateral clefts. Complete orthodontic study models were available for 124 patients, 63 in group I and 61 in group II. Specific timing sequences and surgical details are discussed. Dental cast analyses evaluated alveolar gap, arch width, anteroposterior distance, incisor crossbite (single tooth and multiple tooth), and buccal crossbite (single and multiple tooth) at birth and at 3, 6, and 9 years of age. X-ray studies evaluated the bony bridge. Additional comments are made regarding the incidents and effects of pharyngeal flaps and bone grafts on maxillary-mandibular relationships. Results included less buccal crossbite in the POPLA group, with a wider transverse distance of the upper dental arch. There was a greater frequency of anterior crossbite in the POPLA group, and the anteroposterior distance was shorter at 6 years of age but less so by age 9. This may be because of the different orthodontic care received by the patients in the two groups. In conclusion, the POPLA approach achieves the main goal of moving the palate into a normal position and stabilizing the arch with a bony bridge that attracts teeth. It avoids the difficult anterior fistulae and presents a more symmetrical platform upon which the lip can be united and the nose can be corrected early.

Infants with congenital craniofacial malformations often have associated severe mandibular hypoplasia causing obstruction of the hypopharynx by retroposition of the base of the tongue into the posterior pharyngeal airway. Management depends on the severity of the airway obstruction. Most cases can be managed by prone positioning until the infant outgrows the problem at 3 to 6 months of age. In more critical cases, monitoring of oxygen saturation, temporary placement of a nasopharyngeal tube, and placement of an endotracheal tube will be useful procedures. Tracheotomy is an effective method for more severe cases, but longstanding tracheotomies result in high morbidity and occasional mortality. Mandibular distraction was performed in seven patients, ranging in age from 1 to 18 months, with critical obstructive apnea secondary to mandibular hypoplasia characterized by an apnea/hypopnea index greater than 20 apneas per hour and oxygen saturation below 80 percent. Two patients were tracheotomized previously. Mandibular lengthening, from 16 to 25 mm on the left side and from 10 to 22 mm on the right, was achieved in 21 to 25 days. Improvement of airway obstruction parameters was measured on polysomnograms and lateral cephalograms. Mandibular lengthening by gradual distraction is a successful method for young patients with severe mandibular hypoplasia causing critical obstructive apneas. Avoidance of tracheotomy or early decannulation in previously tracheotomized patients is a great advantage for patients with congenital craniofacial malformation.

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Primary nasal correction at the time of rotation-advancement of the lip has been rendered practical by developing a symmetrical nasal platform by presurgical orthodontics and a gingivoperiosteoplasty. The nasal correction involves unilateral columella lengthening, alar cartilage positioning, and alar base cinching to present a nose within normal limits. This is accomplished before the age of recall, and by avoiding retention of the nasal deformity through childhood, it by-passes the ridicule that invariably accompanies it.

Background: Poland Syndrome (PS) is a rare disorder characterized by hypoplasia/aplasia of the pectoralis major muscle, variably associated with thoracic and upper limb anomalies. Familial recurrence has been reported indicating that PS could have a genetic basis, though the genetic mechanisms underlying PS development are still unknown. Case presentation: Here we describe a couple of monozygotic (MZ) twin girls, both presenting with Poland Syndrome. They carry a de novo heterozygous 126 Kbp deletion at chromosome 11q12.3 involving 5 genes, four of which, namely HRASLS5, RARRES3, HRASLS2, and PLA2G16, encode proteins that regulate cellular growth, differentiation, and apoptosis, mainly through Ras-mediated signaling pathways. Conclusions: Phenotype concordance between the monozygotic twin probands provides evidence supporting the genetic control of PS. As genes controlling cell growth and differentiation may be related to morphological defects originating during development, we postulate that the observed chromosome deletion could be causative of the phenotype observed in the twin girls and the deleted genes could play a role in PS development.

Amniotic band syndrome has been proposed as a sequela of intrauterine rupture of the amnion, resulting in oligohydramnios and passage of the fetus into the chorionic cavity. Amnion disruption with loss of amniotic fluid, causing fetal compression and localized fetal ischemia, possibly results in a pathogenic mechanism of extremely variable malformations. The prominence of the nasal processes and the adjacent stomodeal orifice facilitates free band attachment and adherence, resulting in a spectrum of similarly oriented facial defects. The authors present six consecutive cases of amniotic band syndrome with cleft lip and palate (facial cleft 3, 5, 7, and 10, isolated or combined) that were associated with other craniofacial anomalies, such as craniosynostosis and hypertelorbitism. They also present limb malformations and discuss the proposed pathogenesis and the surgical challenges in functional and aesthetic restoration.

Adding the Mulliken approach allows alar cartilage manipulation to be performed more easily than when using the retrograde approach alone. Tip projection and alar base narrowing are facilitated using the combined technique rather than the Mulliken approach alone. Prolabial flap manipulation is safe using this combined approach, even in cases with a severely projected premaxilla. We believe that the combined approach is safe and yields better long-term results than either technique alone.

The Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and airway obstruction. Conservative management in Pierre Robin sequence consists of nasogastric tube feeding and positioning of the neonate (prone or lateral position) that facilitates the anterior position of the tongue or the application of continuous positive nasal pressure. In case of failure of this treatment, emergency tracheostomy and/or mandibular distraction must be performed.Mandibular distraction is a standard technique used by craniofacial surgeons to achieve an anteroposterior horizontal lengthening of the mandibular body, correcting the posterior position of the base of the tongue and thus retropharyngeal enlargement of the airway.The authors present 2 clinical cases of hypertrophy of the sublingual salivary glands with the use of mandibular distractors in SPR patients with severe airway obstruction.