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A patient with Noonan syndrome with multiple lentigines (NSML) and multiple giant cell lesions (MGCL) in mandibles and maxillae is described. A mutation p.Thr468Met in the PTPN11-gene was found. This is the second reported NSML patient with MGCL. Our case adds to the assumption that, despite a different molecular pathogenesis and effect on the RAS/MEK pathway, NSML shares the development of MGCL, with other RASopathies.

Summary The results of reconstruction of residual alveolo-palatal bone defects in 40 patients are evaluated clinically and radiographically in a retrospective study. Age at the time of surgery varied from 7.4 to 32.7 years. The patients were divided into two age groups: before and after 12 at the time of operation. Free autogenous anterior iliac crest bone grafts were used in 26 patients and free autogenous mandibular symphyseal bone grafts were used in 14 patients. The results show unsuccessful closure of the oronasal communication in the alveolo-palatal area of the cleft in 3 out of 31 patients. In 3 out of 40 patients, radiographic incorporation of the bone graft was unsuccessful. The radiographic success was not related to increasing experience of the surgeons. The study indicates that the best grafting results are achieved when grafting is performed before 12 years of age. The radiographic results of mandibular symphyseal bone grafts tend to be slightly better compared with results obtained with anterior iliac crest bone.

In a retrospective study (mean follow-up 76 months), the use of a free autogenous rib graft for reconstruction of anterior residual bone defects in seventeen cleft patients is evaluated on a long term basis. The results show recurrence of a palatal oronasal fistula in 2 out of 17 patients. 6 patients had acceptable results, while 9 had good to excellent scores at all sites. The costal graft material does not prevent permanent upper cuspids from erupting into this bone, after its incorporation. It is concluded that autogenous rib bone is a good material for secondary bone grafting of the residual alveolar and palatal defect in cleft patients.

A re-evaluation is presented more than a decade after the 1973 review by Koberg of bone grafting in cleft palate. The various indications for, and aims of the procedure, are enumerated. The optimal age for grafting is discussed as well as operative procedures. Results of a few more detailed studies are included in the review. In the conclusion the present shifts of emphasis are mentioned: An in depth analytical study of numerical comparisons between the many studies covering this subject is hardly appropriate. Initially, primary and early secondary osteoplasties became very popular, thereafter late secondary or tertiary osteoplasty found general favour. At present a clear trend exists to operate at a younger age again: secondary osteoplasty being performed at 6-12 years of age. However, in a number of cleft centres primary osteoplasty remains in favour. The overall results of the different procedures appears to continue to improve, though the ideal solutions are still not exactly known, nor generally agreed upon. For the time being, a good overall result should be obtained in more than 80% of cases, complete failures should not be seen in more than 5% of a series. Autogenous bone appears to be by far the best graft material. Disagreement exists on the viability of autogenous bone from different donor sites. Periodontal criteria and parameters are used more frequently in a number of recent publications for assessment of the results of the different procedures. Osteoplasty has a relatively high chance of success, especially in the younger age groups. In most institutions, however, too early an age at operation is considered to cause disturbance in growth and development of the middle third of the face. Nevertheless, in this respect operative technique and/or orthodontic treatment seem to play an important role.

In a retrospective study of 63 patients, an attempt is made to determine in how many cases retained permanent upper incisors erupt after the removal of supernumerary teeth, and what predisposing and etiological factors are of importance. It proved that the eruption of a supernumerary tooth positively contributed to the chance of a spontaneous eruption of the permanent incisors. No relationship could be demonstrated between the age of a patient from whom supernumeraries were extracted, the number of supernumeraries, the extent of root formation, marked apical displacement and the chance of spontaneous eruption of the permanent incisors. It also proved that only in 1 out of 2 children did spontaneous eruption occur after removal of the supernumeraries. It therefore seems advisable to provide all impacted upper incisors with a cervical ligature or bracket with twined wire sling during the first operation in order to create the possibility for orthodontic traction if spontaneous eruption of the incisor does not occur, and thus to prevent a second operation for the child.

The technique and results of replacement of the articular disc by autogenous aural cartilage are presented. Out of 7 patients operated with a follow-up longer than 6 months, 5 have markedly improved. The cartilage seems to remain vital. It is concluded that this procedure may be indicated in patients with dysfunction and/or apparent damage of the disc. However, it is difficult in some cases to base the indication on more than circumstantial evidence.