Frontal plagiocephaly: synostotic, compensational, or deformational.

Plagiocephaly is a term commonly used to describe congenital forehead asymmetry. Sixty patients with frontal plagiocephaly were evaluated retrospectively and separated into three types: synostotic (N = 24), compensational (N = 3), and deformational (N = 33). Categorization of frontal plagiocephaly as synostotic or deformational was reliably made by physical examination, focusing on the supraorbital rims, nasal root, ears, and malar eminences. Other anatomic parameters useful in the differential diagnosis included chin point, palpebral fissures, and facial height. This study documented that birth histories were similar for synostotic and deformational frontal plagiocephalic infants. However, other deformational anomalies were more common in deformational frontal plagiocephalic infants, whereas malformations had an equal incidence in deformational and synostotic frontal plagiocephalic infants. Torticollis was an associated finding in 64 percent of infants with deformational frontal plagiocephaly; almost all were ipsilateral. In contrast, head tilt, usually to the contralateral side, was noted in 14 percent of patients with synostotic frontal plagiocephaly. Female preponderance was noted in both synostotic (79 percent) and deformational (76 percent) frontal plagiocephaly. Left-sided involvement was seen in 73 percent of patients with deformational frontal plagiocephaly and in 46 percent of patients with synostotic frontal plagiocephaly. Premature pelvic descent, in the left occipital anterior position, may account for the high incidence of left-sided deformational plagiocephaly and ipsilateral torticollis.     

Molecular analysis of patients with synostotic frontal plagiocephaly (unilateral coronal synostosis)

Mutations in genes known to be responsible for most of the recognizable syndromes associated with bilateral coronal synostosis can be detected by molecular testing. The genetic alterations that could cause unilateral coronal synostosis are more elusive. It is recognized that FGFR and TWIST mutations can give rise to either bilateral or unilateral coronal synostosis, even in the same family. The authors undertook a prospective study of patients presenting with synostotic frontal plagiocephaly (unilateral coronal synostosis) to Children's Hospital Boston during the period from 1997 to 2000. Mutational analysis was performed on all patients and on selected parents whenever familial transmission was suspected. Intraoperative anthropometry was used in an effort to differentiate those patients in whom a mutation was detected from those in whom it was not. The anthropometric measures included bilateral sagittal orbital-globe distance, inter medial canthal distance, and nasal angulation. Macrocephaly and palpebral angulation were also considered possible determinants. There was a 2:1 female preponderance in 47 patients with synostotic frontal plagiocephaly. Mutations were found in eight of 47 patients: two patients with different single-amino-acid changes in FGFR2, three patients with FGFR3 Pro250Arg, and three patients with TWIST mutations. Another patient had craniofrontonasal syndrome for which a causative locus has been mapped to chromosome X, although molecular testing is not yet available. Two features were strongly associated with a detectable mutation in patients with synostotic frontal plagiocephaly: asymmetrical brachycephaly (retrusion of both supraorbital rims) and orbital hypertelorism. Other abnormalities in the craniofacial region and extremities were clues to a particular mutation in FGFR2, FGFR3, TWIST, or the X-linked mutation. Neither macrocephaly nor degree of nasal angulation nor relative vertical position of the lateral canthi correlated with mutational detection. An additional four patients in this study had either unilateral or bilateral coronal synostosis in an immediate relative and had anthropometric findings that predicted a mutation, and yet no genetic alteration was found. This suggests either that the authors' screening methods were not sufficiently sensitive or that perhaps there are other unknown pathogenic loci. Nevertheless, molecular testing is recommended for infants who have unilateral coronal synostosis, particularly if there are the anthropometric findings highlighted in this study or an otherwise suspicious feature in the child or a parent. Infants with either an identified or a suspected mutation usually need bilateral asymmetric advancement of the bandeau and may be more likely to require frontal revision in childhood.     

Analysis of fronto-orbital advancement for Apert, Crouzon, Pfeiffer, and Saethre-Chotzen syndromes

The purposes of this study were (1) to document outcome after primary fronto-orbital advancement for the four major eponymous craniosynostotic syndromes (Apert, Crouzon, Pfeiffer, and Saethre-Chotzen) and (2) to identify factors that might influence need for primary and secondary fronto-orbital advancement or foreheadplasty. Also tested was the hypothesis that coincident sagittal synostosis could modulate brachycephaly and affect whether a primary or secondary frontal operation was necessary. Data were collected on age and indications for initial operation, type of primary and secondary frontal procedures, and concomitant sagittal synostosis. Patients initially managed by subcranial Le Fort III were included in the study group but excluded from analysis of fronto-orbital advancement. Patients treated by monobloc advancement or Le Fort III osteotomies with frontal grafting or Anderl modification were assessed as having had primary fronto-orbital advancement. Minimum time to follow-up was 5 years. A total of 126 patients met inclusion criteria. Lateral photographs were examined to assess preoperative and postoperative sagittal position of supraorbital rims-to-globes. Frontal re-advancement was indicated if the corneal apex was anterior to the supraorbital rim. Foreheadplasty was indicated for unacceptable frontal contour and normal supraorbital rim-to-globe relationship. Primary correction for frontal retrusion was not required in 4 percent of Apert (1 of 25), 16 percent of Crouzon (7 of 44), 6 percent of Pfeiffer (2 of 31), and 19 percent of Saethre-Chotzen (5 of 26) patients. Of those infants who had a primary fronto-orbital advancement, reoperation for either supraorbital retrusion or frontal deformity was necessary in all 16 Apert patients and in 5 of 19 Crouzon (26 percent), 10 of 26 Pfeiffer (38 percent), and 13 of 20 Saethre-Chotzen (65 percent) patients (p < 0.001). Age at initial fronto-orbital advancement did not influence reoperative rate. No correlation was found between concomitant sagittal synostosis and necessity for primary or secondary frontal correction (p = 0.22). In summary, phenotypic diagnosis was determinant for outcome as defined by need for secondary fronto-orbital advancement, foreheadplasty, or both. Apert patients had the highest incidence of reoperation for frontal retrusion or forehead contour. Crouzon and Saethre-Chotzen patients were most likely to express a minor phenotype and not require fronto-orbital correction. Coincident sagittal synostosis did not influence frontal projection, as reflected in need for either primary or secondary frontal advancement.     

Unilateral fusion of the frontosphenoidal suture: a rare cause of synostotic frontal plagiocephaly

Unilateral coronal synostosis is the common appellation for premature, one-sided fusion of the frontoparietal suture-the most common cause of synostotic frontal plagiocephaly. However, frontal asymmetry can also result from isolated fusion across the anterior cranial base without involvement of the frontoparietal suture. This article describes three patients with localized synostosis of the frontosphenoidal suture, the medial extension of the coronal ring. Two patients were initially misdiagnosed as having unilateral coronal synostosis and the other as having deformational frontal plagiocephaly. The patients had variable frontal flattening, with depression and recession of the ipsilateral orbital rim. The nasal root was midline or slightly deviated to the contralateral side. The sagittal position of the ipsilateral malar eminence was slightly retruded in one patient and symmetric in the other two. The auricular position was symmetric in the sagittal plane for all patients. In all three patients, computed tomography examination demonstrated a patent frontoparietal suture and fusion of the frontosphenoidal suture (basilar hemicoronal ring). Two patients had involvement of contiguous sutures: one had fusion extending to the sphenoethmoidal suture and the other's involved part of the sphenozygomatic suture. The sagittal suture was midline in all patients. In summary, synostotic frontal plagiocephaly denotes a relatively broad phenotypic spectrum that includes unilateral coronal synostosis and more isolated fusions in the basilar coronal ring. The physical findings resulting from frontosphenoidal synostosis are unique, yet careful evaluation will minimize confusion with other causes of asymmetric frontal flattening. Proper diagnosis necessitates awareness of this uncommon entity and requires focused computed tomographic assessment.     

The boxy nasal tip: classification and management based on alar cartilage suturing techniques

The boxy nasal tip is characterized by a broad, rectangular appearance of the tip lobule on basal view. This manifests anatomically as one of three types: type I, which features an increased intercrural angle of divergence (greater than 30 degrees) and normal domal arc (4 mm or less) manifesting as the tip-defining points; type II, which features an increased angulation of the domes of the lower lateral segments of cartilage, creating a widened domal arc (greater than 4 mm) and normal angle of divergence (30 degrees or less); and type III, which features a combination of increased angle of divergence (greater than 30 degrees) and widened crural domal arc (4 mm or greater). In this article, the available techniques for correction of the boxy tip are reviewed and an algorithmic approach for the management of this problem is demonstrated using the open approach to rhinoplasty. Using an individualized algorithmic approach with intraoperative nasal tip analysis and three nasal tip suture reshaping techniques, consistent aesthetic results can be obtained in the correction of the boxy nasal tip.     

A method for early diagnosis of reduced mobility of hand joints in diabetes mellitus type I

The study aimed at elaborating the technique of an early diagnosis of cheiroarthropathy. The study involved 170 patients with diabetes mellitus type I aged between 16 and 45 years and disease duration ranging from 1 year to 33 years. Advanced cheiroarthropathy with shining waxy skin was diagnosed in 41 patients (group I). No lesions characteristic for cheiroarthropathy was diagnosed in 122 patients (group II) while in 7 patients (group III) only skin lesions without contractures were noted. Proliferative retinopathy was significantly more frequent (p less than 0.001) in the group with cheiroarthropathy--39% to 8%. Mean age of patients of group I is 31.5 +/- 5.9 years, in group II--31.0 +/- 6.6 years. Duration of diabetes mellitus is 17.8 +/- 6.2 and 9.6 +/- 7.2 years respectively (p less than 0.05). An angle of metacarpophalangeal joint of the V finger extension was measured in all patients with goniometer. A significant difference was noted in both groups: 34.4 +/- 8.08 degrees and 56.5 +/- 7.1 degrees, respectively (p less than 0.01). Mathematic models were designed basing on the value of measured angle and duration of the disease. These models facilitate possible risk of cheiroarthropathy. Systematic measurements of metacarpophalangeal joint extension seems valuable means of early diagnosis of diabetic cheiroarthropathy and follow-up of such patients.     

Blood pressure and blood lipids in normotensive patients with diabetes mellitus type I with microalbuminuria]

The study involved 50 normotensive men (means age = 34 years) with diabetes mellitus type I (mean duration of the disease 14 years). Group I included 29 patients with normal albumin excretion with the urine (UAE below 30 mg daily), and group II-21 patients with microalbuminuria (UAE 30-300 mg daily). Both groups were similar in relation to the age and duration of diabetes mellitus. Blood cholesterol was significantly higher in patients of group II than in patients of group I (p = 0.02) similarly to blood triglycerides levels (p = 0.01). Mean arterial pressure was lower in patients of group I than that in patients of group II (94.3 +/- 7.0 vs 99.1 +/- 6.0 mm Hg; p = 0.01). HbA1c was positively correlated with blood cholesterol (p = 0.01) and blood triglycerides levels (p = 0.05).     

Coronal ring involvement in patients treated for unilateral coronal craniosynostosis

The etiopathology of the clinical entity normally referred to as unilateral coronal synostosis is commonly used to connote unilateral fusion of the frontoparietal suture. However, other sutures in the coronal ring may exhibit synostosis concomitant with or independent from frontoparietal synostosis and give rise to similar clinical phenotypes. This study retrospectively analyzes high-resolution computed tomographic data sets to determine patency of sutures within the coronal ring. Computed tomographic scan digital data from 33 infants who subsequently underwent surgical correction of unilateral coronal synostosis were assessed for sutural patency using Analyze imaging software. The frontosphenoidal suture was subdivided into intraorbital frontosphenoidal and extraorbital frontosphenoidal portions, and the patency of the frontoethmoidal suture was also assessed. Patients were sorted into two groups on the basis of the status of their frontosphenoidal sutures: group 1 had patent frontosphenoidal but synostotic frontoparietal sutures (n = 21) and group 2 had both frontosphenoidal and frontoparietal synostoses. Observer reproducibility was tested. The vertical and horizontal dimensions of the bony orbit and the endocranial base deflection angle were measured with the observer blinded with regard to sutural status group. Frontoethmoidal synostosis was not noted in any patients in either group. Two patients had no frontoparietal suture synostosis with isolated intraorbital frontosphenoidal and extraorbital frontosphenoidal suture closures. Suture diagnosis reproducibility was 99 percent. In group 1, the ipsilateral-to-contralateral vertical orbit dimension ratio averaged 1.11, whereas in group 2 it averaged 1.04 (p < 0.05). The ratio of horizontal orbit measurements was not significantly different between groups. In both groups, the endocranial base was deflected ipsilateral to the synostotic frontoparietal suture, with an average angle of 12 degrees in group 1 and 17 degrees in group 2 (p < 0.005). The extent of synostosis along the coronal sutural ring contributes to the dysmorphology of the orbit and the endocranial base deflection in patients whose clinical phenotypic diagnosis is unilateral coronal synostosis.     

Prospective anthropometric analysis of sagittal orbital-globe relationship following fronto-orbital advancement in childhood

Fronto-orbital advancement is a common procedure for correction of supraorbital retrusion in patients with coronal craniosynostosis. The aim of this study was two-fold: to quantitate change in the sagittal orbital-globe relationship following fronto-orbital advancement in childhood and to determine the ratio of skeletal-to-soft tissue movement. Soft-tissue points on the orbital rim, orbitale superius (os), orbitale laterale (ol), orbitale inferius (oi), and nasion (n), referenced to apex corneae (ac), were measured preoperatively and postoperatively by a custom-made anthropometer. Intraoperative bony advancement was measured with a caliper. Patients were selected with uniform advancement at the fronto-nasal suture and laterally at the mortise and tenon. Fifteen patients with syndromic craniosynostosis were included in the study (six male, nine female): Apert (n = 2), Crouzon (n = 5), Pfeiffer (n = 4), Saethre-Chotzen (n = 3), and Boston type (n = 1). Average age at operation was 8.7 years (range, 4.5 to 10.5 years). Age, sex, method of fixation, postoperative interval, diagnosis, and skeletal movement were analyzed for possible effect on the magnitude of soft-tissue advancement. Average intraoperative skeletal advancement was 12.1 mm, and average postoperative soft-tissue movement was 10.3 mm (p < 0.001), measured at the midpoint of the supraorbital rims (os). The soft tissue: skeletal movement ratio was 0.9:1. Os was the only point at which soft-tissue advancement could be predicted (Spearman's rank correlation coefficient = 0.67); soft-tissue changes at ol, oi, and n were unpredictable. Skeletal movement was the only determinant of soft-tissue advancement of all variables tested, i.e., diagnosis, age, sex, previous fronto-orbital advancement, and wire versus plate fixation. We make recommendations for calculating the magnitude of fronto-orbital advancement, based on preoperative anthropometry and a soft-to-hard tissue advancement factor.     

The use of nasal splints in the primary management of unilateral cleft nasal deformity

Primary surgical correction of the cleft lip nasal deformity is routinely performed at the Craniofacial Center at Chang Gung Memorial Hospital. Over time, however, there is a tendency for the lower lateral cartilage to retain its memory and, subsequently, recreate the preoperative nasal deformity. Therefore, it is current practice to use a nostril retainer for a period of at least 6 months to maintain the corrected position of the nose. The aim of this study was to qualitatively assess the benefit of postoperative nasal splinting in the primary management of unilateral cleft nasal deformity. Data from two groups of 30 patients with complete unilateral cleft lips each were retrospectively collected and analyzed. The first group served as a control (no nasal splints), and the second group used the nasal retainer compliantly for at least 6 months postoperatively. All patients had their primary lip repair at 3 months of age. A photographic evaluation of the results when the patients were between 5 and 8 years of age was conducted. The parameters used to assess the nasal outcome were nostril symmetry, alar cartilage slump, alar base level, and columella tilt. The first scores were based on residual nasal deformity, and the second set were based on overall appearance. It was found that the mean scores of residual nasal deformity for all four parameters in patients who used the nasal stent were statistically better than the scores of patients who did not (p values ranged from 0.0001 to 0.005). The overall appearance scores for the four parameters in the patients who used the nasal stent after surgery were also statistically better than the scores for those who did not (p values ranged from 0.0001 to 0.01). The results show that postoperative nasal splinting in the primary management of the unilateral cleft nasal deformity serves to preserve and maintain the corrected position of the nose after primary lip and nasal correction, resulting in a significantly improved aesthetic result. Therefore, it is recommended that all patients undergoing primary correction of complete unilateral cleft deformity use the nasal retainer postoperatively for a period of at least 6 months.     

Idiopathic central precocious puberty in girls as a model of the effect of plasma estradiol level on growth, skeletal maturation and plasma insulin-like growth factor I

Girls suffering from idiopathic central precocious puberty (CPP) may have different levels of estrogenic activity. This study was performed to evaluate the relationship between the estrogenic activity and the hypothalamopituitary activation and the effect of various plasma estradiol (E2) levels on growth, skeletal maturation and plasma insulin-like growth factor I (IGF-I). Fifty-eight girls with CPP were divided into 2 groups: group I with E2 less than 25 pg/ml (13 +/- 1 pg/ml, mean +/- SEM, n = 26) and group II with E2 greater than or equal to 25 pg/ml (52 +/- 3 pg/ml, n = 32). The mean ages at onset and at evaluation were lower in group I (5.9 +/- 0.4 and 6.8 +/- 0.4 years) than in group II (6.8 +/- 0.3 and 8.1 +/- 0.2 years, p less than 0.01), but the durations since onset (greater than 0.5 and less than 2 years) in the two groups were similar. The mean peak luteinizing hormone/follicle-stimulating hormone (LH/FSH) ratios were lower in group I (0.8 +/- 0.2) than in group II (1.7 +/- 0.2, p less than 0.001) and correlated with E2 (r = 0.41, p less than 0.01). The mean height gains during the year preceding the initial evaluation were similar in the two groups (8.7 +/- 0.5 vs. 9.2 +/- 0.4 cm). They were independent of the plasma E2 level. Conversely, the mean plasma IGF-I values were lower in group I (2.4 +/- 0.3 U/ml) than in group II (4.2 +/- 0.6 U/ml, p less than 0.01) and correlated with E2 (r = 0.52, p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)     

Postoperative hypoparathyroidism: long term observation and therapy]

Hundred thirty patients with surgical hypoparathyroidism were followed up. Group I involved 45 patients with mild and group II--85 patients with severe surgical hypoparathyroidism. A delay in vitamin D3 therapy was X +/- SD = 4.2 +/- 8.1 years. A delay in introducing vitamin D3 therapy correlated with the duration of hypoparathyroidism (r = 0.93; 8.9 +/- 9.6 years). Follow up period lasted for 15 years and was 4.3 +/- 3.8 years out of which the attempts to establish ultimate and effective dose of vitamin D3 lasted 1.8 +/- 2.4 years. Dose of vitamin D3 was adjusted 5 times, on the average. Effective daily dose was 4,200-22,500 IU (9,311 +/- 7,252) in group I, and 30,000-195,000 IU (51,385 +/- 32,978) in group II whereas maximum daily dose was 75,000 and 250,000 IU respectively (p < 0.001). Some patients were given 25-OH-D3 in daily doses of 50-225 micrograms or 25(OH)2-D3 in daily dose of 0.10-0.75 micrograms. Calcium oral doses of 400-1600 mg daily were administered to 115 patients. In case of high hypercalciuria (over 350 mg/24 h) hydrochlorothiazide (43 +/- 17 mg a day) or chlorthalidone (60 +/- 22 mg a day) normalized calciuria. Low phosphate diet and aluminium oxide (4.4 +/- 1.7 g a day) were more frequently used in group II. Period of time necessary to establish an effective dose of vitamin D3 is long in patients with surgical hypoparathyroidism. Several dose adjustments are required. Maximum daily vitamin D3 dose required for normocalcemia is approximately higher by 1/3 in the early period of the treatment than the effective maintenance dose. A decrease in diet phosphate content, inhibition of phosphates absorption in the gut or blocking increased calcium loss with the urine are necessary in some patients, only.     

Postoperative mental and morphological outcome for nonsyndromic brachycephaly

Bilateral coronal synostosis causes functional and morphological problems that require fronto-orbital advancement in infancy to correct the brachycephalic deformity and to prevent mental impairment caused by the intracranial hypertension. In this study, 99 children with isolated cases of brachycephaly were prospectively followed to study their preoperative and postoperative mental outcome, which was evaluated using developmental or intelligence quotients. Several factors were analyzed: age before treatment, age at the time of surgery, and the correlation between mental assessments before and after surgery. In a subgroup or patients tested for the FGFR3 P250R mutation (n = 48), mental and morphological assessments were analyzed. Before surgery, mental status was better in the patients tested before 1 year of age (p < 0.001). The preoperative mental assessment always correlated with the postoperative assessment (p < 0.0001). The postoperative mental outcome was better when surgery was performed before the patient reached 1 year of age (p < 0.02). Although both the morphological and functional outcomes were better in the subgroup of noncarriers of the mutation, the differences were not statistically significant. Prominent bulging of the temporal fossae was frequently responsible for poor morphological outcome in carriers of the mutation. This study confirms the need for early corrective surgery before 1 year of age in brachycephalic patients to prevent impairment of their mental development. Suboptimal morphological and mental outcomes can be expected in patients with nonsyndromic brachycephaly who carry the FGFR3 P250R mutation. Primary correction of the temporal bulging should be performed in conjunction with fronto-orbital advancement to improve the morphological outcome in patients with the mutation.     

Cytogenetic analysis of buccal cells from shoe-workers and pathology and anatomy laboratory workers exposed to n-hexane, toluene, methyl ethyl ketone and formaldehyde.

People employed in the shoe manufacture and repair industry are at an increased risk for cancer, the strongest evidence being for nasal cancer and leukaemia. A possible causal role for formaldehyde is likely for cancer of the buccal cavity and nasopharynx. Exfoliated buccal cells are good source of tissue for monitoring human exposure to inhaled and ingested occupational and environmental genotoxicants. To assess the cytogenetic damage related to occupational exposure to airborne chemicals during shoe-making and the processes in pathology and anatomy laboratories, the micronuclei (MN) count per 3000 cells was measured in buccal smears from shoe-workers (group I, n = 22) exposed to mainly n-hexane, toluene and methyl ethyl ketone (MEK) and from anatomy and pathology staff (group II, n = 28) exposed to formaldehyde (FA). Eighteen male university staff were used as controls. The mean time-weighted average (TWA) concentrations of n-hexane, toluene and MEK in 10 small shoe workshops were 58.07 p.p.m., 26.62 p.p.m. and 11.39 p.p.m., respectively. The measured air concentrations of FA in the breathing zone of the anatomy and pathology laboratory workers were between 2 and 4 p.p.m. Levels of 2,5-hexadione (2,5-HD) and hippuric acid (HA), metabolic markers of n-hexane and toluene exposure, respectively, were significantly higher in the urine of workers in group I than in control subjects (p < 0.001 and p < 0.01, respectively). The mean (+/- SD) MN (0/00) [corrected] frequencies in buccal mucosa cells from workers in group I, group II and controls were 0.62 +/- 0.45%, 0.71 +/- 0.56% and 0.33 +/- 0.30%, respectively (p < 0.05 and p < 0.05 compared with controls for group I and group II, respectively). The effects of smoking, age and duration of exposure on the frequency of micronucleated buccal cells from workers in all three groups studied were also evaluated. Overall, the results suggest that occupational exposure to organic solvents, mainly n-hexane, toluene, MEK and FA, may cause cytogenetic damage in buccal cells and that use of exfoliated buccal cells seems to be appropriate to measure exposure to organic solvents.     

Long-term outcome of autogenous rib graft nasal reconstruction.

Whereas reconstruction of the hypoplastic nose with rib grafting is common, the long-term outcomes of nasal growth and aesthetics are unknown. This study assessed nasal morphometrics, patient satisfaction, and the perception of nasal appearance by others up to 15 years after nasal reconstruction using cantilevered autogenous chondro-osseous rib grafting with rigid internal fixation in children. Records of all patients who received nasal rib grafts between 1983 and 1998 by one senior surgeon were reviewed. Patients in this study were operated on before their late teens and had greater than 1-year follow-up including serial photographic documentation. Nasal growth was determined by comparing anthropometric measurements preoperatively, perioperatively, and postoperatively. Patient satisfaction was determined through a questionnaire that addressed memory, donor-site morbidity, and nasal perception. Independent, blinded skilled observers who reviewed frontal and lateral photographs of the preoperative, perioperative, and postoperative intervals assessed nasal aesthetics. Thirty-two patients who underwent 38 rib graft reconstructions of the nasal dorsum and tip at an average age of 8.8 years constitute the study population. Six patients underwent secondary augmentation. The average interval between initial nasal reconstruction and evaluation for this study was 7.9 years. Comparative anthropometric measurements before and after surgery documented increases in both tip projection (2.3 percent) and nasal length (3.0 percent) and a decrease in nasolabial angle (1.9 percent). Patient satisfaction interview response rate was 100 percent of those whom we were able to contact (28 of 32). The average age at interview was 17.2 years. Most patients recalled the operation and denied recollection of pain. Donor-site long-term morbidity was not an issue for 86 percent of patients. Sixty-four percent of patients remembered their preoperative nasal appearance and 89 percent of these preferred the postoperative change and were not concerned with nasal scars or texture. Almost two-thirds of the patients had fixation screws removed from the nasal dorsum because of skin erosion, easy palpability, or visibility. Although several patients expressed a desire to make minor additional changes to their nose, only one of these elected offered presurgical consultation and none have had such surgery. The postoperative nasal appearance compared with that preoperatively was rated as improved for 66.3 percent of responses, 26.5 percent as unchanged, and 7.2 percent as deteriorated. Cantilevered autogenous chondro-osseous rib graft reconstruction of the nasal dorsum is an effective means of reconstruction for the hypoplastic nose in childhood with respect to morphometric measurements, patient self-perception, and the assessment of nasal appearance by others.     

Progression of facial asymmetry in hemifacial microsomia

Hemifacial microsomia is a common craniofacial anomaly, variably affecting structures derived from the first and second pharyngeal arches. Correction of the skeletal deformity in children has been advocated to improve growth potential and reduce secondary deformity. However, contrary reports have suggested that facial asymmetry in hemifacial microsomia does not increase with growth; therefore, skeletal correction can be postponed, even until adolescence. The purpose of this study was to test the hypothesis that facial asymmetry in hemifacial microsomia is progressive. This is a retrospective evaluation of 67 patients with untreated hemifacial microsomia. The patients were categorized as: group I (mandible type I, IIa), n = 38, and group II (mandible type IIb, III), n = 29. Pretreatment posterior-anterior cephalometric radiographs were used to analyze asymmetry by measuring the angle between the true horizontal and the following planes: piriform rim, maxillary occlusal plane, and intergonial angle. Angular measurements were averaged for patients in the deciduous (<6 years), mixed (> or =6<13 years), and permanent dentition (> or =13 years). In group I, angle piriform rim, maxillary occlusal plane, and intergonial angle increased from 7.0, 4.3, and 4.4 to 8.4, 6.6, and 6.1 degrees, respectively [mean age, 4.1 (deciduous) to 8.6 (mixed) to 21.0 (permanent) years]. In group II, angle piriform rim, maxillary occlusal plane, and intergonial angle increased from 9.5, 6.2, and 5.3 to 11.7, 7.6, and 8.0 degrees, respectively [mean age, 3.4 (deciduous) to 8.0 (mixed) years]. These data demonstrate that hemifacial microsomia is progressive and underscores the importance of early surgical correction of mandibular asymmetry in this disorder.     

Delayed cranial vault reconstruction for sagittal synostosis in older children: an algorithm for tailoring the reconstructive approach to the craniofacial deformity

An algorithm for the management of sagittal synostosis in older children who underwent delayed cranial vault reconstruction is presented. This algorithm tailors the surgical approach to the specific craniofacial deformity present in each case. The scaphocephalic deformity characteristic of sagittal synostosis varies significantly when presentation is delayed beyond the first year of life, the time during which reconstruction is usually performed. Sixteen patients with sagittal synostosis who presented after 12 months of age, and were a mean of 3.2 years of age at the time of cranial vault reconstruction, were reviewed. Four patients demonstrated preoperative symptoms and objective findings indicative of increased intracranial pressure, including frequent headaches and emesis, papilledema, or digital markings on computed tomographic scan. Each of the 16 patients underwent either (1) single-stage total vault reconstruction with or without concomitant fronto-orbital expansion; (2) two-stage total vault reconstruction with anterior two-thirds vault expansion followed by transverse occipital expansion and recession a mean of 8.7 months later; or (3) anterior two-thirds vault reconstruction with or without fronto-orbital expansion. In each case, the extent of the scaphocephalic deformity determined the procedure used. The presence of severe frontal bossing associated with transverse restriction of the orbitotemporal region was an indication for fronto-orbital expansion in addition to vault reconstruction, whereas significant occipital protrusion was an indication for transverse posterior vault expansion and recession in addition to anterior two-thirds vault reconstruction. Excellent aesthetic results were obtained in all cases regardless of the type of reconstruction performed. However, it is essential that the extent of the deformity be carefully evaluated preoperatively to permit selection of the appropriate technique for reconstruction.     

Effect of atrio-ventricular conduction on the appearance of supraventricular arrhythmia and thrombotic complications in patients with chronic stimulation of cardiac ventricles in sick sinus syndrome]

Two hundred three patients with sick sinus node disease were treated with continuous ventricular stimulation between 1981 and 1985. To 1988, 168 patients aged between 26 and 88 years were followed-up for 5.1 years on the average. All these patients were divided into two groups: I (93 patients) with sinusal bradycardia, and group II (93 patients) with brady-tachycardia. Ventriculo-atrial conduction was seen in 82.61% of patients of group I in whom the implantation of electric stimulator produced the attacks of atrial fibrillation, and in 44.23% of patients without such attacks (p < 0.01); in 80.77% of patients of group II in whom atrial fibrillation became stable with time, and in 50.57% with intermittent atrial fibrillation (p < 0.01) ventriculo-atrial conduction was noted. It may be concluded, that the presence of ventriculo-atrial conduction in patients with prolonged stimulation of the cardiac ventricles favor the occurrence and stabilization of the paroxysmal atrial fibrillation and thrombotic complications.     

Postoperative hypoparathyroidism: Direct para-operative period]

Surgical hypoparathyroidism is a severe complication of the operations on thyroid gland. Hundred thirty patients aged between 17 and 77 years have been analysed clinically. Group I involved 45 patients with surgical hypoparathyroidism of transient or partial character, group II involved 85 patients with severe irreversible surgical hypoparathyroidism. Hypocalcemia in postoperative period was significantly higher in group II than that in group I (p < 0.02), and negatively correlated with the maximal dose of vitamin D3 necessary to compensate calcemia (r = -0.42). Follow-up results indicate that the age and sex of patients, preoperative diagnosis, radiotherapy, and type of surgery have no effect on the degree of damage to parathyroid glands, tetany development rate, degree of hypocalcemia and hyperphosphatemia. High degree hypocalcemia diagnosed in the early postoperative period suggests irreversible injury to parathyroid glands. Management of patients with postoperative tetany requires a close cooperation of surgeon and endocrinologist.     

Idiopathic scoliosis and concentrations of zinc,copper, and selenium in blood plasma

The concentration of zinc, copper, selenium, albumin, and ceruloplasmin in blood plasma and the activity of superoxide dismutase and glutathione peroxidase in erythrocytes were determined in a set of patients with idiopathic scoliosis (n=51). A significant decrease of selenium concentration (0.50±0.16 μmol/L) was found when compared with a control group (0.69±0.07 μmol/L) (p<0.01). The same levels of significance were found out for selenium levels corrected for albumin content. In a group of patients with a curvature over 45° indicated for a surgical correction, the average plasma concentrations of selenium were significantly lower (p<0.05) in comparison with a group of patients with a curvature below 45° treated conservatively. The GSH-Px activity in erythrocytes was the same in both sets. In comparison with the controls, no significant differences were revealed in all of the other parameters. The detection of the decreased blood plasma concentration of selenium has suggested possible disturbance of well-proportioned distribution and of general optimal availability of selenium in the organism of patients with idiopathic scoliosis with likely effects on the process of synthesis and maturation of collagen affecting the axial skeleton stability.