Complete sternal cleft associated with right clavicular, manubrial, and thyroid hypoplasia, pectus deformity, and spinal anomalies.

Sternal cleft anomalies are rare. Associated anomalies include pentalogy of Cantrell and posterior fossa abnormalities, hemangiomas, arteriopathy, cardiac anomalies, eye abnormalities, and sternal defects syndrome. There is only a single report of complete sternal cleft, pectus excavatum, and right clavicular hypoplasia in an adult. Thyroid hemiagenesis is also very rare. To our knowledge, this is the first case of complete sternal cleft, pectus deformity, and right clavicular hypoplasia in a child and the first case with right thyroid hemiagenesis.

Relevance of discrete traits in forensic anthropology: From the first cervical vertebra to the pelvic girdle.

In forensic anthropology, identification begins by determining the sex, age, ancestry and stature of the individuals. Asymptomatic variations present on the skeleton, known as discrete traits, can be useful to identify individuals, or at least contribute to complete their biological profile. We decided to focus our work on the upper part of the skeleton, from the first vertebra to the pelvic girdle, and we chose to present 8 discrete traits (spina bifida occulta, butterfly vertebra, supraclavicular nerve foramen, coracoclavicular joint, os acromiale, suprascapular foramen, manubrium foramen and pubic spine), because they show a frequency lower than 10%. We examined 502 anonymous CT scans from polytraumatized individuals, aged 15 to 65 years, in order to detect the selected discrete traits. Age and sex were known for each subject. Thin sections in the axial, coronal and sagittal planes and 3D volume rendering images were created and examined for the visualization of the selected discrete traits. Supraclavicular foramina were found only in males and only on the left clavicle. Coracoclavicular joints were observed only in males. The majority of individuals with a suprascapular foramen were older than 50 years of age. Pubic spines were observed mostly in females. Other traits did not present significant association with sex, age and laterality. No association between traits was highlighted. Better knowledge of human skeletal variations will help anthropologists come closer to a positive identification, especially if these variations are rare, therefore making them more discriminant.

The role of the external auditory canal in the development of the malleal manubrium in humans.

OBJECTIVE: To determine if the external auditory canal (EAC) plays a role in the induction and proper positioning of the malleal manubrium in humans. STUDY DESIGN: Retrospective study between 1994 and 2002. SETTING: Academic, tertiary care referral medical center. Patients Fifty-five ears of 50 patients with congenital atresia (n = 47) or stenosis (n = 8) of the EAC, for which meatoplasty was performed at the University hospital between 1994 and 2002. MAIN OUTCOME MEASURES: The presence of the manubrium was examined during surgery, and the corre-lation between the presence of the manubrium and the grade of the microtia was evaluated. RESULTS: The manubrium was identified in all ears with EAC stenosis, whereas it was absent in all ears with EAC atresia. No correlation was observed between manubrium formation and auricular deformity. CONCLUSIONS: Our results demonstrated a close relationship between the formation of the EAC and that of the malleal manubrium in humans. This is consistent with the recent findings in knockout mice. This information is useful for surgical intervention in cases of congenital EAC anomalies.

Less extensive techniques for repair of pectus carinatum: the undertreated chest deformity.

BACKGROUND: Although patients with pectus carinatum (PC) often experience moderate to severe symptoms, there are sparse published data about the indications for correction, the newer techniques of surgical repair, and the results. This study reviews clinical experience with new, less extensive, open operative techniques for repair of PC. STUDY DESIGN: Since 1970, 154 patients (119 men and 35 women) with symptomatic PC (mean severity index 1.76) underwent correction at the UCLA Medical Center using modifications of the Ravitch repair. The last 60 consecutive patients had a less extensive open repair with resection of only small chips of cartilage medially and laterally, and suture reattachment of the remaining costal cartilages to the corrected sternum and ribs. For patients with chondromanubrial protrusion, two or three sternal osteotomies were used, and for those with concomitant lower sternal depression, a lower sternal wedge osteotomy was used. For most patients, a temporary support bar anterior to the sternum and cartilages was used for 6 months. RESULTS: Each of the 154 patients with reduced endurance or dyspnea with mild exercise experienced marked improvement within 6 months. There were no major recurrent deformities; six patients underwent minor surgical revision of localized persistent costal cartilage protrusion or depression. Postoperative complications in the last 60 patients were minor and less frequent, pain was less severe, hospitalization was shorter (mean 2.5 days), and postoperative results were better than when more extensive repairs were used in previous years. With a mean overall followup of 7.9 years, more than 97% of all patients experienced a very good or excellent result. CONCLUSIONS: New, less extensive, open techniques for repair of PC have low morbidity, short hospital stay, and very good physiologic and cosmetic results.

Manubrium sterni in patients with diastrophic dysplasia--radiological analysis of 50 patients.

BACKGROUND: Patients with diastrophic dysplasia (DD) have many typical and some peculiar radiological findings in their skeleton. Currarino reported recently that 6 of his 12 patients with DD had accessory ossification centers of the manubrium sterni. OBJECTIVES: This study analyzed abnormalities of the manubrium sterni in patients with DD to find out more about the prevalence and natural history. MATERIALS AND METHODS: A total of 50 patients with DD (22 male, 28 female) were included in this retrospective analysis. The average age of patients at the time of the first radiograph,was 7.1 years (range: newborn-34.7 years). Lateral radiographs of the spine or the chest were included. Follow-up was of 20 patients (40%) with an average age of 8 years (range: 1-24 years). The number, location, and time of the radiological fusion of accessory ossification centers were analyzed. Additionally, the form of the manubrium was evaluated using an arbitrary scale with three grades. RESULTS: Accessory ossification centers were seen in the first radiograph of 16 (32%) patients. The first radiographs of 16 patients were taken before the age of 18 months; 13 (81%) of them had accessory ossification centers. The most common finding was an accessory ossification center located ventral to the cranial part of the manubrium (a double-layered manubrium). In older patients, the manubrium was usually bulging anteriorly, resembling an asymmetric club. It was as if there had been an accessory ossification center that had fused with the main part of the manubrium. CONCLUSIONS: The manubrium sterni is abnormal in almost all patients with DD. The prevalence of accessory ossification centers in childhood is high. The presence of an accessory ossification center ventral or cranial to the manubrium in radiographs may help in making the diagnosis of DD.

Tassier cleft no 30 (median cleft from lower lip to manubrium).

One-year-old female presented with cleft of lower lip, mandible, fissured tongue, absent hyoid bone, cleft of manubrium sterni, later is detected clinically by suprasternal bulge at suprasternal region while crying. It is radiologically detected by wide apart medial end of clavicle. This is extremely rare anomaly known as Tassier Cleft no 30.

Double-layered manubrium sterni in young children with diastrophic dysplasia.

A review of the roentgenograms, which included a lateral view of the sternum in 12 children with diastrophic dysplasia, revealed that 4 children (2 newborns, and 2 aged 1.5 years) had a double-layered manubrium with the accessory ossification located ventral to the normal manubrium. In the other 2 children, aged 5 and 11.5 years, the manubrium showed a deformity consistent with a previous double-layered manubrium in which the ventral ossification had fused with the normal dorsal element. The nature of this anomaly is not certain, but a survey of the embryology of the manubrium raises the possibility of a persistence and ossification of a primitive midline embryonic structure that normally is resorbed in its cartilaginous state.

Bifid sternum: neonatal surgical treatment.

The sternal cleft is a rare congenital anomaly resulting from a fusion failure of the sternum, generally observed at birth and asymptomatic. Surgery is indicated to protect the heart and major vessels from trauma, to improve respiratory dynamics, and for aesthetic reasons. We observed 2 neonates for a superior and medial thoracic mass. The defect involved the upper two thirds of the sternum. The surgical operation for both patients consisted in the primary closure of the defect.

Median cleft of the lower lip, mandible and manubrium. A case report.

There is a wide variation in the severity of median clefts of the lower lip and mandible. We report a severe case demonstrating clefts of the lower lip and mandible together with lack of supporting structures in the neck and upper sternum. Since a case with a cleft of the manubrium sterni is very rare, the literature is reviewed and discussed.

Trisomy 21 in newborn infants: chest radiographic diagnosis.

Chest radiographs of 30 infants with trisomy 21 and 881 unaffected infants were evaluated for the following findings common in trisomy 21: multiple manubrial ossification centers, 11 rib pairs, and a bell-shaped chest. Radiographs were obtained in the first 48 hours of life. Of the 881 unaffected infants, 85 (9.6%) exhibited multiple manubrial ossification centers; 46 (5.2%), 11 rib pairs; and 208 (23.6%), bell-shaped chest. Of the 30 infants with trisomy 21, 24 (80%) exhibited multiple manubrial ossification centers; ten (33%), 11 rib pairs; and 24 (80%), bell-shaped chest. The probability of trisomy 21 in routinely radiographed newborn infants is 0.05% when none of the three findings is present, 1.6% with multiple manubrial ossification centers alone, 0.2% with 11 rib pairs alone, and 0.7% with bell-shaped chest alone. The probability of trisomy 21 increases in patients with two findings and reaches 58.4% in patients with all three findings.

[The cleft sternum]. / Rasshcheplenie grudiny.

An analysis of 14 patients with cleft sternum is presented. Eleven patients were operated upon. A classification of this defect is proposed. The clinical picture of each type of cleft sternum is given with a description of the operative technique. There were no lethal outcomes. Remote results were good.

Thoracopagus conjoined twins: radiological evaluation of one case.

Radiological study of conjoined thoracopagus twins. The digestive and biliary tracts, the urinary and cardiovascular systems have been examined. The problems and difficulties encountered in the various procedures and in the interpretation of their results are described.