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Two cases of nevoid basal cell carcinoma syndrome.

Two cases of nevoid basal cell carcinoma syndrome were reported with a review of pertinent literature. The first case was a 59-year-old man, whose autopsy was warranted. Signs and symptoms manifested in this case were basal cell carcinoma, generalized multiple nevi, multiple cysts in the jaws and long bones, pits in the palm and sole, frontal and occipital bossing, ossification of the falx cerebri, a bifid rib, renal fibroma and a patent foramen ovale. The family history revealed a hereditary predisposition as to the syndrome. The patient in the second case included basal cell carcinoma, multiple nevi, multiple jaw cysts, pits in the palm and sole, frontal bossing, calcification of the falx cerebri, cervical vertebral fusion and high-arched palate.

Adult↗

The learning curve in learning the curve: a review of Nuss procedure in teenagers.

BACKGROUND: The Nuss procedure is a new minimally invasive repair for pectus excavatum that was first published in 1998. Modifications in technique are constantly evolving to minimize complications, especially bar displacement, which are higher in adolescents and adults. The present study reviews our early experience with this procedure in a much older group of children than previously reported and suggests an alternative method of avoiding bar displacement. METHODS: Retrospective chart review was carried out on 78 consecutive patients who underwent the Nuss procedure between December 1999 and January 2004. All patients underwent a uniform technique using bilateral lateral stabilisers and thoracoscopy was not used. Operative details, subjective cosmetic results and complication rates were assessed. RESULTS: The mean age was 15.4 +/- 3.2 years. Single bars were used in 95%, double bars in 5%. The defect was asymmetrical in 26%. The defect was mild in 7%, moderate in 59% and severe in 34%. The median length of stay was 6.0 days (range 4-11). Total operating time was 58 min (range 35-95). Patient controlled analgesia (morphine) for pain relief was used for 105 h (range 61-169) or 4.4 days; the epidural infusion was stopped 1 day earlier. Cosmetic results were excellent in 80.3%. There was residual asymmetry in 75% of the asymmetrical defects although all were improved. Complications included eight (10%) reoperations for bar displacement. This was related to the learning curve as seven of these occurred in the first 2 years of the series. Removal of the bar has been accomplished in 31 (40%) patients. This was elective in all but four patients (three early removals for pain and one for infection). CONCLUSIONS: The Nuss procedure gives good results even in teenagers. Modification of technique and increased experience has reduced complications. The use of bilateral lateral stabilisers without additional wire fixation is an alternative method to avoid bar displacement.

Adolescent↗

Positioning for the Nuss procedure: avoiding brachial plexus injury.

BACKGROUND: In 1987, Nuss developed a minimally invasive technique for the treatment of pectus excavatum. The procedure has had excellent results with minimal complications. Anesthetic concerns include risks of cardiothoracic injuries, dysrythmias, pneumothorax, pleural effusions, and hemorrhage. In addition, we identified a risk of brachial plexus injury that can occur secondary to patient positioning. We report our experience with the Nuss procedure and modified patient positioning. METHODS: A retrospective chart review was performed on 95 children or adolescents who underwent a Nuss procedure at the Children's Hospital in Denver, CO, USA. The first group of patients (58) was positioned with their arms extended above their heads at a 90 degrees angle at the shoulder. The second group of patients (37) was positioned in an arthroscopy sling in a neutral position utilizing a right angled suspension device attached to the operating room bed. RESULTS: In the first group of patients, three of 58 (5.2%) children had transient brachial plexus injuries. To date, in the second group of patients, there have been no brachial plexus injuries using the suspension device. CONCLUSIONS: We found that patients are at risk of transient brachial plexus injury during the Nuss procedure due to positioning of the arms. A readily available device that permits the extremity to be maintained in a neutral position but also allows intraoperative manipulation during critical aspects of the Nuss procedure eliminates transient brachial plexus palsy.

Adolescent↗

Fingerprint body myopathy: a report of twins.

Ultramicroscopic changes of subsarcolemmal fingerprints in the muscle of children with infantile hypotonia and weakness may represent a specific congenital entity. Four children have been reported so far. The two children reported in the present paper are the first full siblings to be described and, in addition, are identical twins. Four of these six children also had mental retardation, which suggests that this disorder may carry with it a significant risk of central nervous system abnormality.

Child↗

A technique for concurrent repair of pectus excavatum and intracardiac defects.

Pectus excavatum deformity may present problems when median sternotomy is required for repair of intracardiac defects. We have used a partial median sternotomy extending from the manubrium to the level of the second intercostal space with extension into that space. The perichondral sheaths are disconnected from the sternum on the right side after removing the deformed costal cartilages. Exposure is thus provided for the cardiac procedure. Following this, the upper sternal fragments are sutured together and the repair is completed in a manner similar to the Ravitch technique.

Funnel Chest↗

Atypical pectus excavatum in two Welsh terrier littermates.

Two six-week-old intact Welsh terrier littermates, a male and a female, were presented for congenital ventral thoracic wall deformities characterised by noticeable funnel-like depressions of the cranial sternum associated with inversion of the rib cage. No exercise intolerance or cardiac murmurs were noted. Thoracic radiographic examination revealed a significant dorsal deviation of the first to the fifth sternebrae. At 12 weeks of age, the thoracic depressions had improved markedly in both puppies. Thoracic radiography to reassess the sternal deviation was at this stage within normal limits, demonstrating complete radiographic resolution of the sternal deformity.

Abnormalities, Multiple↗

Pectus excavatum surgically repaired using sternum realignment and splint techniques in a young cat.

Successful surgical repair of pectus excavatum deformities has so far only been reported in very young animals. This case report describes an alternative technique for repairing pectus excavatum in a young cat. The cat had shown moderate deformity with slight respiratory impairment when young and had experienced clinical problems with age. A sternum realignment technique involving a pin associated with an external splint was used. This alternative technique was a safe and efficient procedure in the five-month-old cat.

Animals↗

Pectus excavatum.

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Congenital Abnormalities↗