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Pectus excavatum.

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Age Factors↗

Repair of pectus excavatum using a substernal metal strut within a Marlex envelope.

In a retrospective review, 82 children aged 3 to 16 years who had repair of pectus excavatum from 1963 to 1987 were divided into three groups according to type of repair: those without a metal substernal strut, those with a metal substernal strut, and those with a metal substernal strut within a Marlex envelope. In group 1 (n = 50) there were five recurrences; in group 2 (n = 18) and group 3 (n = 14) there were no recurrences. There was migration of the substernal strut in eight patients in group 2 (44%) and three patients in group 3 (21%). There were minor wound infections in groups 1 and 2 only. There were no deaths. Results of this study suggest that the use of a substernal strut significantly reduced recurrence and that the addition of a Marlex envelope around the strut reduced migration of the strut with no associated complications.

Adolescent↗

Pectus excavatum: a 15-year perspective.

Pectus excavatum is relatively uncommon. Our experience with 177 children during a 15-year period produced changes in our surgical technique, which now includes a small transverse incision, minimal subcutaneous flap elevation, a muscle-relaxing incision over the fifth costal cartilage, complete resection of involved cartilage, use of Adkins' strut, suspension of sternum to strut, taut reefing of intercostal muscle, no tubes or drains, epidural analgesia, a patient-controlled analgesia device postoperatively, and eventual strut removal. Use of the evolved technique gives excellent cosmetic results, good functional results with minimal discomfort, and a shorter convalescent period.

Adolescent↗

Eating disorder in a patient with phenotypical features of Lujan-Fryns syndrome.

We report a young patient with phenotypic, cognitive and behavioural features consistent with a diagnosis of Lujan-Fryns syndrome. He exhibited a severe eating disorder with food refusal and psychogenic loss of appetite, which led to a serious state of malnutrition. Behavioural difficulties including shyness, hyperactivity and emotional instability are present in almost 80% of the described patients with Lujan-Fryns syndrome but eating disorders have not been previously reported in this condition. A review of the behavioural problems in Lujan-Fryns syndrome and an approach to their management is discussed.

Abnormalities, Multiple↗

Soft-tissue augmentation and the role of poly-L-lactic acid.

BACKGROUND: Facial rejuvenation has traditionally centered on the correction of ptosis, lines, and wrinkles, typically achieved by surgical tightening of the skin or by injecting soft-tissue fillers. In recent years, volumetric augmentation and recontouring have been recognized as fundamentally important aspects of facial rejuvenation. METHODS: An overview of volume restoration and recontouring is presented. A literature search was also conducted to identify the injectable products representing potential treatment options for facial recontouring. The author also presents the results of his extensive experience with poly-L-lactic acid in the treatment of thousands of cosmetic patients in Europe, with special emphasis on the efficacy and tolerability of this product. RESULTS: Several injectable treatment options can be used to recontour and restore volume, to the face and beyond. All of these products, except calcium hydroxylapatite and poly-L-lactic acid, are nonresorbable, offering permanent volume augmentation, but with the increased risk of severe foreign-body reactions. Poly-L-lactic acid provides semipermanent results and has been documented to offer volume augmentation with a favorable safety profile. Achieving good efficacy and safety is dependent on the appropriate reconstitution of the product and adhering to the correct injection technique. CONCLUSIONS: Injectable products offer a flexible means of recontouring the face and localized regions beyond the face. The potential of some of these products is supported by a wealth of published results and clinical experience, helping the physician to identify the most appropriate treatment for their patient.

Adult↗

Leopard syndrome: a tropical rarity.

This is a case report of a classical presentation of the Leopard syndrome from the Indian subcontinent. The male patient had progressively increasing generalised multiple lentiginosis, ocular hypertelorism, pulmonary stenosis, short stature, cryptorchidism, and pectus excavatum. The case is documented for its rarity in this part of the world and typical clinical presentation.

Abnormalities, Multiple↗

Oto-palato-digital syndrome type II in two unrelated boys.

We report on two boys with oto-palato-digital syndrome type II, characterized by growth retardation, bowed long bones, missing or hypoplastic fibulae, sclerosis of the skull base and wavy, irregular clavicles and ribs. The facial appearance is distinctive due to prominent forehead, widely spaced eyes, antimongologid slant of palpebral fissures, flattened nasal bridge and retrogenia. The mother of one patient showed a mild manifestation of oto-palato-digital syndrome type II. Only about 20 cases of this rare X-linked disorder have been reported so far. The similarities and dissimilarities to oto-palato-digital syndrome type I are discussed.

Abnormalities, Multiple↗