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Poland's syndrome: different clinical presentations and surgical reconstructions in 18 cases.

BACKGROUND: The literature reports many variations of Poland's syndrome. This article describes 18 cases of Poland's syndrome in different stages of treatment, with variable clinical presentations and reconstructive techniques. METHODS: This study evaluated 15 females and 3 males, ages 2 to 43 years, for breast deformity, nipple-areolar complex position, pectoralis muscle malformation, thoracic deformities, and the presence of brachysyndactyly. Surgical treatment was performed for 14 patients, individualized for each case. RESULTS: For the women, the hypoplastic breast was treated with a latissimus dorsi muscular flap associated with silicone gel implant in five cases. Two other patients still are receiving tissue expansion for a future muscular and prosthetic reconstruction. Prosthetic implants alone were used on the affected side in four cases. The nipple-areolar complex was reconstructed for two patients. Seven women underwent contralateral breast surgery: reduction mammoplasty in three cases, mastopexy in two cases, and prosthetic implants in two cases. The only man who underwent surgery was treated with endoscopic rotation of the latissimus dorsi muscle flap. CONCLUSIONS: This study demonstrated several breast reconstruction options for patients with Poland's syndrome, reinforcing the importance of an individualized treatment to achieve complete and adequate rehabilitation.

Adolescent↗

Nonepidermal origin of malignant melanoma associated with a giant congenital nevocellular nevus.

We report an adult who developed primary malignant melanoma arising in association with residual nevus cells in the pectoralis muscle under an intact skin graft 13 years following the partial excision of a giant congenital nevocellular nevus (GCNN). The case serves to emphasize the malignant potential of GCNN, for which we estimate a lifetime risk of at least 6.3 percent and an approximate 17-fold risk for melanoma when compared with the general population. This case supports previous observations that melanoma may arise in nonepidermal components of a GCNN and suggests the need to consider excision of the GCNN to muscle fascia or deeper if nevus cells extend beyond this anatomic zone. We estimate the risk of death from general anesthesia during the first year of life to be substantially less than the risk of malignant degeneration during the same time period. The decision to excise the GCNN as soon as the problem is recognized should depend on the general health of the infant and the risk of surgical complications.

Adult↗

Invasive ductal carcinoma of the breast associated with Poland's syndrome: report of a case.

We report herein a rare case of invasive ductal carcinoma of the breast associated with Poland's syndrome. The patient was a 59-year-old woman who was referred to our department after a nodule had been found in the upper outer portion of the left breast by a breast cancer screening program. On physical examination, marked hypoplasia of the right breast and upper limb was noted. Preoperative computed tomography also revealed a defect in the right pectoralis muscles. A quadrantectomy of the left breast with lymphadenectomy was subsequently performed and pathological examination of the resected specimen showed invasive ductal carcinoma. Her medical history revealed that her mother had attempted to abort the pregnancy around the fifth week of her gestation. The present case suggests that such an event during gestational development may be associated with congenital anomalies predisposing to malignant disorders.

Arm↗

Thoracic wall involvement by Hodgkin disease and non-Hodgkin lymphoma: CT evaluation.

Thoracic computed tomographic (CT) scans of 250 patients with newly diagnosed or recurrent lymphoma revealed thoracic wall involvement in 24 patients (11 with Hodgkin disease, 13 with non-Hodgkin lymphoma). Thoracic wall involvement occurred without contiguous mediastinal or parenchymal involvement in 17 patients. Of these, 13 patients had masses beneath the pectoralis muscles or within the breast, and four had masses arising from the ribs. Five additional patients had mediastinal masses with thymic involvement and parasternal extension through the thoracic wall. Pulmonary parenchymal lymphoma with thoracic wall invasion was noted in the remaining two patients. In five of nine patients receiving radiation therapy, treatment plans were modified by CT demonstration of thoracic wall lymphoma.

Adolescent↗

Management of necrotic neck wounds with a "sandwich" pectoralis myocutaneous flap.

A modified pectoralis major myocutaneous flap was used to stabilize necrotic neck wounds rapidly in irradiated patients. The flap was a "sandwich" flap that included an overlying "parasternal" pectoral skin paddle for pharyngeal reconstruction, the pectoralis muscle for carotid protection, and a meshed skin graft applied to the undersurface of the muscle to replace cervical skin. This flap has been used to reconstruct seven patients with severe wound necrosis from pharyngeal fistula and infection. All patients had carotid exposure in the infected wound. Reconstruction in all patients accomplished restoration of pharyngeal continuity, carotid protection, and cervical skin replacement. Some patients required more than one procedure for closure. There were no carotid "blowouts" in any of the patients. This technique enables the head and neck surgeon to stabilize these contaminated wounds rapidly and to reconstruct complex defects of the pharynx and cervical skin.

Adult↗

[Local recurrence in breast cancer. Course of the disease with reference to morphologic parameters].

In 124 patients suffering from a local recurrence after primary treatment of a breast cancer the course of disease was checked in a retrospective manner. This particular study points out the dependence of the clinical outcome on morphological indicators. The results show that local recurrence is mostly accompanied by a generalisation of the disease and on the whole by a poor prognosis. After five years only 29% of the patients with local recurrence are still alive. Of the morphological indicators the lymphatic carcinomatosis is the most important factor related with poor prognosis. Infiltration of the pectoralis muscle has no influence on the further course. Only in few cases breast tissue that has been left after the first surgical therapy can be regarded as the starting point of a local recurrence. To avoid a further local recurrence the excision with clear tumour margins is necessary. If an incomplete removal has been carried out or in cases of narrow resection margins one has to reckon with new local recurrencies in half of all cases despite adjunctive radiotherapy. The results suggest that in cases of local recurrence beside the local treatment systemic therapy with hormones or chemotherapy might be important.

Breast↗

Lipid composition and catalytic properties of sarcoplasmic reticulum from normal and dystrophic chicken muscle.

The lipid composition of sarcoplasmic reticulum vesicles isolated from the pectoralis muscle of dystrophic chickens (line 413) and of the genetically related line of normal chickens (line 412) were studied. The total lipid content in the dystrophic preparation was increased by 12% when compared to the normal. The increase was restricted to the neutral lipids in the membrane, with a significant increase of 25% in the cholesterol and of 23% in the triacylglycerol contents. Identical phospholipid composition and content was found. Most of the fatty acid acyl chains from the phospholipids were similar, with an increase in the degree of saturation of phosphatidylcholine, phosphatidylserine and phosphatidylinositol. Similar profiles of the fatty acid acyl chains from triacylglycerol were found. The affinities of the ATPase enzyme for ATP and Ca2+ were similar in the normal and dystrophic preparations. The K0.5 for ATP in both preparations was 28 microM for the Mg2+-dependent activity, and 5-8 microM for the Ca2+-dependent activity which exhibited a secondary increase in the velocity of hydrolysis, in the millimolar ATP concentration range. The K0.5 for Ca2+ in both preparations was approximately 0.3 microM.

Animals↗

Increased turnover of proteins from the sarcoplasmic reticulum of dystrophic chicken muscle cells in tissue culture.

Chicken myoblasts were cultured from the pectoralis muscles of dystrophic and normal 11-day-old embryos. Cells were allowed to grow to fusion (differentiation) and exposed to [35S]methionine for a short period. Subsequently, the decay of labeled proteins in the presence of cycloheximide was measured for various cellular fractions as well as individual proteins isolated from the sarcoplasmic reticulum and separated by gel electrophoresis. Some dystrophic material showed an increased decay when compared to normal material. The most significant (p less than 0.005) difference was found in a Mr = 65,000 component of the sarcoplasmic reticulum. This same component accumulates label at an accelerated rate in the presence of the protease inhibitor leupeptin. Increased turnover of this protein, possibly calsequestrin, may be a manifestation of the genetic disease.

Animals↗

Effects of intense exercise on intracellular glycogen and fat in pigeon pectoralis.

The pigeon pectoralis muscle was electrically stimulated in vivo at a high intensity of 25 V and the intracellular glycogen and fat reserves of the muscle examined histochemically to determine the pattern of substrate utilization in the component broad white and narrow red fibre types during the intense flight-simulated exercise. 4 h of continuous stimulation resulted in the depletion of intracellular glycogen from both fibre types and a simultaneous reduction in the intracellular fat stores of the red fibres. While glycogen provides the only source of energy for the white fibres, glycogen as well as fat serves as fuel for the red fibres. It is postulated that, though fat is the major fuel for the red fibres, their glycogen reserves are utilized to ensure a steady supply of oxaloacetate essential in the oxidation of fatty acids via the Krebs cycle, and that fatty acid catabolism helps to maintain gluconeogenesis.

Animals↗

Regional differences in the expression of myosin light chains and tropomyosin subunits during development of chicken breast muscle.

Types of myosin light chains and tropomyosins present in various regions and at different developmental stages of embryonic and posthatched chicken breast muscle (pectoralis major) have been characterized by two-dimensional gel electrophoresis. In the embryonic muscle all areas appear to accumulate both slow and fast forms of myosin light chains in addition to alpha and beta forms of tropomyosin. During development regional differences in myosin and tropomyosin expression become apparent. Slow myosin subunits become gradually restricted to areas of the anterior region of the muscle and finally become localized to a small red strip found on its anterior deep surface. This red region is characterized by the presence of slow and fast myosin light chains, alpha-fast, alpha-slow, and beta-tropomyosin. In all other areas of the muscle examined only fast myosin light chains, beta-tropomyosin and the alpha-fast form of tropomyosin, are found. In addition, beta-tropomyosin also gradually becomes lost in the posterior regions of the developing breast muscle. In the adult, the red strip area represents less than 1% of the total pectoralis major mass and of the myosin extracted from this area approximately 15% was present as an isozyme that comigrated on nondenaturing gels with myosin from a slow muscle (anterior latissimus dorsi). The red region accumulates therefore fast as well as slow muscle myosin. Thus while the adult chicken pectoralis major is over 99% fast white muscle, the embryonic muscle displays a significant and changing capacity to accumulate both fast and slow muscle peptides.

Animals↗

Effects of thiol protease inhibitors on myoblast fusion and myofibril assembly in vitro.

To investigate the roles of thiol proteases such as cathepsins and calpains in muscle differentiation, we have treated primary cultures of pectoralis muscle with a variety of protease inhibitors and examined the effects these agents have on myoblast fusion and myofibrillogenesis. We have found that a membrane-permeable inhibitor, E64D, has dramatic effects on both events of muscle differentiation. Cells treated with this inhibitor display gross morphological changes, severe delays in myofibril assembly, and reduced ability to fuse in culture. These morphological changes are correlated with a build up of beta1-integrin throughout the cytoplasm. These effects could also be produced using NH4Cl, a lysosomotrophic agent. In addition, we show that two nonpermeable inhibitors (leupeptin and E64) slightly decrease myoblast fusion, but have no effects on the ability of the cells to form mature myofibrils. These results are discussed in terms of their relevance to the inheritable disease of muscular dystrophy and I-cell disease (mucolipodosis II).

Animals↗

[What results can be expected following the biceps replacement operation? An analysis of retrospective data].

The authors reviewed the literature and analysed the results of Steindler's technique, the transfer of pectoralis major muscle and its modifications, and the transfer of latissimus dorsi muscle. They determined the average extension lag, flexion, mobility, and strength in the publications they reviewed. Their own results were included in the statistics. The analysis of the results of the various techniques showed, 1. There is not always good correlation between the theoretical advantages and disadvantages of a surgical method and the clinical results of a particular operation in the restoration of elbow flexion. 2. The technical difficulty of a particular surgical method and the postoperative results must be taken into consideration when selecting the right operation. 3. There is a need for controlled studies of the different methods.

Adult↗

Myocutaneous vascular territory of the thoracoacromial artery. A topographical and morphometric study of the arterial vascularization of the pectoralis major myocutaneous flap.

In order to clarify the vascularization of the pectoralis major muscle by the pectoral branch of the thoracoacromial artery, morphometric investigations and selective injection studies of the vessel were performed. A comparison with the anatomical orientation points clinically used for locating the vessel and its course confirms that the origin of the artery is at a constant spot at half the sternoacromial distance; the main vessel axis, however, deviates considerably from the clinically used acromioxiphoid axis in lateral direction. The lumen of the thoracoacromial artery has a range of more than 150% of the smallest vessel. Atherosclerotic alterations are not the reason for this variety but only an additional handicap for the vessel capacity. Two types of muscle-perforating arteries were found, and the cutaneous area of blood supply was defined.

Acromion↗