Management of the chest-wall deformity in male patients with Poland's syndrome

The chest-wall deformity associated with Poland's syndrome was reconstructed in eight male patients 16 to 38 years old (average age 20 years). Follow-up ranged from 1 to 10 years. Two patients had custom silicone implants placed subcutaneously. In one of these patients, the edge of the implant could be seen. Three patients had transfer of an ipsilateral pedicled latissimus dorsi muscle flap with intact thoracodorsal nerve. All these patients had noticeable atrophy of the flap, and one underwent subsequent implantation of a custom silicone implant beneath the flap. Three other patients had a custom silicone implant covered immediately by a latissimus dorsi muscle flap. All four patients who had a combination of silicone implant and latissimus dorsi muscle flap had satisfactory correction of their deformity.     

Surgical correction of Crouzon syndrome

This study analyzes the results of surgical treatment in 39 patients with the Crouzon syndrome. Early fronto-orbital advancement and craniectomy were universally successful in relieving raised intracranial pressure and in reducing ocular proptosis. However, definitive cosmetic correction was not achieved, and early cranial surgery was not able to prevent the development of midface hypoplasia. Thirty-two midfacial advancements have been performed in 30 patients. Sixteen patients had sufficient follow-up data for more than 2 years postoperatively. In all patients, a satisfactory early postoperative result was achieved. In the long-term follow-up group, 11 patients have maintained a satisfactory appearance, while 5 have developed recurrent deformity. Analysis shows this to be associated with a younger age at operation and continued mandibular growth. Frontofacial advancement in adults achieves good long-term results but is associated with a higher incidence of complications.     

Anterior thoracic hypoplasia: a separate entity from Poland syndrome

SUMMARY: Women presenting with anterior thoracic depression, breast hypoplasia, and subsequent asymmetry are often diagnosed with Poland syndrome regardless of pectoralis involvement, or are placed in the generic category of breast asymmetry or skeletal dysplasias. Recently, though, the term "sunken chest" has been used to describe forms of chest wall depression that previously may have fallen under generic skeletal dysplasias. The authors believe that, combined with hypoplasia of the ipsilateral breast, superior location of the nipple-areola complex compared with the contralateral side, and normal pectoralis muscles, this represents a previously undefined and real condition called anterior thoracic hypoplasia. During the past 4 years, the authors have treated eight women who have presented with a diagnosis of Poland syndrome or pectus excavatum, all of whom share the same characteristics-unilateral sunken anterior chest wall, hypoplasia of the breast, superiorly placed nipple-areola complex, normal pectoralis muscle, and normal sternal position. All of the patients underwent correction of breast asymmetry and unilateral anterior thoracic hypoplasia with augmentation mammaplasty, a method that when tailored for each side yields good aesthetic results. The average age of the patients was 31 years and the average chest size was 34. Cup size, as measured by the patient's standard bra, was a B on the nonaffected side in all patients and an A on the affected side in all patients except one. Of the eight patients, seven had the right anterior chest and breast involved, whereas one patient had involvement on the left. For all of the patients, the nipple and areola of the hypoplastic side were smaller and in a more superior position compared with the contralateral side on visual inspection. In the eight patients, a total of 19 augmentations (15 primary augmentations and four revisions) and one mastopexy were performed. Ten inframammary-fold approaches and nine periareolar approaches were used, and all of the implants were placed in a partial submuscular position, except for two implants placed in a subglandular position that were converted to partial submuscular positions in a secondary setting. In all the women, the sternal head of the pectoralis muscle was present and the pectoralis muscle appeared to be equal in size compared to the contralateral side. Nine different types of implants were used. Average implant fill volume measured 412 cc on the hypoplastic side and 257 cc on the contralateral side. In follow-up, all of the patients were satisfied with their operation and rated their aesthetic outcome as very good to excellent. The authors believe that anterior thoracic hypoplasia is a real, previously misdiagnosed and undescribed condition, and that both chest wall and breast deformities can be corrected safely and with excellent results using proper augmentation planning and implant selection.     

New Poland's old dilemma: The Polish minority of Lithuania

This article seeks to explore the growing problem posed by the Slav/Polish ethnic minority in Lithuania and attempts to place this conflict in the general context of twentieth‐century East European ethnic conflicts. Particular attention is given to explaining the unique historical circumstances that produced this ethnically transitional area between the Byelorus, Polish and Lithuanian societies where throughout their history, the representatives of these ethnic groups as well as a large Jewish community and many other smaller ethnic groups have cohabited together as an intertwined mixture. However, with the spread of the idea of a single ethnic dominated national state, the transitional area in southwestern Lithuania ‐ as well as similar ethnically transitional areas elsewhere in eastern Europe ‐ were destined to be liquidated. Such areas could be liquidated either through some kind of enforceable dispersal of the now ‘undesirable’ ethnic groups from the region or through a state‐driven programme which would impose a new national identity on the ethnic groups involved. With Lithuania being much too weak a society to enforce the dispersal of its Slav minority, and with its élites determined to transform it into a single ethnic dominated national state, the only remaining option was a state policy of ‘Lithuanization’ of the Slav minority. The Slavs’ resistance to such a policy spurred on the growth of ethnic conflict in Lithuania and threatened to spill over into neighbouring countries. This article explores the regional ramifications of this ethnic conflict.     

Pneumothorax after a clinical breast fine-needle aspiration of a lump in a patient with Poland's syndrome

We report the first case in the medical literature of a pneumothorax complicating fine needle aspiration cytology (FNAC) of a breast lump in a woman with a mild form of Poland's syndrome. The pneumothorax was treated conservatively. This is the first case of breast FNA-related pneumothorax seen in our clinical practice. We believe that the absence of pectoral muscles has increased the risk of this complication. We have also diagnosed an incidental screen-detected breast cancer affecting the ipsilateral breast in the same patient. We conclude that caution should be exercised when performing FNAC of breast lesions in patients with Poland's syndrome. The procedure should be preferably performed under image guidance in such patients in order to minimise the risk of this complication.     

Metabolic correction of cerebral radiation syndrome.

DNA strand breaks that occur after irradiation activate the repair enzyme adenosine diphosphoribosyl transferase, which consumes NAD as a substrate and causes depletion first of neuronal NAD and then of the ATP pool. This is considered to be the crucial link in the mechanism underlying the cerebral radiation syndrome (CRS). In this study, two ways to correct the CRS metabolically were examined: (a) prevention of depletion of NAD after irradiation by administration of the enzyme inhibitor nicotinamide and (b) shunting the NAD-dependent oxidative phosphorylation pathway of ATP resynthesis by administration of a substrate of NAD-independent oxidation, succinate. Cerebral lesions induced by radiation were modeled by irradiation of rats or rat brain homogenates with 150 Gy of X rays. The manifestations of CRS in rats (excitement, convulsions, etc.) closely resembled those seen after acute hypoxia. In brain homogenates, pyruvate tetrazolium-reductase activity decreased after irradiation and could be corrected by addition of NAD after irradiation. Succinate tetrazolium-reductase activity was not affected by irradiation. Oxygen consumption by brain homogenates after irradiation in vitro and in situ decreased, as did oxygen consumption by rats in vivo after cranio-caudal irradiation. Administration of nicotinamide or succinate prevented both the postirradiation decrease in respiration (in both rats in vivo and brain homogenates in vitro) and the development of cerebral radiation syndrome. These results help to clarify the mechanisms underlying CRS and its metabolic correction.     

Report of a girl with Klippel-feil syndrome and Poland anomaly

Report of a girl with Klippel-feil syndrome and Poland anomaly: Klippel-Feil syndrome, consisting of the triad of a short neck, low posterior hairline, and limitation of neck movement, is a congenital anomaly characterized by the fusion of cervical vertebrae, Poland anomaly consists of unilateral aplasia of the chest wall muscles and ipsilateral anomalies of upper extremity. We report a 7-year-old girl with typical findings of Klippel-Feil syndrome and Poland anomaly. To the best of our knowledge a case of Klippel-Feil syndrome and Poland anomaly has not been described before, although a combination of Poland, Klippel-Feil and Moebius anomalies has been reported in the literature.     

Rapid acquisition of Raman spectral maps through minimal sampling: applications in tissue imaging

A method is presented for acquiring high‐spatial‐resolution spectral maps, in particular for Raman micro‐spectroscopy (RMS), by selectively sampling the spatial features of interest and interpolating the results. This method achieves up to 30 times reduction in the sampling time compared to raster‐scanning, the resulting images have excellent correlation with conventional histopathological staining, and are achieved with sufficient spectral signal‐to‐noise ratio to identify individual tissue structures. The benefits of this selective sampling method are not limited to tissue imaging however; it is expected that the method may be applied to other techniques which employ point‐by‐point mapping of large substrates. (© 2012 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)     

From the deep sea to the stars: human life support through minimal communities

Support of human life during long-distance exploratory space travel or in the creation of human habitats in extreme environments can be accomplished using the action of microbial consortia inhabiting interconnected bioreactors, designed for the purpose of reconversion of solid, liquid and gaseous wastes produced by the human crew or by one of the compartments of the bioregenerative loop, into nutritional biomass, oxygen and potable water. The microorganisms responsible for bioregenerative life support are part of Earth's own geomicrobial reconversion cycle. Depending on the resources and conditions available, minimal life support systems can be assembled using appropriately selected microorganisms that possess metabolic routes for each specific purpose in the transformation cycle. Under control of an engineered system, a reliable life-support system can hence be provided for.     

Evaluation of phase correction and low gas density to improve thoracic gas volume measurement

We investigated two methods of decreasing the error on plethysmographic determinations of thoracic gas volume (TGV) related to cheeks movements during panting maneuvers: lowering gas density in the airways with an 80% He-20% O2 mixture and computing TGV from the in-phase component of the plethysmographic signal (TGVr). The methods were tested by measuring how TGV estimates varied when panting frequency was raised from 0.8 to 2.5 Hz during the same occlusion. The measurements were performed in 6 normal subjects and 12 patients with chronic bronchitis with and without cheeks support and when the airway was connected to an external device simulating an increased cheeks compliance. A small negative frequency dependence of TGV (delta TGV/delta f = -1.2 +/- 0.8%/Hz with cheeks support), most probably unrelated to upper airway walls, was found in normal subjects. Delta TGV/delta f was positive and algebraically larger in patients than in normals, reaching 2.2 +/- 3.4%/Hz without cheeks support and 11.8 +/- 8.0%/Hz with the additional cheeks. The latter value was only 20% smaller when computed on the basis of TGVr, demonstrating the limited usefulness of the phase-based correction. In contrast, breathing He-O2 decreased delta TGV/delta f to approximately 50% of its air value (P less than 0.01) and appears as an effective way to diminish the error in obstructive patients.