A 22-year-old woman with severe developmental disability, percutaneous endoscopic gastrostomy dependence, and chronic constipation presented with progressive abdominal distension and obstipation. Initial abdominal radiography demonstrated diffuse gaseous distension without a clear transition point, and she was discharged after symptomatic management. She returned within 24 hours with worsening distension, tachycardia, leukocytosis, and an elevated lactate level. Computed tomography of the abdomen and pelvis demonstrated marked dilation of the right colon with a maximal diameter of approximately 13 cm, along with pronounced cephalad displacement and organ shift, raising concern for toxic megacolon or acute colonic pseudo-obstruction. A water-soluble contrast enema demonstrated abrupt tapering at the distal transverse colon with a classic bird's beak configuration, consistent with mechanical torsion. Urgent exploratory laparotomy confirmed a long-segment right-colon volvulus involving the cecum, ascending colon, and proximal transverse colon with obstruction at the mid-transverse colon. The patient underwent decompression and right hemicolectomy with primary ileocolic anastomosis and recovered without complication.

Morphological integration of the bird skeleton is of great interest because it relates to issues of specialization, plasticity, and rate of evolutionary transformations of a skeleton as a whole and its anatomical regions. Despite growing interest, the integration and modularity of the skeleton of birds, in general, remain little studied. We evaluated the change of relative sizes and integration of shapes of skull, sternum and pelvis, and factors that influence the covariation of these regions among passerines. Results of both standard and phylogenetic reduced major axis showed that the relative lengths of the most studied skeletal traits were largely determined by body mass. The length of the skull scaled isometrically on body mass, and the lengths of both synsacrum and ilium showed positive allometry. Within the skull, beak length was positively allometric, whereas cranium length was negatively allometric with body mass. We found the presence of covariation between shapes of skull, sternum and pelvis using standard partial least squares (PLS) analysis and absence of covariation between most of these blocks using evolutionary PLS analysis on phylogenetic independent contrasts. Evolutionary integration is confirmed only for shapes of skull and pelvis (dorsal view).

An 85-year-old female with an extensive past medical history attended our accident and emergency department with vague abdominal pain and distension. Clinical examination and initial blood tests demonstrated features of sepsis and she was commenced on broad spectrum antibiotics. Following admission, a contrast-enhanced CT scan of the abdomen and pelvis was performed, which showed an unusual configuration of the gallbladder. There was significant luminal distension of the gallbladder, which was found to lie in an unusual horizontal position anterior to the liver edge. This was a new finding compared with a recent CT pulmonary angiogram that demonstrated a normal gallbladder anatomy. In addition, there was abrupt angulation of the gallbladder neck, demonstrating a "beak" sign as well as indrawing of the vascular pedicle forming a mini "swirl" sign. The constellation of gallbladder distension, abnormal lie, "beak" and mini "swirl" sign together is a strong indicator of gallbladder torsion. Coexistent right lower lobe pneumonia was noted. The patient was too frail for surgical intervention and succumbed to underlying sepsis. Post-mortem examination demonstrated a gangrenous gallbladder secondary to volvulus. We present a rare case of gallbladder volvulus and highlight CT findings to help the radiologist make a pre-operative diagnosis.

Dysosteosclerosis (DSS), an extremely rare dense bone disease, features short stature and fractures and sometimes optic atrophy, cranial nerve palsy, developmental delay, and failure of tooth eruption in infancy or early childhood consistent with osteopetrosis (OPT). Bone histology during childhood shows unresorbed primary spongiosa from deficient osteoclast action. Additionally, there is remarkable progressive flattening of all vertebrae and, by adolescence, paradoxical metaphyseal osteopenia with thin cortical bone. Reports of consanguinity indicate autosomal recessive inheritance, yet more affected males than females suggest X-linked recessive inheritance. We investigated a nonconsanguineous girl with DSS. Osteosclerosis was discovered at age 7 months. Our studies, spanning ages 11 to 44 months, showed weight at approximately 50th percentile, and length diminishing from approximately 30th percentile to -2.3 SD. Head circumference was +4 SD. The patient had frontal bossing, blue sclera, normal teeth, genu valgum, and unremarkable joints. Radiographs showed orbital and facial sclerosis, basilar thickening, bone-in-bone appearance of the pelvis, sclerotic long bone ends, and fractures of ribs and extremities. Progressive metaphyseal widening occurred as vertebrae changed from ovoid to flattened and became beaked anteriorly. A hemogram was normal. Consistent with OPT, serum parathyroid hormone (PTH) concentrations reflected dietary calcium levels. Serum bone alkaline phosphatase, osteocalcin, and TRACP-5b were subnormal. The iliac crest contained excessive primary spongiosa and no osteoclasts. No mutations were identified in the splice sites or exons for the genes encoding chloride channel 7, T-cell immune regulator 1, OPT-associated transmembrane protein 1, and monocyte colony-stimulating factor (M-CSF) and its receptor C-FMS, ANKH, OPG, RANK, and RANKL. Genomic copy-number microarray was unrevealing. Hence, DSS is a distinctive OPT of unknown etiology featuring osteoclast deficiency during early childhood. How osteopenia follows is an enigma of human skeletal pathobiology.

A case of uterine lipoleiomyoma in a 72-year-old woman is presented. Although a series of imaging studies suggested a lipomatous tumor, diagnosis was difficult because the tumor appeared as a pedunculated mass extending from the uterine body. To distinguish the tumor from an ovarian lipomatous tumor, the "beak sign" in a magnetic resonance imaging study was diagnostic in this case. The purpose of this paper is to review lipomatous masses of the female pelvis, to discuss the differential diagnosis of the unusual imaging features, and to discuss imaging techniques to optimize pelvic mass characterization.

The case of a patient with surgically proven internal herniation of a loop of ileum through the sigmoid mesocolon is described. This 66-year-old man presented clinically with acute lower abdominal pain and an elevated white blood cell count. A computed tomography (CT) scan showed a thickened bowel loop with "bird-beak" appearance in the pelvis, centered towards the medial side and lying aside the effaced sigmoid colon. We think this CT picture is highly suggestive of internal herniation of the ileum through the sigmoid mesocolon, which is a rare clinical entity.

We report a hitherto unknown, lethal osteochondrodysplasia in two Japanese siblings born to consanguineous parents. The skeletal abnormalities are characterised by mesomelic brachymelia with bowed forearms, a round pelvis with shortened greater sciatic notches, an ossification defect of the pubic bones, and absence of ossification centers in the cervical vertebral bodies. The associated visceral anomalies comprised periportal fibrosis and cystic dysplasia of the intrahepatic bile ducts, pancreatic ductal ectasia, a simple renal cyst, microcephaly with multifocal laminar necrosis and ectopic gray matter, dysplastic tracheobronchial cartilage, abnormal lobulation of the lung, diaphragmatic hernia, and stenotic pulmonary valve. Thrombocytopenia was present but megakaryocytes were slightly increased in the bone marrow. The patients showed various dysmorphic features including aniridia, a long palpebral fissure, prominent nasal bridge, beaked nose, flat philtrum, low-set fleshy ears, micrognathia with submucosal cleft palate, and multiple joint contractures.

In order to investigate further the effects of paralysis on skeletal development in the chick embryo, paralysis was induced at 6 days of incubation by dropping 0.2% solution of decamethonium bromide onto the chorioallantoic membrane and maintaining paralysis through to 20 days of incubation. General effects of paralysis included lower body weight, marked subcutaneous oedema, twisting of the neck to the right with marked rigidity, and protrusion of the lower beak beyond the upper. Skeletal abnormalities included cartilaginous and later bony fusion between cervical vertebrae and distortion of scapula and pubis. Long bones were normal in their general form but showed marked reductions in full length and in length of the calcified diaphysis. The patella occasionally underwent chondrification, unlike the tibial cartilage at the tarsus. Reduction in length of the bones of both jaws occurred but was substantially greater in the upper, resulting in the protrusion of the lower. Ossification times of most skeletal elements were little affected by paralysis but some centres appearing nearer the end of incubation showed slight delay in their times of appearance.

The clinical, radiological, and neuropathological findings in early onset Cockayne syndrome are illustrated in identical twins and their brother. Their appearance of dwarfism with small head and prominent beaked nose strongly resembled that seen in the Seckel syndrome, but unlike patients with that syndrome they had a normal birth weight (for twins), thick cranial vaults, intracranial calcification, and a severe degree of mental retardation. The twins were deaf and blind, with optic atrophy and retinal pigmentation, while their brother had cataracts. Their hands and feet were large in proportion to their small trunk. They had cutaneous sensitivity to any slight exposure to ultraviolet light and severe neurologic problems with incoordination and spasticity. Radiologic findings included microcephaly, a thick cranial vault, a small pelvis, coxa valga, and "ivory epiphyses" in terminal phalanges of hands and feet. Pathologic findings included atrophy of white matter with widespread patchy demyelination, and massive siderocalcific deposits in the brain, particularly in the basal ganglia and cerebellum. While autosomal recessive inheritance is most likely, formally X-linked inheritance cannot be excluded.

The processes are as follows: 1) Life-sized photoelastic models molded from a cadaver pelvis are made of Epoxy resin. 2) In order to practice the stress freezing process, the models are set to our original loading apparatus in the electric heater. The forces exerted on the hip models are both body weight and four kinds of muscular forces, those of the abductor, adductor, rectus femoris and gluteus maximus. 3) After the stresses are frozen in the models, they are cut off into slices to observe isochromatic and isoclinic lines. 4) In the stress analysis both the shear difference method and the double slice method are utilized to obtain more accurate stress distributions. And the results have been summarized as follows: 1) In the ring-shaped slices large stresses are observed especially at four areas; namely the central area of the iliac bone, the acetabulum proximal to the ilium, the pubic ramus and the area adjacent to the sacroiliac joint. The specific areas where hydrostatic pressure works are seen at two points in the deep acetabular cavity. 2) The principal stress lines of almost all slices tend to flow smoothly and monotonously along the ilium, the sacrum and the pubic bone, but as they approach the acetabulum they run in complex patterns and make some singular points. 3) Around the acetabulum the principal stress lines of one group run in concentric circles and those of the other group radiate in all directions from the center of the acetabulum at the upper half of the dome which supports the load. The stress here was 5 to 6 times greater than in the lower half of the dome. 4) The acetabulum is stretched vertically and deformed into an oval shape. The horse shoe-shaped articular facet is deformed as both beaks approach each other. 5) As for the slices of the pelvic ring the normal acetabulum tends to be obviously deformed as it holds or embraces the femoral head. As a result of this deformity the stability between the acetabulum and the head increases. On the other hand the dysplastic acetabulum shows none of those phenomena. 6) The iliac bone of the pelvic ring is deformed into the "S" shape and the pubic ramus the "C" shape, the "S" shape, or the "Z" shape depending on the limb position. The pelvic ring tends to be deformed into a heart shape during abduction.