Visceral heterotaxy syndrome causes abnormal arrangement of thoracoabdominal organs and severe complex cardiac anomalies by abnormal laterality. The purpose of the present study is to analyze the incidence and pattern of heterotaxy syndrome in etretinate and all-tran retinoic acid treated pregnant DDY mice. Pregnant DDY mice were intragastrically given a single dose of 15 mg/kg of etretinate at day 6, 7 of gestation, 30 mg/kg of etretinate at day 7 of gestation and 20 mg/kg of all-trans retinoic acid at day 7 of gestation. The incidence of visceral heterotaxy was highest in the etretinate 15 mg/kg treated group on day 7 of gestation (38.5%). The major cardiovascular anomalies in heterotaxy syndrome were common atrium, common atrioventricular valve, atrioventricular septal defect, transposition of great arteries, pulmonary atresia, pulmonary artery hypoplasia and aortic arch anomalies. Atrial situs of heterotaxy syndrome were right isomerism, solitus-like, inversus-like and left atrial aplasia, but right isomerism was observed most frequently. The results suggest that retinoic acid exerts a significant effect on the determination of atrial situs during the development of mouse embryo.
*Abnormalities, Drug-Induced ; Animal ; Blood Vessels/abnormalities ; Female ; Heart Defects, Congenital/chemically induced ; Mice ; Pregnancy ; Syndrome ; Tretinoin/*toxicity

BACKGROUND AND OBJECTIVES: The blood supply of the nasal tip and columella was examined to determine whether the blood supply to the nasal tip could be damaged from transcolumellar incision during an external rhinoplasty approach in Koreans. MATERIALS AND METHODS: The blood vessels that supply the nasal tip were examined by dissecting 51 cadavers, and their corresponding 102 nasal sections were injected with red latex prior to the dissection. The size and distribution of the vessels were measured with unaided eyes to determine the primary supplying vessels. The subdermal layer in which the vessels lie and the course of the vessels were also investigated. RESULTS: The main vessels of the nasal tip proved to be the lateral nasal artery (78%) and the dorsal nasal artery (22%). Columellar branches were narrower in diameter than the lateral nasal and dorsal nasal arteries and varied in their size and appearance, and therefore they appeared insufficient to be main vessels to supply blood. These arteries passed through the musculoaponeurotic layer, but they were also close to the main surgical plane in the dome of the lower lateral cartilage. CONCLUSIONS: We speculate that the blood supply to nasal tip in Koreans is primarily derived from the lateral nasal or dorsal nasal arteries, with variable contribution from the columellar arteries. Therefore, it is the most important to determine the surgical plane below the musculoaponeurotic layer in order to prevent necrosis of skin flap or deformity due to damage of vessels in the case of external rhinoplasty.
Arteries ; Asian Continental Ancestry Group ; Blood Vessels ; Cadaver ; Cartilage ; Congenital Abnormalities ; Humans ; Latex ; Necrosis ; Rhinoplasty ; Skin

It has been reported that pulsations in abnormal vessels are observed on indocyanine green (ICG) angiography in half of patients with polypoidal choroidal vasculopathy (PCV), although the mechanism of the pulsation is unknown. In this study, we report a case of PCV showing venous pulsations at an arterio-venous (A-V) crossing, and discuss a possible mechanism of polypoidal vessel formation and pulsations in PCV. A 66-year-old female presented with a reddish-orange elevated lesion and serous retinal detachment in the macula of her left eye, and was diagnosed as PCV. She was treated with photodynamic therapy (PDT), and followed-up through routine examinations, including ICG angiography. ICG angiography at presentation showed a branching vascular network and choroidal venules with dye leakage (polypoidal vessels) in the left eye. Pulsations, supposedly of venous origin, were observed at an A-V crossing in the abnormal vessels. Within 3 months after PDT, the polypoidal vessel ceased to leak and the pulsations vanished. The reddish-orange lesion gradually decreased in size with complete disappearance of retinal detachment. This study suggests that an unusual compression at an A-V crossing may make a venule polypoidal, and fluctuations of blood flow and pressure in the venule may cause pulsatile movements of the vessel wall.
Aged ; Choroid/*blood supply ; Choroid Diseases/*diagnosis/physiopathology ; Diagnosis, Differential ; Female ; Fluorescein Angiography ; Fundus Oculi ; Humans ; Retinal Vessels/*abnormalities/physiopathology

PURPOSE: The aim of the present study was to investigate the risk factors for the development of coronary artery lesions (CALs) and to determine whether hyponatremia is associated with CALs in children with Kawasaki disease (KD). METHODS: We retrospectively analyzed the data of 105 children with KD who were admitted to Ilsan Hospital between January 2000 and July 2011. RESULTS: Erythrocyte sedimentation rate (P = 0.013), total bilirubin levels (P = 0.017) were higher and serum sodium levels (P = 0.027) were lower in KD children with CALs than those without. White blood cell (WBC) counts (P = 0.006), neutrophil counts (P = 0.003) were higher and albumin levels (P = 0.009) were lower in KD children with hyponatremia than those without. On multiple logistic regression analysis, hyponatremia (P = 0.024) and intravenous immunoglobulin??resistance (P = 0.024) were independent risk factors for CALs in KD. Furthermore, serum sodium levels were correlated negatively with WBC counts (P = 0.004), neutrophil counts (P < 0.001), total bilirubin levels (P = 0.005) and positively with albumin levels (P = 0.009). CONCLUSION: Our study indicates that hyponatremia may reflect severe inflammation in children with KD.
Bilirubin ; Blood Sedimentation ; Cardiovascular Abnormalities ; Child* ; Coronary Vessels ; Humans ; Hyponatremia* ; Inflammation* ; Leukocytes ; Logistic Models ; Mucocutaneous Lymph Node Syndrome* ; Neutrophils ; Retrospective Studies ; Risk Factors ; Sodium

Rhino-orbito-cerebral mucormycosis (ROCM) is caused by invasion of orbital and intracranial structures directly or through the blood vessels of fungi of the Order Mucorales. It is the most fulminant form of mucormycosis and can manifest brain abscess, cranial nerve palsies, thrombosis or aneurysm, as well as sinusitis, facial or nasal deformity. We report one ROCM case complicated by recurrent cerebral infarctions after involvement of cavernous sinus and meningeal inflammation, despite treatment with surgical debridement and high dose intravenous amphotericin B.
Amphotericin B ; Aneurysm ; Blood Vessels ; Brain Abscess ; Cavernous Sinus ; Cerebral Infarction ; Congenital Abnormalities ; Cranial Nerve Diseases ; Debridement ; Fungi ; Inflammation ; Meningitis ; Mucorales ; Mucormycosis ; Orbit ; Sinusitis ; Thrombosis ; Vasculitis

Free -flap which is transferred to recipients is a thin flat tissue isolated from human body and nutrient vessel of free - flap is ligated with similarly sized vessel. Free -flap reconstruction is widely used, due to high rate of flap survival and functional refinements, although it gives disadvantage of long surgery time and inefficient adaptation. This study demonstrates the characteristics and arterial supply of rectus femoris muscle of thigh. Seventy thighs (35 right, 35 left) of the 35 cadavers (19 males/16 females) which were clinically normal and without deformity were dissected and measured, according to Martin measurements. 1. The average length and width of rectus femoris muscle were 396.7 +/-39.9 mm and 36.5 +/-7.9 mm, respectively. The length of female subjects was significantly different from that of male subjects (P 0.05). 2. In the origin and insertion of rectus femoris muscle, anterior tendon and posterior tendon were not different between female and male subjects. 3. Artery of the rectus femoris muscle arises from deep femoral artery. The length of pedicle from the entering point of the rectus femoris muscle was average 19.0 +/-11.9 mm. The length from the entering point of the rectus femoris muscle to spliting point was 13.7 +/-7.7 mm of P1, 12.6 +/-7.5 mm of P2, and 14.8 +/-11.63 mm of P3 pedicles. 4. The frequency of the blood vessels inserted into the rectus femoris muscle was the first highest at the portion of 32.5 ~35% and the second highest of 35 ~37.5% and 37.5 ~40% ranges, when anterior superior iliac spine from center of the patellar was considered as 100% of portion. 5. According to the method by Kimata et al. (1998) classifying perforators based on the anatomic variations, type 1 and VIII were 83.58% and 5.97% respectively. Type 5, 6 and 7 were not observed. 6. Femoral nerves inserted into the rectus femoris muscle were distributed within 7.5 ~45% portion when anterior superior iliac spine from center of the patella was converted into 100%. The portion of 17.5 ~20% had the highest frequency of nerves. In conclusion, length of the rectus femoris muscle in Korean were different depending on gender, whereas tendon lengths were not. These data demonstrate artery pattern in Korean and provide anatomical potential for rectus femoris muscle to be used as a free -flap muscle.
Arteries ; Blood Vessels ; Cadaver ; Congenital Abnormalities ; Female ; Femoral Artery ; Femoral Nerve ; Human Body ; Humans ; Male ; Patella ; Quadriceps Muscle* ; Spine ; Tendons ; Thigh

BACKGROUNDHemangiomas are the most common tumors in children. Some hemangiomas may require intervention because of their location, size, behavior, or potential for important complications. Pharmacological therapy with glucocorticoids is the mainstay treatment, but there is no consensus on therapeutic regimens or candidate selection, therapeutic efficacy varies, and the mechanism mediating the beneficial effects of glucocorticoids remains unclear. This study was performed to investigate the expression patterns of the glucocorticoid receptor (GR) and its alpha isoform (GRalpha) in cutaneous hemangiomas and vascular malformations.

METHODSSP immunohistochemical technique was used to examine the expression of GR(e-20) (GR) and GR(p-20) (GRalpha) on vascular endothelial cells in 80 specimens that included 33 proliferating hemangiomas, 32 involuting hemangiomas, 7 vascular malformations as well as 8 normal skin tissues, all obtained from infants and children. GR and GRalpha expression in prepared tissue slides were examined using automated computer-assisted microscopic analysis. Mean gray scale values were compared among the various tumor types.

RESULTSThe mean gray scale values of GR were 127.0 +/- 6.4 and 121.4 +/- 6.6 in hemangiomas and vascular malformations respectively, but this difference was not statistically significant (P = 0.104). However, these values were all markedly higher than that of normal skin, which was only 108.6 +/- 6.8 (P = 0.001 and P = 0.000 for comparison with hemangiomas and vascular malformations respectively). The gray scale of GR in proliferation and involuting hemangiomas were 127.9 +/- 4.8 and 126.0 +/- 5.8 respectively, but this difference was not significant (P = 0.146). However, GRalpha expression in hemangiomas, vascular malformations and normal skin declined gradually in stepwise fashion (127.3 +/- 5.4, 120.4 +/- 6.1 and 109.9 +/- 5.3 respectively; P < 0.001). GRalpha expression was higher in proliferating hemangiomas than in involuting hemangiomas (127.2 +/- 6.3 and 122.5 +/- 6.3; P = 0.004).

CONCLUSIONSGR and GRalpha are strongly expressed in hemangiomas and vascular malformations. The expression of GRalpha is closely related to the phase of the hemangioma. Determination of GR and GRalpha may be a positive significance to understand the information of hemangiomas and vascular malformations and may further help determining proper strategies of steroid therapy for hemangiomas and vascular malformations.

Blood Vessels ; abnormalities ; Child ; Child, Preschool ; Female ; Hemangioma ; chemistry ; pathology ; Humans ; Immunohistochemistry ; Infant ; Male ; Protein Isoforms ; Receptors, Glucocorticoid ; analysis ; Skin Neoplasms ; chemistry ; pathology

BACKGROUNDVascular anomalies are common and multidisciplinary involved diseases. The greatest impediment to their treatment in the past was their confusing terminology and clinical heterogeneities. This hospital-based retrospective study assessed some clinical characteristics, diagnosis, therapies and outcomes of patients with vascular anomalies in southeast China.

METHODSA total of 592 vascular anomalies patients (patients with intracranial tissues or viscera involved were excluded), admitted to the First Affiliated Hospital of Sun Yat-sen University from January 2006 to September 2009, were enrolled in the study. Data for clinical characteristics, diagnosis, therapies and outcomes were collected and analyzed.

RESULTSOf the 592 patients, the male:female ratios in the vascular tumor group (n = 187) and the vascular malformation group (n = 405) were 1:1.49 and 1:1.06 respectively, with no significant difference between them. The mean onset age of the vascular tumor group was significantly younger than that of the vascular malformation group (p < 0.001). The head and neck were the most commonly (31.4%) involved areas in vascular anomalies. A total of 23.8% of the patients with vascular anomalies had definite symptoms caused by the vascular lesions. In the vascular tumor group, 94.1% of them were infantile hemangiomas. Venous malformation was the most common (41.0%) subtype of vascular malformations. Surgical therapy was undertaken in 94.2% of the patients with vascular anomalies. Of the 519 patients available for the 16 - 58 month follow-up, 322 patients (62.0%) were cured, 108 patients (20.8%) were markedly improved, 57 patients (11.0%) were partially improved, and 32 patients (6.2%) were uncured.

CONCLUSIONSVascular anomalies are clinically heterogeneous. While the outcome is generally favorable, further effort should be made to determine the appropriate terminology and management.

Adolescent ; Blood Vessels ; abnormalities ; Child ; Child, Preschool ; China ; epidemiology ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Middle Aged ; Retrospective Studies ; Vascular Neoplasms ; epidemiology

A Le Fort I osteotomy is a common procedure for correcting dental and facial deformities in orthognathic surgery. In rare cases, a delayed hemorrhage can occur as early as several hours or up to 12 weeks, postoperatively. The most frequently involved blood vessels in a delayed hemorrhage are the descending palatine artery, the internal maxillary artery, and the pterygoid venous plexus of veins. Intraoral bleeding accompanied by severe epistaxis in these cases makes it difficult to locate the precise bleeding focus. Eventual uncontrolled bleeding would require Merocel packing or surgical intervention. In general, a severe late postoperative hemorrhage is most effectively managed by angiography and embolization. Herein we describe a delayed hemorrhage case in which the cause was not evident on angiography. We were able to detect the bleeding point through an endoscopic nasal approach and treat it using direct cauterization.
Aneurysm, False ; Angiography ; Arteries ; Blood Vessels ; Cautery ; Congenital Abnormalities ; Epistaxis ; Hemorrhage ; Maxillary Artery ; Orthognathic Surgery ; Osteotomy ; Postoperative Complications ; Postoperative Hemorrhage ; Veins

OBJECTIVE: Craniovertebral junctional anomalies constitute a technical challenge. Surgical opening of atlantoaxial joint region is a complex procedure especially in patients with nuchal deformity like basilar invagination. This region has actually very complicated anatomical and functional characteristics, including multiple joints providing extension, flexion, and wide rotation. In fact, it is also a bottleneck region where bones, neural structures, and blood vessels are located. Stabilization surgery regarding this region should consider the fact that the area exposes excessive and life-long stress due to complex movements and human posture. Therefore, all options should be considered for surgical stabilization, and they could be interchanged during the surgery, if required.METHODS: A 53-year-old male patient applied to outpatients’ clinic with complaints of head and neck pain persisting for a long time. Physical examination was normal except increased deep tendon reflexes. The patient was on long-term corticosteroid due to an allergic disease. Magnetic resonance imaging and computed tomography findings indicated basilar invagination and atlantoaxial dislocation. The patient underwent C0–C3–C4 (lateral mass) and additional C0–C2 (translaminar) stabilization surgery.RESULTS: In routine practice, the sites where rods are bound to occipital plates were placed as paramedian. Instead, we inserted lateral mass screw to the sites where occipital screws were inserted on the occipital plate, thereby creating a site where extra rod could be bound. When C2 translaminar screw is inserted, screw caps remain on the median plane, which makes them difficult to bind to contralateral system. These bind directly to occipital plate without any connection from this region to the contralateral system. Advantages of this technique include easy insertion of C2 translaminar screws, presence of increased screw sizes, and exclusion of pullout forces onto the screw from neck movements. Another advantage of the technique is the median placement of the rod; i.e., thick part of the occipital bone is in alignment with axial loading.CONCLUSION: We believe that this technique, which could be easily performed as adjuvant to classical stabilization surgery with no need for special screw and rod, may improve distraction force in patients with low bone density.
Atlanto-Axial Joint ; Blood Vessels ; Bone Density ; Congenital Abnormalities ; Dislocations ; Head ; Humans ; Joints ; Magnetic Resonance Imaging ; Male ; Methods ; Middle Aged ; Neck ; Neck Pain ; Occipital Bone ; Physical Examination ; Posture ; Reflex, Stretch ; Spine