Efficacy and safety of recombinant human soluble thrombomodulin (ART-123) in disseminated intravascular coagulation: results of a phase III, randomized, double-blind clinical trial.

BACKGROUND: Soluble thrombomodulin is a promising therapeutic natural anticoagulant that is comparable to antithrombin, tissue factor pathway inhibitor and activated protein C. OBJECTIVES: We conducted a multicenter, double-blind, randomized, parallel-group trial to compare the efficacy and safety of recombinant human soluble thrombomodulin (ART-123) to those of low-dose heparin for the treatment of disseminated intravascular coagulation (DIC) associated with hematologic malignancy or infection. METHODS: DIC patients (n = 234) were assigned to receive ART-123 (0.06 mg kg(-1) for 30 min, once daily) or heparin sodium (8 U kg(-1) h(-1) for 24 h) for 6 days, using a double-dummy method. The primary efficacy endpoint was DIC resolution rate. The secondary endpoints included clinical course of bleeding symptoms and mortality rate at 28 days. RESULTS: DIC was resolved in 66.1% of the ART-123 group, as compared with 49.9% of the heparin group [difference 16.2%; 95% confidence interval (CI) 3.3-29.1]. Patients in the ART-123 group also showed more marked improvement in clinical course of bleeding symptoms (P = 0.0271). The incidence of bleeding-related adverse events up to 7 days after the start of infusion was lower in the ART-123 group than in the heparin group (43.1% vs. 56.5%, P = 0.0487). CONCLUSIONS: When compared with heparin therapy, ART-123 therapy more significantly improves DIC and alleviates bleeding symptoms in DIC patients.

Efficacy of endoscopic hemoclipping for GI bleeding in relation to severity of shock.

BACKGROUND/AIMS: To examine the efficacy of prospectively performing endoscopic hemoclipping in patients with severe gastrointestinal bleeding by assessing in relation to the severity of the case. METHODOLOGY: Endoscopic hemoclipping was performed in 44 patients in shock caused by severe gastrointestinal bleeding. The level of severity was categorized using two factors. One factor was the presence or absence of severe shock, and the other was the presence or absence of active bleeding at endoscopy. Age, blood transfusion volume, APACHE III score, coagulopathy, initial hemostatic, rebleeding and mortality rate were recorded in relation to those four subsets. If rebleeding occurred, the systolic blood pressure, heart rate and serum hemoglobin concentration were compared between the initial time of bleeding and rebleeding. RESULTS: Initial hemostasis was successfully achieved in all cases. The severe shock group required significantly more blood transfusions. The most severe subset had the highest APACHE III score, and coagulopathy. Rebleeding occurred in seven cases in the severe shock group only. In the rebleeding group, the systolic body pressure was lower, heart rate was higher, and serum hemoglobin concentration was lower than the non-rebleeding group. Four patients, all in subset 1, died, but no patient died due to gastrointestinal bleeding. CONCLUSIONS: The endoscopic hemoclipping method is very effective for severe gastrointestinal bleeding in shock. The severity of shock was a more important risk factor than the presence of active bleeding. Our category of severity is simple, it reflects the patients' clinical condition accurately, and is very useful for patients with gastrointestinal bleeding.

Transient hematopoietic stem cell rescue using umbilical cord blood for a lethally irradiated nuclear accident victim.

We performed stem cell rescue and allogeneic skin transplantation on a lethally neutron-irradiated nuclear accident victim. HLA-DRB1 mismatched unrelated umbilical cord blood cells (2.08 x 10(7)/kg recipient body weight) were transplanted to an 8-10 Gy equivalent neutron-irradiated patient because of a lack of a suitable bone marrow or peripheral blood donor. Pre-transplant conditioning consisted of anti-thymocyte gamma-globulin alone, and GVHD prophylaxis was a combination of cyclosporine (CYA) and methylprednisolone (mPSL). Granulocyte colony-stimulating factor (G-CSF), erythropoietin (EPO), and thrombopoietin (TPO) were concurrently administered after transplantation. The absolute neutrophil count reached 0.5 x 10(9)/l on day 15, the reticulocyte count rose above 1% on day 23, and the platelet count was over 50 x 10(9)/l on day 27, respectively. Cytogenetic studies of blood and marrow showed donor/recipient mixed chimerism. Rapid autologous hematopoietic recovery was recognized after withdrawal of CYA and mPSL. Repeated pathological examinations of the skin revealed no evidence of acute GVHD. Eighty-two days after the irradiation, skin transplantation was performed to treat radiation burns. Almost 90% of the transplanted skin engrafted. Immunological examination after autologous hematopoietic recovery revealed an almost normal T cell count. However, immune functions were severely impaired. The patient died from infectious complication 210 days after the accident.

[A patient with facioscapulohumeral muscular dystrophy accompanied by myasthenia gravis].

A patient was a fifty-year-old man, who had a 35 year-history of facioscapulohumeral muscular dystrophy (FSHD). He was admitted to our hospital because of acute progressive weakness involving his lower extremities without any fluctuation in the recent 3 weeks. We clinically followed him for 30 years and he was able to do all daily activities, walked alone, drove a car and climbed stairs with a handrail. His 76-year-old mother had about 60 year-history of FSHD and could walk with support. On admission, neurological examination revealed moderate to marked muscle weakness and atrophy of the face, limb-girdle and all extremities, predominantly in the upper proximal limbs. He could hardly stand and needed a stick for walking. He had no blepharoptosis or ocular movement disturbance, and did not complain of difficulties in swallowing and chewing. CK values and other laboratory data were normal, and serum anti-Jo-1 antibody, anti-SSA/Ro antibody and anticardiolipin IgG antibody were negative. Because EMG examination revealed myopathic changes and an X-ray examination of the lumbar spine was normal. Thus, polymyositis and neurologenic disorders were ruled out. Disturbance in chewing and swallowing, that were uncommon in FSHD, appeared about a month after admission. Repetitive stimulation test revealed typical waning pattern. Edrophonium chloride injection was effective for decreased waning and the clinical symptoms. The titer of serum anti-ACh receptor antibody was 97 nmol/l, confirming the diagnosis of myasthenia gravis. Because of fluctuated dyspnea, thymectomy was done and his condition gradually relieved after administration of corticosteroid and choline esterase inhibitor. From this experience, we learned that we have to consider other neuromuscular disorders, even rare ones, if there existed unusual weakness of underlying muscular dystrophy.

The role of interventional radiology in the management of blunt renal injury: a practical protocol.

OBJECTIVE: The purpose of this study was to evaluate the efficacy of a protocol designed to minimize the need for surgery in the management of severe blunt renal injury. METHODS: Forty-six of 752 trauma patients had evidence of renal injury on computed tomographic (CT) scan. Two patients required emergency laparotomy, and the remaining 44 patients were classified by CT scan grade using the American Association for the Surgery of Trauma classification system. Patients with CT scan grade 3 or over underwent renal angiography. RESULTS: Twenty-one patients had a high-grade injury on CT scan (> or =3). Eight had angiographic evidence of extravasation from renal arterial branches and underwent transarterial embolization. One patient with a grade 5 injury had extravasation from a main renal vein and underwent immediate laparotomy. This was the only patient who required surgery for renal injury. CONCLUSION: Surgery can be avoided in most cases of blunt renal injury. Hemodynamic instability and injury to main renal veins remain indications for surgical exploration.

Spontaneous rupture of the pancreaticoduodenal artery possibly related to prior occlusion of the common hepatic artery: report of a case.

We report herein a case of spontaneous rupture of the pancreaticoduodenal artery (PDA) associated with obstruction of the common hepatic artery. A 68-year-old man was admitted to our hospital following the sudden onset of severe upper abdominal pain. Computed tomography revealed a large mass formation in the peritoneal cavity. Hemorrhagic shock rapidly developed during the initial evaluation, necessitating an exploratory laparotomy to be performed in the emergency room. This revealed a large hematoma in the retroperitoneal space, and a ruptured PDA was sutured. Postoperative angiography showed obstruction of the common hepatic artery and also suggested that the source of the bleeding was the PDA. Thus, a diagnosis of spontaneous rupture of a PDA aneurysm associated with occlusion of the common hepatic artery was made. Following this case report, we discuss the development of true aneurysms of the PDA and the treatment of ruptured true PDA aneurysms resulting in shock.

Reduction of resuscitation fluid volumes in severely burned patients using ascorbic acid administration: a randomized, prospective study.

HYPOTHESIS: High-dose ascorbic acid (vitamin C) therapy (66 mg/kg per hour) attenuates postburn lipid peroxidation, resuscitation fluid volume requirements, and edema generation in severely burned patients. STUDY DESIGN AND SETTING: A prospective, randomized study at a university trauma and critical care center in Japan. SUBJECTS AND METHODS: Thirty-seven patients with burns over more than 30% of their total body surface area (TBSA) hospitalized within 2 hours after injury were randomly divided into ascorbic acid and control groups. Fluid resuscitation was performed using Ringer lactate solution to maintain stable hemodynamic measurements and adequate urine output (0.5-1.0 ml/kg per hour). In the ascorbic acid group (n = 19; mean burn size, 63% +/- 26% TBSA; mean burn index, 57 +/- 26; inhalation injury, 15/ 19), ascorbic acid was infused during the initial 24-hour study period. In the control group (n = 18; mean burn size, 53% +/- 17% TBSA; mean burn index, 47 +/- 13; inhalation injury, 12/18), no ascorbic acid was infused. We compared hemodynamic and respiratory measurements, lipid peroxidation, and fluid balance for 96 hours after injury. Two-way analysis of variance and Tukey test were used to analyze the data. RESULTS: Heart rate, mean arterial pressure, central venous pressure, arterial pH, base deficit, and urine outputs were equivalent in both groups. The 24-hour total fluid infusion volumes in the control and ascorbic acid groups were 5.5 +/- 3.1 and 3.0 +/- 1.7 mL/kg per percentage of burn area, respectively (P<.01). In the first 24 hours, the ascorbic acid group gained 9.2% +/- 8.2% of pretreatment weight; controls, 17.8% +/- 6.9%. Burned tissue water content was 6.1 +/- 1.8 vs 2.6 +/- 1.7 mL/g of dry weight in the control and ascorbic acid groups, respectively (P<.01). Fluid retention in the second 24 hours was also significantly reduced in the ascorbic acid group. In the control group, the ratio of PaO2 to fraction of inspired oxygen at 18, 24, 36, 48, and 72 hours after injury was less than that of the ascorbic acid group (P<.01). The length of mechanical ventilation in the control and ascorbic acid groups was 21.3 +/- 15.6 and 12.1 +/- 8.8 days, respectively (P<.05). Serum malondialdehyde levels were lower in the ascorbic acid group at 18, 24, and 36 hours after injury (P<.05). CONCLUSIONS: Adjuvant administration of high-dose ascorbic acid during the first 24 hours after thermal injury significantly reduces resuscitation fluid volume requirements, body weight gain, and wound edema. A reduction in the severity of respiratory dysfunction was also apparent in these patients.

Molecular epidemiological investigation using a randomly amplified polymorphic DNA assay of Burkholderia cepacia isolates from nosocomial outbreaks.

We experienced two Burkholderia cepacia outbreaks over a 1-year period. During this period, 28 B. cepacia isolates were obtained from clinical specimens, and 2 were obtained from environmental specimens (i.e., from a nebulizer solution and a nebulizer tube). These 30 isolates were subjected to the PCR-based randomly amplified polymorphic DNA (RAPD) assay as well as to pulsed-field gel electrophoresis (PFGE). In the first outbreak, in which eight patients hospitalized in the Trauma and Critical Care Center were involved, the RAPD assay revealed that all 20 isolates obtained from clinical specimens and the 2 isolates from environmental specimens had identical DNA profiles. These RAPD data enabled us to pinpoint a possible source and to take countermeasures to prevent further spread of the epidemic-causing strain. In the second outbreak, two consecutive B. cepacia infection/colonization cases were seen in the surgery ward. The RAPD profiles of four isolates obtained were again identical, but they were distinct from those seen in the first outbreak, clearly indicating that the second outbreak was not related to the first. Thus, our experience demonstrated that the RAPD assay is a useful and reliable tool for epidemiological studies of B. cepacia isolates from nosocomial outbreaks. Since the RAPD assay could provide discriminatory potential and reproducibility comparable to those of the widely used PFGE assay with less complexity and in a shorter time, the introduction of the RAPD assay into hospital microbiology laboratories as a routine technique may help prevent nosocomial outbreaks.

Enzyme-free method for cultured skin grafting.

Cultured skin autografting has promise to improve the prognosis of severe burn injury. Established methods for cultured epithelial cell autografting need enzymatic proteolytic reaction that have potential risks of losing cell surface structures and causing allergic reactions in the recipients. Therefore, we have developed an enzyme-free method of cultured skin autografting. Donor skin was obtained in 0.2-0.3 mm thickness during an operation of debriding burned eschar. The skin was cut into pieces and spread on removable soft culture surface membrane in culture plates. Modified MCDB 153 medium with 10 microg/L epidermal growth factor and 150 mg/L bovine pituitary extract was used. The tissues were incubated at 37 degrees C in a water-saturated atmosphere of 5% CO2 in air. After 3 weeks, the cultured tissue on the membrane was removed mechanically from the bottom of the plates and implanted on granulated recipient sites in upside down manner. The cultured auto-skin grafting was successfully performed in two patients with severe burn. The recipient sites were partially epithelized in 2 weeks and maintained thereafter. Our method is performed easily using partial thickness donor skin without enzymatic processes.

Laparoscopic drainage of an intramural duodenal hematoma.

A 21-year-old man was admitted with vomiting and abdominal pain 3 days after sustaining blunt abdominal trauma by being tackled in a game of American football. A diagnosis of intramural hematoma of the duodenum was made using computed tomography and upper gastrointestinal tract contrast radiography. The hematoma caused obstructive jaundice by compressing the common bile duct. The contents of the hematoma were laparoscopically drained. A small perforation was then found in the duodenal wall. The patient underwent laparotomy and repair of the injury. Laparoscopic surgery can be used as definitive therapy in this type of abdominal trauma.

Sensory afferent properties of immobilised or inflamed rat knees during continuous passive movement.

We studied the sensory afferent properties of normal, immobilised and inflamed rat knees by recording the activity of the medial articular nerve (MAN). When the knee was inflamed by kaolin-carrageenan or immobilised for six weeks, MAN activity significantly increased during rest and continuous passive motion (CPM). The maximal discharge rate tended to increase depending on the angular velocity of the CPM. When the knees were then rested for one hour before again starting CPM, activity was further increased at the initial CPM cycle, the 'post-rest effect'. Analysis of the conduction velocity showed that 94% and 66% of spike units on the recorded discharge of the immobilised and inflamed knees, respectively, belonged to fine nerve fibres. Our findings show that the sensory receptors in the knee are sensitised in a similar manner by immobilisation and by inflammation, suggesting a relationship to pain. The post-rest effect may be related to a characteristic symptom of osteoarthritis called 'starting pain'.

Hyperechoic appearance of hepatic parenchyma on ultrasound examination of patients with blunt hepatic injury.

OBJECTIVE: To study the significance of a geographic hyperechoic liver parenchyma pattern on ultrasound (US) examination of patients with blunt abdominal injury. DESIGN: Prospective clinical study with double-blind evaluation of images and clinical data. METHODS AND MAIN RESULTS: We performed US examinations in 831 consecutive patients admitted to our hospital for blunt abdominal trauma and identified 33 with a geographic hyperechoic pattern in the liver. We correlated the appearance with computed tomographic images and with clinical, angiographic, and scintigraphic data. All patients with a geographic hyperechoic pattern showed mild computed tomographic evidence of hepatic injury (Mirvis grade 2, 69%; Mirvis grade 3, 31%). Excluding patients who required urgent surgery for other reasons and patients in shock, patients with the geographic hyperechoic pattern were managed conservatively with no complications. CONCLUSION: The geographic hyperechoic pattern of liver parenchyma on US examination of trauma patients is a mild injury that, of itself, does not require surgical therapy.

Nonsurgical management of patients with blunt hepatic injury: efficacy of transcatheter arterial embolization.

OBJECTIVE: We evaluated the efficacy of transcatheter arterial embolization (TAE) for patients with blunt hepatic injury. SUBJECTS AND METHODS: Of 372 patients with trauma, 60 had evidence on CT of hepatic injury (Mirvis classification). Six of the 60 patients required emergency laparotomy and were excluded. Of the 54 remaining patients, 28 were classified as having high-grade hepatic injury (Mirvis classification of 3 or 4). All 28 underwent arteriography, and TAE was performed in single or multiple hepatic arterial branches when extravasation was seen. Angiography was repeated and cholescintigraphy was performed on patients with continued bleeding or biloma. RESULTS: Injuries detected were grade 1 (n = 13), grade 2 (n = 13), grade 3 (n = 20), and grade 4 (n = 8). The injury was correlated with the degree of hemoperitoneum seen on CT. Patients with low-grade injuries (Mirvis classification of 1 or 2) were treated conservatively, and no deaths or liver-related morbidity occurred. Of the 28 patients with high-grade injury, 15 also had angiographic evidence of extravasation and underwent TAE. The average fluid resuscitation volume was significantly larger in this group than in the other 13 patients with high-grade injuries who did not undergo TAE. Embolization was successful in all 15 patients, and the shock index was significantly reduced after TAE. All patients survived, with follow-up at 1-8 months (2.5 +/- 1.8 months, mean +/- SD). CONCLUSION: TAE is an effective alternative to surgery for patients with high-grade liver injury.

Reduced resuscitation fluid volume for second-degree burns with delayed initiation of ascorbic acid therapy.

BACKGROUND: Oxygen radicals may play an important role in injury due to thermal burns. High-dose antioxidant ascorbic acid (vitamin C, Cenolete, Abbott Laboratory, Abbott Park, Ill) therapy reduces edema of burned and unburned tissue, lipid peroxidation, and subsequent resuscitation fluid volume requirement in experimental burn models. OBJECTIVE: To determine the hemodynamic effects of delayed initiation (2 hours after injury) of antioxidant therapy in patients with second-degree burns. DESIGN: Experimental study. MATERIALS AND INTERVENTION: Burns over 70% of body surface area were produced by subxiphoid immersion of 12 guinea pigs in 100 degrees C water for 3 seconds. The animals were resuscitated with Ringer's lactated solution according to the Parkland formula (4 mL/kg for 1% of burned body surface area during the first 24 hours) from 0.5 to 2 hours following injury, after which the resuscitation fluid volume was reduced to 25% of the Parkland formula. Animals received Ringer's lactated solution to which ascorbic acid (340 mg/kg during the first 24 hours) was added (vitamin C group [n = 6]) or Ringer's lactated solution only (control group [n = 6]). MEASUREMENTS: Heart rates, mean arterial blood pressure, cardiac output, hematocrit level, and water content in burned and unburned tissue were measured before injury and at intervals thereafter. RESULTS: There were no significant differences in heart rates (P = .29) and blood pressures (P = .53) between the 2 groups throughout the 24-hour study period. No animal died. The vitamin C group showed significantly lower hematocrit levels (P < .05) and significantly higher cardiac output values (P < .05) at 7 hours following burn injury and at intervals thereafter (P < .001). CONCLUSION: With delayed initiation of high-dose ascorbic acid therapy, the 24-hour fluid resuscitation volume was reduced to 32.5% of the Parkland formula, while maintaining adequate cardiac output values.

[Free radicals and surgical stress].

Free radicals have at least one unpaired electron. Some of them have very high reactivity and harmful to cells and tissues. O2-. and HO. are generated during re-perfusion phase of shock. Both induce lipid peroxidation and this attack initiates auto-continuing injury of membrane lipid by lipid peroxide. This may result in damage of membrane function and permeability alteration. NO is an another type of free radical and huge amount of NO is generated by inducible NO synthase with variety of stimulation. NO decreases vascular tonus and may be related with hyperdynamic circulatory status in sepsis. NOx and ONOO- which are NO derivatives, deteriorate mitochondrial function. Free radicals are one of major agents which damages patients under surgical stress, however, they also have important rules in our defense system. In this sense, control rather than suppression may be important to ameliorate injury caused by surgical stress.

Nonsurgical management of patients with blunt splenic injury: efficacy of transcatheter arterial embolization.

OBJECTIVE: We evaluated the efficacy of nonsurgical management of patients with blunt splenic injury using detailed angiographic examinations and transcatheter arterial embolization. SUBJECTS AND METHODS: We prospectively studied 228 patients who had blunt abdominal injury and for whom CT was performed. When splenic injury was revealed by CT, angiography was performed in all patients except those requiring emergency surgery. Transcatheter arterial embolization was performed when patients had the following angiographic criteria: (1) extravasation of contrast material extending beyond or within the splenic parenchyma, (2) arterial disruption or major arteriovenous fistula, or (3) both. Splenic function was subsequently estimated by 99mTc-sulfur colloid scintigraphy and repeat angiography. RESULTS: Of 228 patients with blunt trauma, 31 patients had CT evidence of splenic injury. In three of these 31 patients, emergency laparotomy was performed before angiography because of an associated injury or unstable circulatory status. In 13 of the 28 remaining patients, transcatheter arterial embolization was not required as these patients did not meet the necessary criteria. They were treated with bed rest. Transcatheter arterial embolization was performed in the remaining 15 patients and was completely successful in 13. Because one of these 13 patients died of a brain contusion, follow-up angiography and scintigraphy were performed in the remaining 12 patients and showed preservation of splenic function. Nonsurgical treatment of splenic injury with angiography was successful in 93% of patients. CONCLUSION: Our success rate for nonsurgical management of patients with blunt splenic injury should encourage more extensive evaluation and use of angiography for splenic injury and the subsequent management of splenic injury without surgery.

[A case report of surgical management of blunt ruptured right atrium with blunt ruptured liver].

This is a case report of a 23 year old female involved in a car accident that caused blunt trauma to the patient. First of all, she was found to have cardiac tamponade when the abdomen was explored to suture and to put the gaze compression on for hemostasis of the ruptured liver. Then, she was brought to our institution by the ambulance. Upon the exploration of the heart under standby of extracorporal circulation (ECC), small multiple lacerations were found at the junction of the right atrium and superior vena cava. These were sutured directly to close without ECC. On the 2nd postoperative day, she was bought to the OR again to removal of the gaze tamponade from the ruptured liver and to complete hemostasis. The patient was discarded 35 days after admission.

Multicore myopathy associated with multiple pterygium syndrome and hypertrophic cardiomyopathy.

A boy with multicore myopathy associated with multiple pterygium syndrome and hypertrophic cardiomyopathy is described. Light microscopy of biopsy samples from the skeletal muscle and myocardium revealed multiple cores in the muscle fibers in the former but their absence in the latter. These results suggest that the pathogenesis of the histologic changes might differ between skeletal muscle and myocardium, and that further electron microscopic examination be done on both types of specimen. The prognosis of multicore myopathy is not usually good when cardiac involvement is present.

Effects of the antiprotease ulinastatin on mortality and oxidant injury in scalded rats.

BACKGROUND: Proteolysis and oxidant injury are important mechanisms of injury in thermal burns. The glycoprotein ulinastatin has antiprotease and free radical-scavenging properties and therefore could be useful in the treatment of these injuries. OBJECTIVE: To determine the free radical-scavenging effects of ulinastatin on rats with thermal injury. DESIGN: An experimental study. MATERIALS AND INTERVENTIONS: Sixteen rats with a 70% surface area immersion scald were treated intravenously for 3 hours; immediately after injury, the rats were treated with ulinastatin, 100,000 U/kg, in lactated Ringer's injection. Sixteen control rats were also scalded but were given only lactated Ringer's injection. Sixteen additional sham control rats were immersed in 37 degrees C water and given ulinastatin. MEASUREMENTS: Levels of malondialdehyde, superoxide dismutase, lactate, and pyruvate in skin, along with the water content of the skin, were measured before injury and at intervals thereafter in six rats from each group. RESULTS: The mortality rate was 50% at 72 hours and 90% at 14 days in scalded control rats, but it was 0% at 72 hours and 20% at 14 days in the treated rats (P < .01). Levels of malondialdehyde and superoxide dismutase in the skin and the lactate-pyruvate ratio all remained at the same values (as those before scalding) in treated rats but rose in the skin of scalded control rats (P < .05 for each). The water content of scalded skin gradually increased after injury in all groups, but at 3 hours, the scalded skin in treated rats contained less water than that in scalded control rats (P < .05). CONCLUSION: Ulinastatin may have a therapeutic role in treating thermal injuries.

Left aortic arch with right descending aorta and right ligamentum arteriosum associated with d-TGA and large VSD: surgical treatment of a rare form of vascular ring.

A 1-month-old boy with respiratory distress underwent pulmonary artery banding after diagnosis of d-loop transposition of the great arteries and ventricular septal defect. Respiratory distress recurred after surgery. Angiography, esophagography, and magnetic resonance imaging showed left aortic arch, retroesophageal aorta, and right descending aorta. Surgery at 9 months of age showed a right-sided ligamentum arteriosum. Division of the ligamentum relieved the constriction of the esophagus and trachea caused by this rare form of vascular ring.