Treatment of post-pulsed dye laser purpura with pulsed dye laser.

The pulsed dye laser (PDL) is used for the treatment of a variety of vascular and nonvascular disorders, and its therapeutic efficacy often depends on purpura as an endpoint. However, post-PDL purpura is an undesirable side effect of treatment as it may increase patient dissatisfaction and downtime. Recent studies have suggested that repeat treatment with PDL may speed the resolution of post-procedural purpura; however, there is paucity of data on using repeat PDL treatment specifically for post-PDL purpura. We report three cases of 585 nm PDL-induced purpura treated with 595 nm PDL with clinically significant improvement. We suggest that treatment with PDL, which targets hemoglobin and its breakdown products, after initial PDL treatment may reduce post-PDL purpura.

Recent advances in liver transplantation for metabolic disease.

The indications and outcomes of liver transplantation for metabolic disease have been reviewed recently and this short review concentrates on recent developments and advances. Recently recognized metabolic causes of acute liver failure are reviewed and their implications for transplantation discussed. Newly described indications for liver transplantation in systemic metabolic diseases are described and an update is given on the role of auxiliary and domino liver transplantation.

Liver transplant in ethylmalonic encephalopathy: a new treatment for an otherwise fatal disease.

Ethylmalonic encephalopathy is a fatal, rapidly progressive mitochondrial disorder caused by ETHE1 mutations, whose peculiar clinical and biochemical features are due to the toxic accumulation of hydrogen sulphide and of its metabolites, including thiosulphate. In mice with ethylmalonic encephalopathy, liver-targeted adeno-associated virus-mediated ETHE1 gene transfer dramatically improved both clinical course and metabolic abnormalities. Reasoning that the same achievement could be accomplished by liver transplantation, we performed living donor-liver transplantation in an infant with ethylmalonic encephalopathy. Unlike the invariably progressive deterioration of the disease, 8 months after liver transplantation, we observed striking neurological improvement with remarkable achievements in psychomotor development, along with dramatic reversion of biochemical abnormalities. These results clearly indicate that liver transplantation is a viable therapeutic option for ETHE1 disease.

Treatment of surgical scars using a 595-nm pulsed dye laser using purpuric and nonpurpuric parameters: a comparative study.

BACKGROUND: Many studies have examined laser treatment of scars, but cosmetic results have been variable. Although no studies have examined the effect of purpura on scar improvement using the pulsed dye laser (PDL), many clinicians believe inducing purpura results in better and quicker improvement. OBJECTIVE: To determine whether PDL treatment of fresh surgical scars with purpura-inducing settings improves clinical appearance more than non-purpura-inducing settings or no treatment. METHODS: Twenty-six subjects with surgical scars enrolled in this prospective study. Scars were divided into three equal segments; treatment was randomized: 595-nm PDL with purpuric (1.5 ms) or nonpurpuric (10 ms) settings or no treatment. Fluences were adjusted to Fitzpatrick skin type. Scars were treated three times, 1 month apart, beginning at suture removal. Outcome measures included Vancouver Scar Scale (VSS) and blind clinical ratings. RESULTS: The nonpurpuric condition showed significant improvement on the VSS total score, vascularity, and pliability ratings. The purpuric condition demonstrated a trend for improvement on the VSS total. According to blind observer ratings, all conditions improved, without differences between groups. CONCLUSION: Nonpurpuric settings on the PDL resulted in significant improvements in the appearance of fresh surgical scars for vascularity, pliability, and VSS total scores, although all scar segments improved over time.

Intramedullary lesion expansion on magnetic resonance imaging in patients with motor complete cervical spinal cord injury.

OBJECT: The authors performed a study to determine if lesion expansion occurs in humans during the early hours after spinal cord injury (SCI), as has been established in rodent models of SCI, and to identify factors that might predict lesion expansion. METHODS: The authors studied 42 patients with acute cervical SCI and admission American Spinal Injury Association Impairment Scale Grades A (35 patients) and B (7 patients) in whom 2 consecutive MRI scans were obtained 3-134 hours after trauma. They recorded demographic data, clinical information, Injury Severity Score (ISS), admission MRI-documented spinal canal and cord characteristics, and management strategies. RESULTS: The characteristics of the cohort were as follows: male/female ratio 37:5; mean age, 34.6 years; and cause of injury, motor vehicle collision, falls, and sport injuries in 40 of 42 cases. The first MRI study was performed 6.8 ±2.7 hours (mean ± SD) after injury, and the second was performed 54.5 ± 32.3 hours after injury. The rostrocaudal intramedullary length of the lesion on the first MRI scan was 59.2 ± 16.1 mm, whereas its length on the second was 88.5 ± 31.9 mm. The principal factors associated with lesion length on the first MRI study were the time between injury and imaging (p = 0.05) and the time to decompression (p = 0.03). The lesion's rate of rostrocaudal intramedullary expansion in the interval between the first and second MRI was 0.9 ± 0.8 mm/hour. The principal factors associated with the rate of expansion were the maximum spinal cord compression (p = 0.03) and the mechanism of injury (p = 0.05). CONCLUSIONS: Spinal cord injury in humans is characterized by lesion expansion during the hours following trauma. Lesion expansion has a positive relationship with spinal cord compression and may be mitigated by early surgical decompression. Lesion expansion may be a novel surrogate measure by which to assess therapeutic effects in surgical or drug trials.

Splenic peliosis: a rare complication following liver transplantation.

In this article, we report a rare case of isolated splenic peliosis in an individual who had recently undergone liver transplantation. The disorder had remained clinically and radiologically undiagnosed until he suffered a traumatic rupture of the affected organ. The relevant literature on this topic is briefly reviewed.

Are there specific haemostatic abnormalities in children surviving septic shock with purpura and having skin necrosis or limb ischaemia that need skin grafts or limb amputations?

UNLABELLED: More than 10% of children surviving septic shock with purpura have skin necrosis or limb ischaemia (SNLI.). Among 44 children consecutively admitted to our pediatric intensive care unit, 35 (80%) survived, 6 of them (17%) developed SNLI (defined as the need of a surgical procedure). Two timed haemostasis measurements included the determination of coagulation factors, protein C (PC), protein S (PS), C4b binding protein (C4bBP), antithrombin (AT), and plasminogen activator inhibitor 1 (PAI-1). Two severity scores and CRP levels were determined at admission. Children with SNLI and without SNLI were compared. On admission, severity scores, and AT, PC, PS, C4bBP levels were similar in both groups with and without SNLI. Prothrombin time (23% vs 34%; P < 0.01), factor VII+X (20% vs 31%; P = 0.05) and factor VII (0% vs 19%; P < 0.01) were lower in the group with SNLI. The 2nd sample showed no difference between the two groups. Kinetics of haemostatic abnormalities were no different between the two groups. CONCLUSION: In this series, the only difference between the two groups was lower factor VII levels in children with skin necrosis or limb ischaemia. This suggests the benefit of tissue factor pathway inhibitor administration as an adjunctive therapy to prevent skin necrosis or limb ischaemia. Further studies including more children are needed to determine the potential effects of treatments such as protein C, antithrombin, and plasminogen activator inhibitor antibody administration, and to advocate tissue factor pathway inhibitor in preventing skin necrosis or limb ischaemia.

[Laparoscopic splenectomy: value of the posterior approach]. / Splénectomie laparoscopique: intérêt de l'abord postérieur.

UNLABELLED: Intraoperative bleeding is the main complication and main cause of conversion to open surgery during laparoscopic splenectomy (LS). We present the advantages of the posterior approach to splenic vessels. PATIENTS: We have performed a total of 52 LS using several approaches. In the last 24 patients, we used a posterior approach to the splenic vessels with the patient in full lateral position. Only three ports were used. The major part of the dissection was performed from behind, thus allowing safer vascular control. The division of short gastric vessels and lower pole vessels was performed using US. The main vascular pedicle was stapled. The spleen was removed through a Pfannenstiel incision. The patients were 11 males and 13 females with a mean age of 38 years (17-71 years). Sixteen had immune thrombocytopenic purpura (ITP), 2 had HIV infection related purpura 3 had haemolytic anemia and 2 had spherocytosis. RESULTS: There was no conversion to laparotomy. The average splenic weight was 372 g (162-1420 g). In all but one patient, the intraoperative blood loss was less than 60 cc and was nil in 7 patients. The average operative time was 126 min (70-220), including the time required for change of position and Pfannenstiel incision. There was no mortality. All but one patient had an uneventful postoperative course. The HIV infected patient developed severe postoperative pancreatitis. In those an patients with uncomplicated course, the average postoperative stay was 4.1 days (2-8 days). CONCLUSION: The lateral position with a posterior approach to splenic vessels allows for a safe vascular control. This is illustrated by the average intraoperative bleeding of this series which is much lower than that observed in our previous experience and in other published series.

The role of moist wound healing in the management of meningococcal skin lesions.

This report describes the management of haemorrhagic skin lesions in a patient with meningococcal meningitis.

Surgical intervention for the complications of meningococcal-induced purpura fulminans.

Purpura fulminans is an infrequent but sometimes catastrophic illness that usually complicates a viral, rickettsial or bacterial infection. This communication presents a retrospective review of 152 patients with meningococcemia hospitalized at Children's Medical Center of Dallas from January, 1983, through December, 1993. Eighteen (11.9%) of the 152 patients developed purpura fulminans. Thirteen (72%) of the 18 patients with purpura fulminans needed one or more surgeries including skin grafts, local debridement, microvascular flaps or amputations. Five patients (28%) died.

Changing role of splenectomy for hematologic disease.

In the past decade the development of accurate imaging and the evolution of the medical management of hematologic diseases has led to changes in the indications for splenectomy for these disorders. To assess the impact of these developments, a multi-institutional, retrospective review was undertaken. One hundred fifty-six splenectomies were performed for hematologic disorders between July 1, 1979 and June 30, 1991. Patients were divided into 2 groups: those undergoing splenectomy from 1979-1985 (Period I), and those undergoing splenectomy from 1986-1991 (Period II). Diseases were classified into 3 groups: cytopenic/anemic conditions, symptomatic splenomegaly, and Hodgkin's disease. Data was compared between the two periods using chi-square analysis. More splenectomies were performed for hematologic disorders during Period II than Period I (P < .005). This increase is secondary to a rise in the number of splenectomies performed for cytopenia/anemia during Period II. In contrast, splenectomies for splenomegaly and Hodgkin's disease decreased during Period II (P < .005 and < .05). More Hodgkin's patients were upstaged on the basis of positive laparotomy findings in Period II, compared to Period I (40% versus 10%, P = .01). Surgeons are now performing more splenectomies for cytopenic/anemic diseases, and fewer for splenomegaly and Hodgkin's disease. These results are consistent with recent trends: (1) earlier splenectomy in patients with cytopenia/anemia; (2) earlier medical intervention in infiltrative splenic disorders; and (3) more reliance on radiologic staging in Hodgkin's disease and widespread use of combination chemotherapy, leaving surgical staging for those cases in which treatment would be changed by laparotomy findings.

Extremity amputations in meningococcemia-induced purpura fulminans.

Six children with meningococcemia-induced purpura fulminans were followed. Five of the six required amputations of 14 limb segments. Eight of these amputations required revisions to higher levels.

Apparent response of refractory post-transfusion purpura to splenectomy.

Post transfusion purpura (PTP) is a rare disorder characterized by the abrupt onset of severe thrombocytopenia following transfusion. A patient with PTP and massive bleeding was refractory to corticosteroids, gamma globulin, and plasma exchange but developed an immediate and sustained rise in platelet count following splenectomy. Splenectomy may be a useful therapeutic modality in patients with refractory PTP.

[Expansion of the facial integument in children]. / Expansion du tégument facial chez l'enfant.

In the light of four clinical cases of paediatric facial reconstruction, the authors discuss the advantages and indications of the cutaneous expansion technique which can be combined with other plastic surgery procedures to obtain an abundant skin with ideal texture and sensitivity and minimal scars. Operations for cicatricial alopecia, very extensive forehead naevus, preauricular tumour and nasal mutilation are described.

Purpura fulminans.

The purpose of this article is to call attention to an unusual syndrome that may lead to a major loss of soft tissue, limb, and life. The application of principles learned from trauma and burn care may substantially reduce the morbidity and mortality, which in the past have been associated with purpura fulminans.

Surgical management of life-threatening and disfiguring sequelae of fulminant meningococcemia.

In recent years, because of prompt diagnosis and effective, aggressive resuscitation, the majority of infants and children with fulminant meningococcemia are surviving. From 1974 through 1984, 135 patients with this diagnosis were treated, and 126 of them survived. Although a purpuric skin rash developed in almost all of these patients initially, in eight of them it progressed to multiple confluent areas of cutaneous gangrene, usually associated with extensive necrosis of underlying subcutaneous fat, fascia, skeletal muscle, and even bone. Tissue necrosis seemed to be most extensive in regions of reduced blood flow, such as the extremities, but it almost never followed a pattern of anatomic vascular distribution. A most significant microscopic finding was the presence of multiple fibrin thrombi in vessels, often in close proximity with the foci of tissue necrosis. Five children who ranged in age from 6 months to 12 years required operation. Initially, all surface wounds were treated like full-thickness burns with silver sulfadiazene (Silvadene) dressings. Once the patient's condition had stabilized and the extent of tissue necrosis was apparent, all necrotic tissue was excised and the resulting wounds were temporarily covered with biologic dressings to assure a clean, viable base for skin grafting. Because the resulting quality of life postoperatively in all five surviving patients has been satisfactory, we recommend an aggressive surgical approach in patients with fulminating meningococcemia, despite what may initially appear to be devastating and even lethal complications of this disease.