Giant gluteal lipoma-like liposarcoma: a case report.

BACKGROUND: Liposarcoma is the second most common soft tissue sarcoma in adults with a peak incidence between the 4th and 6th decade of life and slight preponderance to the male gender. It originates from multipotential primitive mesenchymal cells, rather than mature adipose tissue. CASE PRESENTATION: An unusual case of a rapidly growing giant lipoma-like liposarcoma of the left gluteal and perineal areas in a young male was presented. The patient was managed by wide local excision of the lesion and coverage with split thickness skin graft. The key issues surrounding the treatment of lipoma-like liposarcoma and literature review is discussed. CONCLUSION: For such unusual case of this particular rapidly growing tumor, a longer follow-up is needed to evaluate the outcome in these cases.

Paediatric burns in Kuwait: incidence, causes and mortality.

From a prospective study on burn patients admitted at the Burn Center in Kuwait during 1993-2001, 826 paediatric burn cases, in 0-14 years age group, were retrieved to study incidence, causes, and mortality among children. The demographic characteristics, clinical features, and outcome constituted our data file in Statistical Software, SPSS. Overall incidence rate was 17.5/100,000 children aged 0-14 years, being almost twice (34/100,000) among those between 0 and 4 years, constituting 70.8% of all paediatric burns. Scald was the main cause of burn (67%), followed by flame (23%). Mean age (6.4 years) of children with flame burns, was significantly higher (p < 0.001) than those with scalds, or other causes. A positive significant correlation existed between duration of hospital stay and TBSA% (r = 0.56, p < 0.001). Overall mortality rate was 0.23/100,000 children, maximum (0.52/100,000) being in children below 5 years. Among 11 (1.3%) non-survivors, flame burns caused nine fatalities. Multiple logistic regression mooring predicted children aged < 5 years, flame burns and TBSA >or= 70%, (OR = 29.2, p < 0.001), as main contributing factors to fatal outcome among children. Gender and nationality had no influence on incidence or mortality. These findings will hopefully stimulate development of targeted and sustainable interventions for reducing burns occurrence among identified paediatric high-risk groups.

Predicting factors influencing the fatal outcome of burns in Kuwait.

A prospective study was carried out on a total of 2111 burn patients admitted at the Burn Center in Kuwait, during 1993 to 2001, with the purpose of predicting the risk factors influencing the fatal outcome. A total of 111 (5.3%) patients died, giving an annual average of 12 deaths, and a mortality rate of 0.64/100,000 population. The data, from the in-patient records, included the most commonly available demographic features viz. age, gender and nationality, as well as best recorded clinical factors, such as cause of burn, total body surface area (TBSA), duration of hospital stay and outcome, for analysis. Kolmogorov-Smirnov z test showed the median age (30 years) and TBSA (80%) significantly higher (p < 0.001) among those died as compared to 24 years and 10%, respectively in patients survived. The Chi-square test revealed a fatal outcome associated with gender and cause of burn. The multiple logistic regression model predicted patients, aged 60 years and above (OR: 9.9, p < 0.001), female gender (OR: 2.2, p < 0.016), Flame burns (OR: 3.5, p < 0.035), and TBSA > 90% (OR: 23.5, p < 0.001), as the most influencing risk factors for a fatal outcome at this burn center. Patients with these characteristics need to be given special attention during in-patient care.

Only some septicaemic patients develop hypernatremia in the burn intensive care unit: why?

From April 1993 to January 2000, 105 patients in the burn intensive care unit (BICU) that developed septicaemia in the course of their treatment were studied retrospectively to investigate as to why only 36 septicaemic patients (34%) developed hypernatremia (serum sodium >150mmol/l). Septicaemic burn patients who developed hypernatremia were found to have a higher incidence of inhalation injury and a larger burn area (TBSA) signifying greater free water losses in the face of increasing fluid requirements. Patients who developed hypernatremia showed a characteristic pattern of septicaemia: early onset, multiple episodes, polymicrobial, need for multiple antibiotics, longer duration and a higher mortality, indicating a more severe degree of sepsis. The level of incapacitation either from the burn itself, mechanical ventilation or from impaired mental status leading to an inadequate free water intake was more in septicaemic patients who developed hypernatremia. Increased urinary free water losses and solute diuresis from hyperglycemia were significant factors in the development of hypernatremia. Patients who were treated with early wound excisions were less prone to develop hypernatremia when compared to those who did not undergo early wound excision. The close association between the onset of hypernatremia and the onset of septicaemia noted in this study suggests the use of hypernatremia as a marker for septicaemia in burn patients. Hypernatremia in a septicaemic burn patient is multi-factorial and a thorough understanding of the underlying factors will help prevent the onset and progress of hypernatremia.

Radiological manifestation of pulmonary complications in deceased intensive care burn patients.

The chest radiographs of 46 burn patients who died in the burn intensive care unit (BICU) were retrospectively analyzed to study the spectrum of pulmonary complications and their contribution to patient's mortality. There were 25 male and 21 female patients and their mean ages were 34 and 30 years, respectively. Forty-three patients had flame burns, two chemical, and one scald with a mean total burn surface area (TBSA) of 71%. Thirty-six of them had inhalation injury and of these 25 patients developed septicaemia. Out of these 46 patients, 39 had a total of 60 pulmonary complications on various postburn days. The commonest complications were consolidation (28.3%) and adult respiratory distress syndrome (ARDS) (26.7%) mainly due to inhalation injury and/or following septicaemia. The majority of these complications (46.7%) occurred in the late phase (postburn day 5 onwards). Forty-one (89.2%) patients died due to multi-organ failure (MOF) and a good number of them had secondary respiratory failure. The flame burn patients with large TBSA, presence of inhalation injury, and occurrence of septicaemia, are at risk for pulmonary complications that equally affect adult males and females. Pulmonary complications irrespective of the cause significantly contribute to the mortality. This study suggests that serial chest X-rays done in BICU form an important diagnostic tool for pulmonary complications from postburn day 1 onwards, and is useful for subsequent monitoring of the treatment. All burn intensive care units may not be privileged to have a full time radiologist, and intensivist. Therefore, the burn surgeon needs to metamorphose into an intensivist and double as a burn radiologist for early detection and quick treatment if his surgical skills are to be adequately rewarded.

Heterotopic calcification in burn scars and non-healing ulcers.

Heterotopic calcification (HC) is abnormal deposition of calcium salts in tissues other than bone and enamel. The heterotopic calcification in the burn patients is commonly found either in periarticular region or in the muscles, but the occurrence of heterotopic calcification of the burn scar itself in the presence of normal serum calcium, phosphate and alkaline phosphatase is not reported earlier. We present four adult male patients of lower limbs heterotopic calcification in burn scars with unusual presentation of non-healing ulcers with a latent period of 15-20 years. In one of the patient it was Marjolin's ulcer although the lesion was away from the calcified area. Excision of the calcified scars and the release of contracture have cured the non-healing ulcers and this may prevent re-calcification in future.

Septicaemia after burn injury: a comparative study.

Seventy-nine (8.4%) patients during June 1992-May 1996 (Group-1) and 68 (7.2%) patients from June 1996 to May 2000 (Group-2) who developed septicaemia at the burns unit of Al-Babtain Centre for Plastic Surgery and Burns, Kuwait, were retrospectively studied and compared. The mean age of 26 years, male predominance, flame burns as main aetiology and mean burn percentage of >or=40% was observed in both the groups. Both groups revealed extensive flame burn, inhalation injury, intubation and difficult resuscitation as the risk factors. The proportion of satisfactory resuscitation increased significantly (P<0.001) in Group-2. The septicaemia commonly occurred within 2 weeks postburn but the number of episodes during 5 days postburn was less in Group-2. The surface wound was found to be the likely source of entry of the organisms into the blood stream in both the groups. The gram positive organisms were dominant aetiologic factor in both groups but an increase frequency of Acnetobacter was found in Group-2. The proportion of MRSE and Pseudomonas septicaemia was significantly higher (P<0.01) in the Group-1. The rate of survivors, in both the groups was higher among operated patients but it was significantly higher (P<0.001) in the Group-1. A mortality rate 20.6% in Group-2 decreased against Group-1, which can be attributed to better resuscitation, nutritional care, early detection of septicaemia, appropriate antibiotics and early wound excision and skin grafting. MOF was the cause of death of 60.9% in Group-1 and 85.7% in Group-2. There was no role of prophylactic antibiotic in burn patients in the incidence of septicaemia and mortality.

Vomerine hamartoma in a cleft palate child.

A case of a female child born at full term after normal vaginal delivery with bilateral secondary complete cleft palate and vomerine hamartoma mimicking intra-oral midline encephalocoele. Radiologically the tumour was confined to the vomer without intra-cranial extension. The lesion was occupying the oral cavity causing feeding problem. Surgical excision of the tumour at the age of six months and two flaps palatoplasty at the age of twelve months were performed. On histopathology the lesion turned out to be a lipomatous hamartoma of a benign nature. The child was followed for 9 years with no evidence of recurrence and a satisfactory speech. To our knowledge this is the first report of cleft palate with vomerine hamartoma in the English literature.

Unusual clinical presentation of a severely protruding premaxilla in patient with bilateral clefts.

Prominent premaxilla is one of the problems encountered when dealing with bilateral complete cleft lip and palate patients. Secondary alveolar bone grafting with these patients would achieve filling of the osseous defect, supports the alar base, eliminate the oro-nasal fistula and enhance the maxillary instability. This article describes the management of a bilateral cleft lip and palate patient with an extremely protruding premaxilla done in one stage surgery. To our knowledge, this is the first report of such degree of severity in the English literature.

Burn septicaemia in Kuwait: associated demographic and clinical factors.

OBJECTIVE: To study the demographic and clinical factors associated with burn septicaemia patients in Kuwait. MATERIALS AND METHODS: All burn in-patients, who developed septicaemia at the Burns Unit, Al-Babtain Centre for Burns and Plastic Surgery, Kuwait, during a 9-year period (June 1992 to May 2001) were included in the study. The data were recorded for age, sex, nationality, cause and percentage of burns, inhalation injury, resuscitation, number of episodes, septicaemia on post-burn day, the microorganisms responsible in each episode, treatment and outcome for statistical analysis. Using SPSS (PC version 11.0) software, a probability level of p < 0.05 was considered significant. RESULTS: Of the 2,082 patients treated in the Burns Unit, 166 [8%; 99 (60%) males and 67 (40%) females] with a mean age of 26 years (range 1-70) had septicaemia. Significantly higher (p < 0.001) cases were recorded among Kuwaiti children (< or =14 years) and non-Kuwaitis (25-59 years) than other corresponding age groups. The total body surface area burned ranged from 2 to 95% (mean 42%) and the main cause of burn was flame (77.1%). Inhalation injury was diagnosed in 39 (23.5%) patients. A total of 253 septicaemic episodes occurred in all patients. The majority, 123 (74.1%), had a single episode and the remaining 43 (25.6%) had multiple (2-10) episodes. One hundred and fifty-five (61.3%) episodes were due to gram-positive organisms, mainly methicillin-resistant Staphylococcus aureus, and 32 (12.7%) were polymicrobial. One hundred and twenty-four (74.7%) patients had wound excision and skin grafting procedures and their survival was significantly higher (OR = 4.3; 95% CI: 1.98-9.31) than non-surgically treated patients. Thirty-nine (23.5%) patients died mainly due to multi-organ failure. CONCLUSION: The findings indicate that the patients with extensive flame burns were prone to developing septicaemia due mainly to gram-positive bacteria. The surgical excision of eschar and wound covering improved the outcome of the patients while prophylactic antibiotic treatment had no role in the incidence and outcome of the burn patients.

Infantile systemic hyalinosis.

Infantile systemic hyalinosis (ISH) is a rare familial autosomal recessive disease of unknown etiology. The clinical features are evident either at birth or within 6 months of life. The presentation is painful progressive joint contractures, thickened skin with hyperpigmentation over prominences, small pearly facial papules, gingival hypertrophy, fleshy nodules in the perianal region, diarrhea, increased susceptibility to bone fractures, infections, and failure to thrive. This is a progressive disorder that may lead to death within first 2 years of life, mostly due to recurrent chest infection and diarrhea. Two patients with ISH, one aged 14 years and another aged 10 years, with all the clinical features, though crippled but surviving, were seen at our center. Debulking of hypertrophic gingiva and excision of some symptomatic skin masses in these patients are indicated for comfort and smooth nursing care of the patients and to allow better rehabilitation.

Versatility of the distally based superficial sural flap for reconstruction of lower leg and foot in children.

Twenty children are presented after undergoing a distally based superficial sural flap for coverage of defects at the lower leg and foot. The age of the patients was between 1 and 12 years. Fifteen patients had trauma to the lower leg, with eight of them having associated injuries. Three had postburn contracture and two had pressure sore. In 14 cases, the flap was used as a fasciocutaneous flap, whereas in six cases it was used as a fascial flap covered with a skin graft. The flaps were used to cover the defects from the dorsum of the foot distally up to the mid third of tibia proximally. The mean follow-up was for a period of 2 years. Even though free tissue transfer is reliable and safe for the reconstruction of major leg injuries in children, the distally based superficial sural flap has the advantage of being easy to perform, with short operating time, minimal donor side morbidity, and preservation of major arteries of the leg.