Paul K. Minifee

Effect of propranolol administration on hemodynamic and metabolic responses of burned pediatric patients.

Hypermetabolism, increased heart rate, and lipolysis are responses to high catecholamine levels associated with burn injury. This study tests the hypothesis that adrcncrgic beta blockade in burns could reduce myocardial work, lipolysis, and negative nitrogen balance without adversely affecting cardiac or metabolic function. Eighteen patients with burns of 70 ± 3% total burn surface area (TBSA) (Mean ± SEM), were studied after a 5-day infusion of 2 nig/Kg of intravenous (I.V.) propranolol infusion every 24 hours without their cardiac output or resting energy expenditure being adversely reduced. Heart rate, left ventricular work, and rate pressure product were significantly reduced by 20, 22, and 36%, respectively (P < 0.05). Plasma glucose, free fatty acids, triglycerides, and insulin levels remained unchanged. The rate of urea production, however, was significantly increased by 54 ± 12% in fasted patients, and to a much lesser 12 ± 2% in fed patients. The marked decrease in myocardial work afforded by propranolol administration may be of clinical benefit in the treatment of large burns. Variations in drug dosage and feeding regimens will, however, need to be perfected to limit catabolic effects.

Improved myocardial oxygen utilization following propranolol infusion in adolescents with postburn hypermetabolism.

The purpose of this study was to determine if propranolol (0.5 mg/kg and 1 mg/kg), administered intravenously (IV) at the height of the postburn hypermetabolic response, would decrease myocardial oxygen requirements, without adversely affecting overall oxygen delivery or total body oxygen consumption. To test this hypothesis, six nonseptic patients age 17 +/- 3 years with burns over 82% +/- 11% total body surface area were given propranolol with continuous hemodynamic monitoring. Propranolol was administered to these patients 20 +/- 15 days postburn. Two clinically derived indices of myocardial oxygen consumption, pressure-work index (PWI) and rate-pressure product (RPP), were used to estimate the energy expenditure of the working heart. Both PWI and RPP were significantly decreased from baseline after 0.5 mg/kg propranolol, 31% for PWI (P less than .001) and 30% for RPP (P less than .01). Similarly, a decrease from baseline was seen after 1.0 mg/kg propranolol, 32% for PWI (P less than .001) and 35% for RPP (P less than .01). Cardiac index (L/min/m2) demonstrated no significant change [7.4 +/- 1.1 (prepropranolol), 6.5 +/- 1.3 (after 0.5 mg/kg propranolol), and 6.8 +/- 1.0 (after 1.0 mg/kg propranolol)] and exceeded the upper limits of normal (hyperdynamic state) throughout the study. Oxygen delivery index (962 +/- 209 mL/min/m2) and oxygen consumption indices [(254 +/- 78 mL/min/m2 by Fick method and 236 +/- 78 mL/min/m2 by inspired and expired gases)] were elevated at baseline and unaffected by propranolol. The decrease in PWI and RPP was achieved mainly by propranolols effect to lower both heart rate and BP.(ABSTRACT TRUNCATED AT 250 WORDS)

Right lower quadrant pain in children caused by omental infarction

BACKGROUND Omental infarction is a rare cause of abdominal pain, with fewer than 300 cases reported in the literature. Only 15% of reported cases occur in the pediatric population. We present our experience with 18 children diagnosed with omental infarction admitted to Texas Childrens Hospital over a 15-year period. STUDY DESIGN A retrospective review of clinical records, diagnostic images, and pathologic findings was carried out for all children diagnosed with isolated omental infarction from 1986 to 2000 in order to analyze presenting signs and symptoms, preoperative imaging, surgical management, and postoperative outcomes. RESULTS Eighteen children were treated for isolated omental infarction. There were 12 boys and 6 girls with an average age of 7.5 years (range 2 to 13). All patients presented with acute onset of right lower quadrant pain. Only 5 of 18 (24%) had associated gastrointestinal symptoms. The average temperature at presentation was 99.4 degrees F (+/- 0.78). The average white blood cell count was 11.4 (+/- 4.4). Fourteen patients had ultrasonographys performed preoperatively: 6 of 14 incorrectly diagnosed appendicitis, 4 of 14 were nondiagnostic and 4 of 14 correctly diagnosed omental infarction. Two of 18 patients underwent computed tomography scans, which were diagnostic for omental infarction. Resection of the infarcted omentum was performed in all patients and appendectomy in 16 of 18. Thirteen patients underwent an open procedure, 5 were performed laparoscopically. There were no postoperative complications. All patients had resolution of pain postoperatively and were discharged an average of 3.0 (+/- 0.9) days after admission. CONCLUSIONS Omental infarction is an uncommon cause of right lower quadrant pain in children and is often diagnosed as appendicitis preoperatively. Ultrasonography and computed tomography can be diagnostic. Surgical resection of the infarcted omentum results in immediate resolution of pain with no morbidity.

Decreasing blood donor exposure in neonates on extracorporeal membrane oxygenation

Extracorporeal membrane oxygenation (ECMO) has been successful treatment (80% survival) in over 2,000 neonates with severe respiratory failure (80% predicted mortality without ECMO). Neonates on ECMO require frequent blood product replacement, which increases donor exposure (DE) and the risk of transfusion related complications. Successful, widespread usage of ECMO in neonatal respiratory failure is placing increased numbers of surviving infants at risk for acute and long-term transfusion related problems. We assessed DE rates in 21 consecutive neonatal ECMO survivors. In the first 12 patients packed red blood cell (PRBC) transfusions were administered as 10 mL/kg body weight for hematocrit less than 45%. PRBC exchange transfusions were used in patients with hematocrit less than 45% and hypervolemia. Fresh frozen plasma (FFP) and cryoprecipitate (CRYO) infusions were used empirically for evidence of hemorrhage. DE rates (donors per ECMO day, mean +/- SD) were: PRBC (2.8 +/- 0.6), FFP/CRYO (0.5 +/- 0.7), and platelet (2.0 +/- 1.0), with a total donor exposure rate of 5.3 +/- 2.0 donors per ECMO day. Mean duration of ECMO was 4.6 +/- 2.0 days and total DE per infant was 22.8 +/- 9.5 donors per ECMO run. In a protocol (n = 9) to minimize DE risks, exchange transfusions were eliminated and PRBC transfusion volumes were increased to 15 mL/kg. Empiric use of FFP and CRYO was discontinued. The blood bank divided standard units of PRBCs into four aliquots and dispensed each aliquot sequentially before dispensing blood from another unit.(ABSTRACT TRUNCATED AT 250 WORDS)

Intraabdominal desmoplastic small round cell tumor presenting as a gastric mural mass with hepatic metastases

Purpose: An atypical case of childhood intraabdominal desmoplastic small round cell tumor (DSRCT) is presented. Patient and Methods: An 11-year-old boy presented with progressive nausea and vomiting, abdominal pain, hepatomegaly, and an epigastric mass. Computed tomographic scanning as well as findings at gastroscopy and laparotomy revealed a large gastric mural tumor accompanied by multiple large intrahepatic masses. Histopathologic examination of biopsy samples revealed evidence of a polyphenotypic neoplasm diagnostic of DSRCT. Results: Unlike most reported cases of DSRCT, no evidence of peritoneal involvement or ascites was detected. Despite an excellent initial response to multiagent chemotherapy, the patient eventualy died of progressive tumor. Conclusions: Though the radiographic and surgical findings in DSRCT usually suggest a mesenteric, peritoneal, or retroperi-toneal site of tumor origin, this case demonstrates that intraabdominal DSRCT can present with a primary visceral lesion without evidence of peritoneal or mesenteric involvement or ascites. Combination chemotherapy using vincristine, doxorubicin, cyclophosphamide, cisplatin, and 5-fluorouracil may be of some benefit to patients with this rare tumor.

Clear cell sarcoma of the kidney : An unusual presentation and review of the literature

PURPOSE To describe a child with clear cell sarcoma of the kidney (CCSK) with an unusual presentation, including a primary tumor of the left kidney with metastases to the right kidney and soft tissues of the lower extremities, and to review the literature. PATIENT AND METHODS An 8-month-old infant presented with hypertension, an abdominal mass, and soft tissue masses in the left thigh and right foot. Imaging studies revealed a large left-sided renal tumor, left paravertebral soft tissue masses, and left thigh mass. At laparotomy, a lesion was noted in the lower pole of the contralateral kidney. CCSK with metastases to the contralateral kidney and to the soft tissues of left thigh, right foot, and left paravertebral region was diagnosed on histopathologic examination. RESULTS Multimodal oncologic treatment included surgery, chemotherapy, and radiotherapy. Three months after completion of therapy, a soft tissue lesion in the left arm and, later, soft tissue lesions involving multiple parts of the body developed. The patient died 18 months after diagnosis without clinical or radiographic evidence of bone involvement. CONCLUSIONS In a review of the literature, CCSK is most commonly associated with bone and lung metastases. Soft tissue involvement is uncommon. Metastasis to the contralateral kidney at initial diagnosis has not previously been reported. This case represents an unusual metastatic pattern of CCSK.

Subcutaneous palisading granulomas (benign rheumatoid nodules) in children

Subcutaneous palisading granulomas (SPG), also known as benign rheumatoid nodules, occur in otherwise healthy children. Although these lesions have the same histology as rheumatoid nodules in adults, subcutaneous granuloma annulare, and diabetic lipoidicum, they appear to represent a distinct clinicopathologic entity. We reviewed our experience with 17 cases of SPG. All patients presented with one or more painless, nonmobile, subcutaneous nodules. Lower extremity involvement was most common, but lesions presented in the upper extremities and scalp as well. There were 14 girls and three boys ranging in age from 10 months to 15 2/12 years (mean, 4 5/12 years) at the time of diagnosis. Past medical histories were negative. Laboratory and radiologic studies were normal except for one child with an elevated erythrocyte sedimentation rate (ESR). The follow-up period ranged from 6 months to 14 10/12 years (mean, 4 11/12 years). Subsequent new lesions developed in nearly two thirds of the patients. In the one child with an initially elevated ESR, rheumatoid arthritis developed. All others were alive and well except for one who died in a motor vehicle accident without evidence of further disease. Preoperative diagnosis was correct in only three patients. Excisional biopsy was performed in all cases. However, one child had received prior radiation therapy, one received topical steroid therapy, and three patients had subsequent lesions excised. All subsequent lesions not surgically excised underwent spontaneous involution.(ABSTRACT TRUNCATED AT 250 WORDS)

Sonographic diagnosis of multiple small-bowel intussusceptions in Peutz-Jeghers syndrome: a case report

Abstract. Peutz-Jeghers syndrome (PJS) is a rare, though well-described, hereditary polyposis syndrome associated with mucocutaneous pigmentation that typically presents in the second decade of life with complications related to intestinal polyps. We present two cases of teenaged girls presenting with small-bowel intussusceptions within a 3-month period. Sonographic examinations readily revealed small-bowel intussusceptions with secondary small-bowel obstruction. In both the symptoms were less severe than one would expect given the underlying pathology found at subsequent surgery. Sonographic imaging with pathologic correlation is provided.

Immediate positive pressure ventilation with positive end-expiratory pressure (PEEP) improves survival in ovine smoke inhalation injury

BACKGROUND The purpose of this study was to compare the effects of immediate initiation of positive pressure ventilation (PPV) with positive end-expiratory pressure (PEEP) versus the initiation of PPV with PEEP only after hypoxemia ensued following severe smoke inhalation injury. METHODS We prospectively evaluated chronically instrumented adult sheep treated with immediate versus delayed PPV with PEEP and compared oxygen requirements, hemodynamics, pleural fluid formation, postinjury survival, and tracheobronchial pathologic processes among groups. The immediate group (group I; smoke, n = 6; sham, n = 2) underwent tracheostomy and bilateral chest tube placement before they received inhalation injury. They were then immediately placed on PPV with PEEP (12 cm H2O). The animals in the delayed group (group D) (n = 6) were placed on PPV with PEEP when arterial hypoxemia (PaO2 < 80 mm Hg [11.2 kPa] on 0.4 FIO2) or respiratory distress developed. RESULTS Groups were matched for smoke exposure and peak carboxyhemoglobin. Both groups developed a characteristic decrease in PaO2/FIO2 ratio. Initiation of PPV + PEEP improved PaO2 in the delayed group (69 +/- 7 to 126 +/- 21 mm Hg [9.2 +/- 0.9 to 16.7 +/- 2.8 kPa]). Pleural fluid output was greater in the immediate group compared with the delayed group (1559 +/- 415 vs. 426 +/- 236 mL). At 96 hours after injury five of six animals in the delayed group had died. In contrast, six of six animals in the immediate smoke group survived 96 hours (p < 0.05 versus delayed group). The immediate group had fewer and less extensive tracheobronchial casts at necropsy. CONCLUSIONS Immediate PPV + PEEP did not prevent the development of hypoxia and was associated with increased pleural fluid formation. Death within 96 hours in the delayed group was the result of respiratory failure aggravated by bronchial cast formation despite vigorous pulmonary toilet. Early positive pressure ventilation with PEEP, preferably initiated immediately after the inhalation insult, significantly increases short-term survival and is associated with decreased tracheobronchial cast formation in this ovine model of severe smoke inhalation injury.

Traumatic hepatic artery laceration managed by transarterial embolization in a pediatric patient

While blunt abdominal trauma with associated liver injury is a common finding in pediatric trauma patients, hepatic artery transection with subsequent treatment by transarterial embolization has rarely been reported. We present a case of a child who suffered from a hepatic artery injury which was successfully managed by supraselective transarterial microcoil embolization, discuss management strategies in these patients, and provide a review of currently available literature.