Primary osteoma cutis.
This case report was presented because there are few published cases of a solitary lesion of primary osteoma cutis occurring in the foot. It is an unusual cause of heel pain.
Biomedical subjects
Publications and source records attributed to M D Klein.
This case report was presented because there are few published cases of a solitary lesion of primary osteoma cutis occurring in the foot. It is an unusual cause of heel pain.
BACKGROUND: Congenital diaphragmatic hernia (CDH) continues to have a high mortality rate (24 to 57 percent) despite changing management schemes, which include extracorporeal membrane oxygenation (ECMO) for treatment of associated persistent pulmonary hypertension of the newborn. STUDY DESIGN: The medical records of 123 acutely symptomatic newborns with CDH treated from 1972 to 1994 were retrospectively reviewed. Patients were divided into three groups to compare historical treatment modalities: group 1, no ECMO available; group 2, postoperative ECMO if necessary; and group 3, delayed repair with preoperative ECMO if necessary. The blood gas values, alveolar-arterial oxygen gradient (A-aDO2), mean airway pressure (MAP), and oxygenation (OI) and ventilation indices (VI) prior to treatment were compared between survivors and nonsurvivors. Chi-square and Student's t tests were used to determine statistical significance. RESULTS: The overall survival rate was 41 percent: 27 percent in group 1, 45 percent in group 2, and 39 percent in groups 3. If those who were not candidates for ECMO were excluded from analysis, the survival rate improved to 35 percent in group 1, 51 percent in group 2, and 50 percent in group 3. No published prognostic scoring system, such as arterial blood gas values, A-aDO2 gradient, MAP, OI, or VI consistently distinguished survivors from nonsurvivors. Extracorporeal membrane oxygenation decreased the mortality rate of patients having large defects. CONCLUSIONS: Prognostic scoring systems do not predict which patients with CDH should be treated. Extracorporeal membrane oxygenation has improved survival in newborns with CDH who present in early respiratory distress. There is no advantage or disadvantage to using ECMO prior to repair of CDH.
Extracorporeal life support (ECLS) is a recognized treatment for neonatal respiratory distress unresponsive to other forms of therapy. Variations of this technique are being developed in an effort to extend its applicability and safety. Extracorporeal CO2 removal (ECCO2R) is one such modification that requires blood flows of 20% to 50% of cardiac output and therefore lends itself to percutaneous venous cannulation. The authors evaluated ECCO2R in conjunction with low-frequency ventilation, using a lung lavage-induced model of respiratory failure in rabbits. Six rabbits were lavaged an average of 9 times with 15 mL/kg Plasma-Lyte A at 37 degrees C via an endotracheal tube. Incremental ventilatory changes were made during lavage, to an FIO2 of 1.0, rate of 80, peak inspiratory pressure (PIP) of 37 cm H2O, and positive end-expiratory pressure (PEEP) of 4 cm H2O. Arterial blood gas values of PaO2 < 40 mm Hg and PaCO2 > 60 mm Hg resulted, meeting our criteria for respiratory failure. The rabbits were placed on veno-venous ECCO2R using a 0.8-m2 hollow fiber oxygenator and a commercially available double-lumen dialysis catheter. Blood flows of 10 to 20 mL/kg/min were used to manage CO2 removal. A low-frequency ventilation technique was employed using an FIO2 of 1.0 and a rate of 5 breaths per minute. PEEP was increased incrementally to maintain the PaO2 above 80 mm Hg. After initiation of ECCO2R, the arterial PaO2 increased to 165 +/- 109 mm Hg, with PEEP above 15 cm H2O, and PaCO2 decreased to 37 +/- 5 mm Hg, with a bypass flow rate of 15 mL/kg/min.(ABSTRACT TRUNCATED AT 250 WORDS)
Extracorporeal membrane oxygenation has now evolved into standard therapy for patients unresponsive to conventional ventilatory and pharmacological support. This article presents a clinical review of extracorporeal life support and its application to neonatal and pediatric patients as well as children requiring circulatory support after open heart surgery.
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The growing success of liver transplantation and the shortage of donor livers has turned attention to the possibility of utilizing hepatocytes within artificial liver support systems to allow time for donor livers to become available and to improve the condition of patients with hepatic failure. This study evaluated encapsulated hepatocytes, a technology which might allow the possibility of using xenogenic or human hepatoma cells. Rabbit hepatocytes were encapsulated using the ionic polysaccharides carboxymethylcellulose, chondroitin sulfate A, chitosan, and polygalacturonic acid. Encapsulated cells were maintained in perfusion culture for at least 6 days in heparinized, normal human plasma or in a defined culture medium. Parallel cultures of plated hepatocytes were also conducted. The metabolic capability of the cells was evaluated by following the rates of urea, albumin, and transferrin synthesis and the transformation rate of the drug antipyrine. Protein synthesis and ureogenesis in plasma were depressed from the levels expressed in defined culture medium. Drug detoxification as measured by antipyrine metabolism appeared to be enhanced in plasma. We conclude that encapsulated rabbit hepatocytes retain significant levels of function for at least 6 days of perfusion with human plasma, suggesting the feasibility of this technology as a potential method of short-term liver support.
Experience with 250 patients treated for Hirschsprung's disease over the past three decades shows several interesting features. Only 58% had rectosigmoid disease. Twenty-six percent had long-segment disease, and 12% had total colon aganglionosis. This distribution did not change significantly over the decades. The male:female ratio was 3.3 in all groups, but dropped to 2.2 with total colon disease. The mean age at diagnosis decreased from 18.8 months during the 1960s to 2.6 months in the 1980s. The mean age at which the pull-through procedure was performed also dropped from 34 months to 15 months. Twenty-three patients died: 17 in the 1960s and three in each of the succeeding decades. The Swenson procedure was predominant during the 1960s, but was only 2% of the pull-throughs in the 1980s. The endorectal pull-through increased from 22% in the 1960s to 71% in the 1980s. In the 1970s and 1980s the Duhamel accounted for 26% of the pull-throughs. With a mean follow-up of 51 months, the mean number of episodes of post pull-through enterocolitis per patient was 0.53. This increased from 0.44 in the 1960s, to 0.52 in the 1970s, to 0.67 in the 1980s. The incidence of post-pull-through enterocolitis increased somewhat with the length of the bowel involved, and was greatest with the Duhamel (0.95 episodes per patient), less with the Swenson (0.60), and least with the endorectal pull-through (0.38). In contrast, the incidence of complications other than enterocolitis was greatest with the Swenson (20%), less with the endorectal pull-through (17%), and least with the Duhamel (13%).(ABSTRACT TRUNCATED AT 250 WORDS)
This study investigated the relationship between reading comprehension and oral storytelling abilities. Thirty-one Latino junior high school students with learning handicaps were selected as subjects based on learning handicapped designation, home language, and language proficiency status. Reading comprehension was measured by the Reading Comprehension subtest of the Peabody Individual Achievement Test. Storytelling was measured by (a) the Oral Production subtest of the Language Assessment Scales using the standard scoring protocol and (b) a story structure analysis. A comparison of the standard scoring protocol and reading comprehension revealed no relationship, while the comparison of the story structure analysis and reading comprehension revealed a significant correlation. The implications of these results for language assessment of bilingual students are discussed.
BACKGROUND: Outcome for most abdominal wall defects is related to the presence or absence of additional anomalies or prematurity. In gastroschisis, outcome is almost as closely related to the severity of the inflammatory "peel" on bowel that is thought to result from direct contact with amniotic fluid. Improving eviscerated bowel quality would be expected to reduce morbidity in these patients. METHODS: From 1986 to 1991, 32 patients with the antenatal diagnosis of gastroschisis were treated. All were delivered by cesarean section; 13 surgical repairs were made immediately in the delivery room. Surgical repairs in 19 patients were made at less than 6 hours of age after transfer from the delivering hospital to the pediatric surgery center. RESULTS: Thirty percent of infants who underwent surgical repair in delivery room and 32% of infants who underwent urgent surgical repair were either premature or had significant associated anomalies. Seventy-three percent of delivery room repair group had fascial repairs compared with 37% in the transferred group. When infants more than 34-weeks' gestation without associated anomalies are compared with transferred infants, delivery room repair group underwent more frequent fascial repair (8 of 9 vs 5 of 13, p < 0.03), were extubated sooner (2.9 vs 7.4 days, p < 0.04), tolerated enteral feedings earlier (8.1 vs 22.2 days, p < 0.009), and required fewer hospital days (13.6 vs 31.3 days, p < 0.01). Eviscerated bowel of infants who underwent immediate surgical repair lacked the characteristic matted, edematous, and fibrinous coated appearance seen in transferred patients. CONCLUSIONS: Immediate delivery room repair of gastroschisis results in increased fascial repairs and earlier extubation, feeding, and hospital discharge. These benefits appear to be due to the minimal reactive peel on eviscerated bowel at birth.
Early reports of improved survival in newborns with congenital diaphragmatic hernias (CDH) utilizing extracorporeal membrane oxygenation (ECMO) and/or a delayed repair (DR) approach have been tempered by recent failures to document such an improvement. We have used ECMO to salvage emergently repaired patients with CDH since January 1984. From January 1990 to January 1991, we treated 16 CDH patients with mechanical ventilation and other supportive techniques until persistent pulmonary hypertension of the newborn resolved. We compared this DR group to 19 patients emergently repaired from February 1987 to December 1989. Of the 19 emergently repaired patients, 16 had a best post ductal (BPD) PO2 > 50 mm Hg. Eight patients survived (42 per cent of all and 50 per cent of those with a BPD PO2 > 50). Thirteen required ECMO and six of these survived. Five of six ECMO survivors had significant ECMO complications, and ECMO was "inappropriately" performed on three of six nonsurvivors. Of the 16 DR patients, nine achieved a BPD PO2 > 50 mm Hg and seven survived (44 per cent of all and 78 per cent of those with a BPD PO2 > 50). Seven required ECMO and four of these survived. All survivors in both groups had a BPD PO2 > 90 mm Hg. Delayed repair did not improve survival statistics for CDH with early respiratory distress at our hospital but it has allowed stratification of potential survivors, fewer inappropriate ECMO cannulations, and many fewer ECMO complications.
Agenesis of the gallbladder not associated with biliary atresia is quite rare. We wish to report two patients with gallbladder agenesis in association with duodenal atresia without biliary atresia. Liver biopsy in one patient was notable for an absence of both inflammation and ductal pathology. The unremarkable postoperative course included normal direct bilirubins and a HIDA scan that demonstrated very good bile excretion into the duodenum. Both children are doing quite well in follow-up.
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Atrial fibrillation has been found to be a significant risk factor for stroke independent of the contribution this common arrhythmia makes via its previously established stroke risk factors. Results of four recent trials in patients with nonrheumatic atrial fibrillation show that warfarin anticoagulant therapy can substantially reduce the risk of stroke without major bleeding complications.
To evaluate the feasibility of long-term extracorporeal membrane oxygenation (ECMO) without heparin, we placed six lambs on standard venoarterial ECMO for 71 to 96 hours. Group 1 (3 animals) was given doses of heparin to maintain activated clotting times (ACT) greater than 400 seconds. No form of anticoagulant was used for the three animals in group 2. Blood flow was maintained at 60 mL/kg/min. No histological evidence of thrombosis was noted at necropsy. ACT, prothrombin time, and partial thromboplastin time were higher in group 1, and much lower, although still above normal in group 2. Fibrinogen was significantly lower in group 2 (75 +/- 35 v 219 +/- 64 mg/dL group 1), and, although the platelet count was lower in group 2 (142 +/- 76 x 10(3)/mm3 v 225 +/- 167 x 10(3)/mm3), it was clinically acceptable. These results encouraged us to discontinue heparin when faced with severe hemorrhage in four patients on ECMO, rather than withdraw support at a time when there was little chance of survival. Heparin was discontinued for 10.5 +/- 6 hours. The mean ACT was reduced from 220 +/- 23 seconds to 144 +/- 22 seconds. One patient, who required repair of gastric necrosis while on ECMO following repair of a congenital diaphragmatic hernia, survived and had a decrease in blood loss from 2 to 0 mL/kg/h after the heparin was discontinued. One of the three patients who died had an autopsy with no evidence of thrombosis. We conclude that it may be reasonable to discontinue heparin in the face of life-threatening hemorrhage while on ECMO.
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We examined the clinical literature on congenital abdominal wall defects to confirm our impression that gastroschisis had become more common than omphalocele. We then examined the teratology literature and noted that congenital abdominal wall defects were frequently induced by teratogens. This lead us to review the antenatal history of 19 infants with gastroschisis and 54 control infants born with a congenital anomaly unrelated to gastroschisis. When compared to controls, mothers of infants with gastroschisis were more likely to have used aspirin during pregnancy and to have been taking oral contraceptives at the time of conception. Additionally, an increased incidence of illegal drug use (particularly cocaine) was noted among the study mothers. We conclude that gastroschisis is becoming the more common congenital abdominal wall defect, and that it could be related to exposure to an environmental teratogen.
A 77-year-old man with a history of renal cell carcinoma presented with orthostatic syncope. Investigation revealed metastatic carcinoma in the left lung extending through the pulmonary veins into the left atrium obstructing the mitral orifice. On review of the literature, this case appears to be the first to describe metastatic carcinoma to the left atrium presenting as a primary cause of syncope.
We have treated 39 infants and children with congenital heart disease with extracorporeal membrane oxygenation during the past 5 years. Thirty-six were treated for low cardiac output or pulmonary vasoreactive crisis after repair of congenital heart defects. Twenty-two (61%) survived. Most patients were cannulated from the neck via the right internal jugular vein and the right common carotid artery. Six patients were cannulated from the chest, including three who had separate drainage of the left side of the heart with a left atrial cannula. Two of these patients survived and were the only survivors of the nine patients cannulated in the operating room because they could not be weaned from cardiopulmonary bypass after open cardiac operations. We also reviewed 312 patients (the predictor study series) having open cardiac operations before the availability of extracorporeal membrane oxygenation; 27 of these patients died. Data were collected at 1 and 8 hours postoperatively to determine if any parameters might predict early mortality. With these parameters used as criteria, patients who went on extracorporeal membrane oxygenation were as sick as those who died before extracorporeal membrane oxygenation was available. The most common complication was bleeding related to heparinization. The mean transfusion requirement in survivors was 1.50 +/- 1.13 ml/kg/hr, 5.63 +/- 7.0 ml/kg/hr in the nonsurvivors, and 7.46 +/- 8.29 ml/kg/hr in those cannulated in the operating room because they could not be weaned from bypass. Four children had intracranial hemorrhage, and two of them died. There was one late death. Nine of the 22 survivors are entirely normal. All survivors who do not have Down's syndrome are considered to have normal central nervous system function. We conclude that extracorporeal membrane oxygenation can improve survival in patients with both pulmonary artery hypertension and low cardiac output after operations for congenital heart disease.