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Biomedical subjects

J A Haller

Publications and source records attributed to J A Haller.

At least 19 recordsLinked to original sources
PubMed1992

Diagnosis and management of congenital vascular rings: a 22-year experience.

Between 1968 and 1990, we operatively treated 39 patients (19 boys, 20 girls) with congenital aortic arch anomalies. Median age was 7 months (range, 1.5 months to 23 years). Thirty-seven patients (95%) had respiratory symptoms. Barium swallow was diagnostic in 95%. Right arch with aberrant left subclavian artery and double aortic arch were the most common types (11 each). Treatment of an aortic diverticulum was documented in 19 patients; the aortic diverticulum was excised (9), managed by aortopexy (7), or left in situ (3). Postoperative recovery was rapid, with a median intensive care unit stay of 2 days, time to oral feeding of 1 day, and postoperative time to discharge of 7 days. Two deaths occurred: 1 infant had undergone emergent operation for control of hemorrhage from an aortotracheal fistula due to tracheostomy tube erosion, and the other had multiple associated congenital heart defects. Postoperative complications included bleeding (1), pneumonia (5), and chylothorax (4). One boy had persistent severe symptoms due to an untreated aortic diverticulum and underwent subsequent excision of the aortic diverticulum with complete relief of symptoms. Median length of follow-up was 12.5 months, with at least 97% of survivors completely or nearly completely free of symptoms from the vascular ring. These results suggest that early repair of congenital aortic vascular rings, including fixating or excising an associated serious aortic diverticulum, is safe and effective and allows for normal tracheal growth.

Adolescent↗
PubMed1992

Hospitalizations for traumatic injuries among children in Maryland: trends in incidence and severity: 1979 through 1988.

Trends in incidence and severity of hospitalized injury among children aged 0 through 13 years in the state of Maryland from 1979 through 1988 (n = 35,746) were examined using routinely reported hospital discharge data. Hospital discharge rates declined over the study period from 509 per 100,000 population in 1979 to 320 in 1988. There was a decline in incidence trends for both races. However, the decrease in the nonwhite population was smaller than in whites. Analysis of incidence rates for specific Injury Severity Score groups revealed a declining trend in all Injury Severity Score groups, although the mildest group (Injury Severity Score 1 through 4) had the most notable decline of 44% compared with an average decline of 20% in the other severity groups. These data suggest a change in admission practices of mildly injured children as a major cause for the observed overall decline in hospitalization rates. The smaller decrease in the hospitalization rates of non-white children compared with white children requires further study to determine the cause.

Adolescent↗
PubMed1991

Submandibular cystic hygroma resembling a plunging ranula in a neonate. Review and report of a case.

Cystic hygromas are large lymphangiomas that are most often found in the posterior triangle of the neck and the axilla in children. They are most frequently found before age 2 and may be massive. After upper respiratory infection, they may become infected and enlarged, causing dysphagia and toxemia. The diagnosis can usually be made by history and physical examination and confirmed by biopsy. Treatment is by surgical excision of small lesions and staged debulking excisions in more severe cases. A patient with a cystic hygroma having many clinical characteristics of a plunging ranula is presented. The cyst fluid was aspirated and analyzed for its amylase, sodium, potassium, chloride, urea nitrogen, glucose, and total protein content. The characteristics of the fluid were also compared with those of lymph and saliva. This report demonstrates the difficulty in determining the diagnosis of a tumor that has the clinical features of a cystic hygroma, as well as a plunging ranula. The necessity of a proper presurgical diagnosis is essential since the form of therapy for each is different and conflicting. A method that distinguishes between the cervical cystic hygroma and a plunging ranula by means of aspirated fluid is discussed.

Cleft Lip↗
PubMed1990

Characteristics of pediatric firearm fatalities.

An increase in the awareness of the problem of gunshot fatalities in children has occurred nationwide over the last year. Unintentional firearm deaths are more common among children and young adults. These deaths may be addressed by preventive measures beyond those available for pediatric firearm suicide and homicide. This study focuses on the incidence of fatal gunshot injuries in children under 16 years of age during the years 1979 through 1987 in our state. Over the past 9 years 132 children (0 to 16 years) were shot and killed as a result of firearm injuries (M:F ratio, 3:1). No difference in absolute number in racial distribution existed. Deaths were classified as homicide, 61 (46%); accidental, 33 (25%); suicide, 29 (22%); undetermined, 7 (5%); and other, 2 (1.5%). Over 70% of these fatal injuries occurred in the home environment. Types of weapons involved included handguns (48%), shotguns (22%), rifles (17%), unspecified weapon (12%), and air rifle (1%). In a large number of cases, guns were found in the home unsecured. The perpetrator was known by the victim in 64 instances, while unknown in 27. Thirty-nine self-inflicted wounds and two shotgun blasts in the line of fire accounted for the remaining deaths. The most common anatomical injury and ultimate cause of death was cranial central nervous system (CNS) (62%), followed by chest/mediastinum (20%), abdomen (10%), and other (8%). A child who has sustained a firearm injury is more likely to know the perpetrator, be killed in the home by a readily available unsecured firearm, and die from severe head injury.

Accidents, Traffic↗
PubMed1990

Choroidal neovascularization after laser photocoagulation for diabetic macular edema.

Choroidal neovascular membranes (CNVMs) developed in eight patients after photocoagulation for clinically significant diabetic macular edema (DME). The CNVMs developed in areas where Bruch's membrane was ruptured and were diagnosed 2 weeks to 5 months after treatment. Only six patients had symptoms. The CNVMs were treated in four patients; final visual acuity was poor in all eight patients. This serious complication that follows laser treatment for DME may be related to the use of repeated small-size, short-duration laser or intense laser burns, or both.

Aged↗
PubMed1990

Vulvar congenital dysplastic angiopathy.

Congenital dysplastic angiopathy is a syndrome consisting of vascular angiomata, congenital varicosities, and trophic changes of the soft tissue and the skeleton. Frequently referred to as Klippel-Trenaunay or Klippel-Trenaunay-Weber syndrome, it rarely affects the female genitalia. An 18-year-old woman underwent evaluation and treatment for Klippel-Trenaunay syndrome with vulvar involvement. Preoperative evaluation included consultation with pediatric surgeons, gynecologic surgeons, and an interventional radiologist as well as individual and family psychological counseling. Attention to intraoperative detail resulted in minimal operative blood loss and preservation of normal anatomy. A postoperative hematoma was treated aggressively with surgical evacuation and drainage. Six-month follow-up revealed functionally and cosmetically normal vulvar anatomy.

Adolescent↗
PubMed1989

A model: systems management of life threatening injuries in children for the state of Maryland, USA.

In the past two years several guidelines for suggested components of emergency medical systems for children have been suggested, and for the first time, specific standards of pediatric trauma care have also been formulated. The American Academy of Pediatrics new Provisional Committee on Emergency Medicine has been charged with the responsibility of developing national standards of emergency care for children and are currently at work on such a landmark document. We have had a functioning statewide system for the management of life threatening injuries in children for the past 10 years. In 1985, 320 children were admitted to our trauma center. The evolution, organization, and current status of the Maryland system is described in this report. Hopefully it may serve as one successful model which could be modified for use in other regions of the country.

Adolescent↗
PubMed1989

Adenosine triphosphate (ATP) treatment of hypoxic pulmonary hypertension (HPH): comparison of dose dependence in pulmonary and renal circulations.

In this study we investigated the relationship between pulmonary and renal responses to a low dose infusion of adenosine triphosphate-magnesium chloride (ATP-MgCl2) in a newborn piglet model of hypoxic pulmonary hypertension (HPH). Three- to five-day-old piglets were cannulated for the measurement of pulmonary and systemic arterial pressure, pulmonary and renal artery flow, and urine output. The animals were then made hypoxic by ventilation with a mixture containing 10% oxygen, 4% CO2, and balance nitrogen. Serial infusions of ATP-MgCl2 at 0.01, 0.025, 0.05, 0.1, 0.5, and 1.0 mg/kg/min were compared to preinfusion hypoxia baselines. Hypoxia alone produced a significant elevation in pulmonary artery pressure (73%) and a reduction in pO2. All dose rates of ATP-MgCl2 greater than or equal to 0.05 mg/kg/min produced a significant decrease in mean pulmonary artery pressure. However, as baseline mean pulmonary artery pressure increased there was an increased sensitivity to ATP-induced vasodilation. Pulmonary artery flow did not change during ATP infusion, thus, the change in pulmonary vascular resistance accounted for the decrease in pulmonary artery pressure. In contrast to the response of the pulmonary vasculature, systemic pressure was significantly decreased only during the 1.0 mg/kg/min infusion. Renal blood flow decreased by 46% during hypoxia and decreased further only during the highest dose rate of ATP infused (i.e., 1.0 mg/kg/min). Creatinine clearance (Ccr) and fractional reabsorption of Na (FreNa) also fell during hypoxia, but were not further altered during all but the highest dose of ATP infused.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenosine Triphosphate↗
PubMed1989

The effect of adenosine triphosphate on the functional status of the ductus arteriosus.

We investigated whether a low-dose infusion of ATP-MgCl2 could affect the functional status of the ductus arteriosus during hypoxia-induced pulmonary vasoconstriction. Three-day-old piglets were made hypoxic by ventilation with a mixture containing 10% oxygen, 4% CO2, and balance nitrogen. Serial infusions of ATP-MgCl2 at 0.1, 0.5, and 1.0 mg/kg/min were compared with preinfusion hypoxia baselines. The functional status of the ductus arteriosus was determined by change in transit time of a bolus of iced saline between thermistor probes in the pulmonary artery and aorta. The method was validated using a Blalock-Taussing shunt (subclavian to pulmonary artery) in 3-week-old piglets instrumented in a similar manner. In these three-day-old piglets, hypoxia alone produced a significant elevation in pulmonary artery pressure and reduction in PO2. All dose rates of ATP-MgCl2 produced a significant decrease in mean pulmonary artery pressure. Systemic pressure was significantly decreased only during the 1.0-mg/kg/min infusion. Transit times of a bolus of iced saline during the validation were definitive for characterizing a situation of "shunt open" or "shunt closed." Infusion of ATP-MgCl2 produced no change in the status of the ductus arteriosus in 45 (94%) of the determinations. In only three cases was the effect of ATP-MgCl2 sufficient to result in a functional change in the status of the ductus arteriosus. Pre- and postductal pulmonary artery PO2 were not altered during ATP-MgCl2 infusion, thus corroborating the transit time determinations. From these results, we conclude that an infusion of ATP-MgCl2 does not alter the functional status of the ductus arteriosus.

Adenosine Triphosphate↗
PubMed1989

Outcome of pectus excavatum in patients with Marfan syndrome and in the general population.

We reviewed the records of 28 patients with Marfan syndrome and 30 age-matched control patients with presumed isolated pectus excavatum to determine the outcome of surgical repair of the pectus deformity in Marfan syndrome. One third of the patients with Marfan syndrome underwent repair of the pectus excavatum before diagnosis. Of the 30 patients with "isolated" pectus excavatum, 17 had findings by history or physical examination, such as mitral valve prolapse, scoliosis, or a relative with pectus excavatum, suggestive of an underlying disorder of connective tissue. Pectus excavatum of more than moderate severity recurred in 11 of 28 patients with Marfan syndrome and was associated with young age at initial surgery and lack of temporary internal stabilization of the chest after surgery. Only two of the control patients had recurrence of the defect; one of these patients had findings suggestive of an underlying heritable disorder of connective tissue. We conclude that pectus excavatum may indicate the presence of an underlying heritable disorder of connective tissue such as the Marfan syndrome. In patients with Marfan syndrome, and possibly other inherited connective tissue disorders, surgical repair should be delayed if possible until skeletal maturity is nearly complete and should employ internal stabilization.

Adolescent↗
PubMed1989

Evolving management of pectus excavatum based on a single institutional experience of 664 patients.

Most pediatricians and family physicians believe that children with pectus excavatum require surgery only for cosmetic indications and then only in teenagers. We believe pectus excavatum should be repaired in childhood (1) to relieve structural compression of the chest and allow normal growth of the thorax; (2) to prevent pulmonary and cardiac dysfunction in teenagers and adults; and (3) to obviate the cosmetic impact that may cause a child to avoid sports and gymnastics. Preoperative CT scans now help select those children who need repair to prevent progressive deformities. Pulmonary function studies during vigorous exercise can document respiratory dysfunction in teenagers. These features are reversible if repair is completed before the pubertal growth spurt. The ideal age for repair is 4 to 6 years, which permits enough emotional maturity for a positive hospital experience and avoids later psychological effects. Repair at an earlier age has no operative advantages. Our operative technique consists of the removal of three to four overgrown costal cartilages, repositioning of the sternum with a transverse osteotomy, and internal support using the child's lowest normal ribs, avoiding any prosthetic support. To prevent recurrence in teenagers, we add a temporary bar beneath the sternum to prevent depression of the sternum from the weight of the chest-wall muscle mass. Six hundred sixty-four patients have been followed for 1 to 40 years; 95% have excellent long-term results and only 5% have mild to moderate recurrences. Our current techniques of patient selection and surgery will be presented.

Adolescent↗