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Biomedical subjects

B Shandling

Publications and source records attributed to B Shandling.

At least 55 records · Page 3Linked to original sources
PubMed1990

Recurrent pheochromocytomas in children.

Between 1958 and 1987, 13 children (aged 8 to 17 years) with 20 pheochromocytomas were diagnosed and treated. Five of these 13 had multiple (12) tumors and four patients had five recurrences in 6 years (1980 to 1986). There were three girls and one boy between 8 and 15 years of age. One girl had von Hippel-Lindau disease. Three of four original tumors were adrenal and less than 6 cm in diameter. All were excised without complications and went home normotensive. The five recurrent tumors were diagnosed and treated within 6 years of the original tumor when the children were 9 to 17 years; their presenting symptoms and signs (3 months) were hypertension plus others, not necessarily the same as previously. Computed tomography scan was again positive in the three that had this test; vanillylmandelic acid was the only uniformly positive biochemical test. Norepinephrines were positive in the three patients examined. The three girls had their recurrent tumors found in the opposite adrenal and the boy once again had two more tumors (adrenal and chest); all tumors were less than 3 cm in diameter and easily excised. They remain normotensive and well up to 8 years. Within this small group of recurrences, there was no specific pattern that would have led one to suspect that they would return with another pheo. There was nothing unusual about their recurrent tumor, except that it was usually in the opposite adrenal; only one of the four children has no remaining adrenal tissue. Although follow-up is important, recurrent pheochromocytomas seem to be heralded by recurrent symptoms and signs of hypertension.

Adolescent↗
PubMed1990

Complications of gastroesophageal antireflux surgery in neurologically impaired versus neurologically normal children.

Antireflux surgery was performed in 234 children over a 5-year period; 153 were neurologically impaired (NI) and 81 were neurologically normal (NN). Initial presentation, demographic data, and type of antireflux operation were similar in the two groups. Eighty-six percent of the NI group versus 30% of the NN group had gastrostomy tubes placed. The incidence of late postoperative complications was 26% in the NI group and 12% in the NN group (P less than .01). During the late postoperative period, NI children underwent reoperation four times as frequently as NN children (19% v 5%, respectively; P less than .01). Wrap herniation accounted for 38% of complications and 59% of reoperations in the late postoperative period. Mortality due to aspiration occurred in 9% of the NI group versus 1% of the NN group. Combined failure rate (reoperation plus aspiration-induced deaths) was 28% in NI and 6% in NN (P less than .01). We conclude that neurological status is the major predictor of operative success and that wrap herniation due to crural disruption is the most common cause of operative failure.

Adolescent↗
PubMed1990

Thymectomy in juvenile myasthenia gravis.

The long-term results of thymectomy in 24 children with generalized myasthenia gravis are reviewed. Sixteen had complete remission and another seven were improved. This compares favorably with reported spontaneous remission rates of 30%. Because of the low morbidity in recent reports and the possibility that early thymectomy is more beneficial, we recommend thymectomy at the onset of juvenile generalized myasthenia gravis.

Adolescent↗
PubMed1989

Neuroblastoma after prenatal exposure to phenytoin: cause and effect?

We evaluated the causality of the association between intrauterine exposure to phenytoin and postnatal neuroblastoma using an in vitro lymphocyte toxicity assay for phenytoin-induced reactions in an unusual sibship. In addition, we investigated intrauterine phenytoin exposure in a case series of infants and children with neuroblastoma diagnosed over 17 years at our center. The response of lymphocytes from our index case with neuroblastoma exposed in utero to phenytoin was within the normal range, whereas the mother and a sibling with fetal hydantoin syndrome (FHS) exhibited an intermediate toxicity. None of the 188 cases of childhood neuroblastoma diagnosed between 1969 and 1988 had been exposed in utero to phenytoin, indicating that, statistically, the drug cannot be associated with neuroblastoma in more than two cases with this malignancy in our cohort, or in 1.5% of all cases of neuroblastoma. Although our data do not suggest an association between phenytoin in pregnancy and postnatal neuroblastoma, it is still possible that there is an increased risk for neuroblastoma in children with FHS.

Bone and Bones↗
PubMed1989

The late-presenting pediatric Morgagni hernia: a benign condition.

Data concerning 15 infants and children with late-presenting (more than 8 weeks) Morgagni hernias over the last 20 years (1966 to 1986) have been reviewed. Ten of the cases were clinically normal on presentation, and the most common symptoms and signs were gastrointestinal and respiratory. Only one child presented with acute symptoms. Five had previously normal chest x-rays, and two others had an incorrect initial radiologic assessment. Chest x-ray was the most common diagnostic test; preoperative barium studies were performed in three patients. Twelve patients had other major congenital abnormalities. Fourteen of the 15 had surgery, usually within days of presentation. At operation, 10 of the 14 hernias contained a hollow viscus, nine had a sac, and four had abnormal bowel fixation. Postoperatively, two children had radiologic evidence of impaired diaphragmatic motility. There was no mortality in this series. Overall, late-presenting Morgagni hernias are relatively benign.

Child↗
PubMed1989

Esophageal atresia with distal tracheoesophageal fistula: associated anomalies and prognosis in the 1980s.

Ninety-seven newborns with esophageal atresia and distal tracheoesophageal fistula (EA-TEF) were treated between 1979 and 1985 inclusive; there were 54 boys and 43 girls. Their weights ranged from 800 to 4,000 g (average, 2.5 kg). They included: 28 neonates with cardiac defects (most common: patent ductus arteriosus [PDA], ventricular septal defect and atrial septal defect [VSD-ASD]), of whom 18 survived (64%); 17 babies with other gastrointestinal anomalies (imperforate anus, duodenal atresia), of whom 12 survived (70%); 12 patients with skeletal malformations (digital, vertebral), of whom 11 survived (91%); 8 newborns with genitourinary abnormalities (hypospadias, undescended testis), of whom 6 survived (75%); and 16 infants with other congenital lesions (trisomy 18, lung agenesis-hypoplasia), of whom 3 survived (18%). Forty-six infants (average, 2.7 kg) had no other anomalies and all survived. As the number of systems with defects increased, both the weight of the baby and survival rate decreased. From this entire series of 97 newborns with EA-TEF, 81 (83%) survived (average, 2.3 kg). Sixteen babies died (average, 1.9 kg); 11 had defects incompatible with life. Eleven of the 16 were never operated on (seven patients with trisomy died within 5 days, and four patients with complex cardiac defects died within 3 weeks). Four of the 16 who were operated on died between 3 months and 2 years from chest problems and one newborn died in the operating room. We concluded that (1) newborns who have the common type of EA-TEF will almost certainly survive if there are no other anomalies; (2) the most frequently associated congenital defects are cardiovascular (28%), gastrointestinal (17%), skeletal (12%), and genitourinary (8%); (3) as the number of systems with defects increases, the weight of the baby and its survival rate fall; and (4) 11% will have trisomy and/or complex cardiac defects with no survival.

Abnormalities, Multiple↗
PubMed1989

Splenic injury: a 5-year update with improved results and changing criteria for conservative management.

The policy of conservative nonoperative management of the injured spleen is reviewed, and recommendations are made to improve this mode of therapy. From 1981 to 1986, 75 patients were admitted with diagnosed splenic injury. Of thes, ten were operated on (four splenorrhaphies, three total splenectomies, one partial splenectomy; in two, the spleen had stopped bleeding spontaneously) and three died, all from causes unrelated to splenic trauma. Only 23% of the patients treated nonoperatively required blood transfusions, and the length of both hospital stay and time spent in the ICU was reduced. The results of this review show that, in comparison with our previous 5-year report, the number of patients treated without surgery increased from 70% to 87%, those receiving blood transfusions decreased from 36% to 23%, and the number undergoing a splenectomy decreased from 24% to 4%. These data suggest that almost all children with splenic injury can be successfully treated without an operation, those who are hemodynamically stable do not require ICU care, and the total hospital stay for uncomplicated splenic injury can be limited to seven days. A laparotomy can be safely reserved for patients with immediate massive hemorrhage or with transfusion requirements of greater than 40 mL/kg.

Adolescent↗
PubMed1989

Splenic injury: a 5-year update with improved results and changing criteria for conservative management.

The policy of conservative nonoperative management of injured spleens is reviewed and recommendations are made to improve this mode of therapy. From 1981 to 1986, 75 patients were admitted with diagnosed splenic injury. Of these, ten were operated on (4 splenorrhaphies, 3 total splenectomies, 1 partial splenectomy) and three died, all from causes unrelated to splenic trauma. Only 23% of the patients treated nonoperatively required blood transfusions, and the length of both hospital stay and time spent in the intensive care unit (ICU) was reduced. The results of this review show that, in comparison with our previous 5-year report, (1) the number of patients treated without surgery rose from 70% to 87%, (2) those receiving blood transfusions dropped from 36% to 23%, and (3) the number undergoing a splenectomy fell from 24% to 4%. These data suggest that (1) most children with splenic injury can be successfully treated without operation, (2) those who are hemodynamically stable do not require ICU care, and (3) the total hospital stay for uncomplicated splenic injury can be limited to seven days. A laparotomy can be safely reserved for patients with immediate massive hemorrhage or with transfusion requirements of greater than 40 mL/kg.

Adolescent↗
PubMed1988

Childhood diaphragmatic hernias presenting after the neonatal period.

Twenty-six patients with Bochdalek hernias and 15 with Morgagni hernias were evaluated in a survey of children presenting over the age of 8 weeks during a 20 year period. Ten of the children had previously normal chest radiographs. Radiological assessment at presentation was incorrect with 15 Bochdalek and two Morgagni hernias. These errors were more common in interpretations by general radiologists. Inappropriate thoracocentesis had been performed in four cases misdiagnosed as pneumothoraces. Barium studies, fluoroscopy, erect views and plain radiographs after nasogastric intubation were helpful investigations. Post-operative evidence of lobar collapse and pulmonary hypoplasia was frequently not appreciated at the time of clinical and radiological follow-up. Other congenital anomalies were commonly seen, particularly with Morgagni hernias. Bowel malfixation and malrotation, an important association, was frequently present but was occasionally overlooked at operation, necessitating further surgery for bowel obstruction.

Child↗
PubMed1988

Central venous catheters--no X-rays needed: a prospective study in 50 consecutive infants and children.

A technique of central venous line (CVL) placement using ECG guidance is described. The method utilizes equipment found in most operating room departments, is easily learned and taught, and obviates the need for x-rays. CVL placement in 50 consecutive infants and children was performed over a 4-month period by the ECG-guided method, with catheter tip position subsequently determined by x-ray study. The technique demonstrated a 96% success and 100% accuracy rate. The two technical failures were both preventable. Equipment malfunction was responsible for one failure. The second failure was secondary to a preexisting supraventricular arrhythmia, and patients with arrhythmias or pacemakers are not suitable candidates for this technique of central venous catheterization. Radiologic methods of CVL placement were necessary in these two patients.

Adolescent↗
PubMed1988

Timing of surgery for congenital diaphragmatic hernia: is emergency operation necessary?

Congenital diaphragmatic hernia (CDH) is considered by most researchers to be a surgical emergency. However, early repair does not necessarily improve respiratory function or reverse fetal circulation, and many patients deteriorate postoperatively. As a result, in 1985, we began to employ a protocol in which surgery was delayed until the PCO2 was maintained below 40 and the child was hemodynamically stable; children in whom these criteria could not be achieved died without surgical repair. Sixty-one consecutive infants with CDH were managed over 4 years; 31 from 1983 to 1984 (group 1) and 30 from 1985 to 1986 (group 2). The groups were similar with respect to sex, side of the defect, birth weight, gestational age, incidence of pneumothorax, and blood gases. High frequency oscillation was used with increasing frequency during the study period, for patients with refractory hypercarbia (13% in group 1, 30% in group 2). All patients were initially paralyzed and ventilated. Mean time from admission to surgery was 4.1 hours in group 1 and 24.4 hours in group 2 (P less than .05). In group 1, 87% of patients had surgical repair (77% within eight hours of admission, 10% after eight hours), and in group 2 only 70% of patients had surgery (10% within eight hours, 60% after eight hours). All patients who were not operated on died. Overall mortality was 58% in group 1 and 50% in group 2; this difference was not statistically significant. These data indicate that our current approach has not increased overall mortality.(ABSTRACT TRUNCATED AT 250 WORDS)

Emergencies↗
PubMed1988

The late-presenting pediatric Bochdalek hernia: a 20-year review.

A 20-year retrospective study was made of children with congenital posterolateral (Bochdalek) hernias presenting more than 8 weeks after birth. The records of 26 patients (16 boys and 10 girls) were evaluated. Sixteen infants and children (62%) were originally misdiagnosed clinically and radiologically as having either infective lung changes, congenital lung cysts, or pneumothoraces; inappropriate thoracentesis occurred in four patients misdiagnosed as having a pneumothorax. Five patients had previously normal chest radiographs. The most useful investigation was a plain radiograph following passage of a nasogastric tube. Coexisting abnormalities (in particular, gut malfixation and malrotation) were common. All patients except one were operated on within days of presentation, and as emergencies if symptoms were acute. More than one third of our patients were left with a smaller than normal ipsilateral lung after their diaphragmatic hernia repair, and these lungs must be considered hypoplastic to some degree. Chest tubes made no difference in the lung's eventual expansion. Two deaths occurred as a result of acute cardiorespiratory arrest in previously well children. Therefore, the symptoms, signs, and radiologic findings of patients with diaphragmatic hernias presenting after the neonatal period may be difficult to interpret, and may result in diagnostic delay, misguided therapy, and a potentially fatal outcome.

Child↗
PubMed1988

Osteomyelitis of the cervical spine presenting as a neurenteric cyst.

A healthy 3-week-old baby girl developed a cyanotic spell that required intubation and ventilation. During part of her initial emergency examination and treatment, a neck mass was felt, and a positive blood culture grew staphylococcus aureus. She was transferred to the ICU, and was ventilated and treated with intravenous cloxacillin. Bronchoscopy showed a paralyzed left cord. Computerized tomography (CT) scan of her neck showed a midline mediastinal mass (behind the compressed trachea and esophagus), that extended from C7 to the carina. Because of the suspicion of an abnormal C7 vertebral body, diagnosis of a neurenteric cyst was made, and a myelogram showed a complete block at the T1 level and an absent C7 vertebral body. There were no neurologic signs. Her right knee then became red and swollen, and x-rays showed a lytic area in the distal femur. This knee was explored under general anesthesia, and an osteomyelitis found and drained. Several days later, a barium swallow showed the mediastinal mass pushing the esophagus to the left, but several more cervical vertebrae were "missing," and the diagnosis of osteomyelitis of the cervical spine was confirmed. The mediastinal staphylococcal abscess was then drained through the neck. Follow-up has been unremarkable over the last 4 years.

Cervical Vertebrae↗
PubMed1987

Twenty-one year experience with the pediatric gastric tube.

From 1964 to 1985 inclusive, gastric tubes have been constructed in 36 infants and children to replace a congenitally defective esophagus or an esophagus with acquired irreparable damage. This report reviews our entire series to date covering a 21-year experience. There were 24 boys and 12 girls ranging in age from 3 months to 17 years. Twenty-two patients were operated on because of congenital esophageal defects and 14 for acquired lesions. A proximally based antiperistaltic tube was constructed in 32 instances, and a distally based isoperistaltic tube made in four. Thirty tubes were passed retrosternally, five through the chest (four with an anastomosis) and one subcutaneously. Twenty-nine infants and children had their tube construction staged, and the remaining seven had the entire procedure done in one stage. The spleen was removed in 23 early gastric tube operations. There were 19 extratube major complications. Twenty-four gastric tube neck anastomoses leaked (66%); all but one closed spontaneously within 3 months. There were 15 esophagogastric tube neck anastomotic strictures (41%), which were dilated; nine required resection. Thirty-two of the 36 infants and children have been followed for more than 1 year. Twenty-nine are swallowing normally, one is still being dilated, and one infant still does not swallow well. Mild sacculation or tortuosity of the gastric tube has been encountered only one. Growth was slow in four until the school years. One boy had his improperly made tube eventually discarded for a colon replacement. There were three deaths (tracheostomy complication, aspiration, gastric tube ulcer hemorrhage). Despite the above problems, the eventual outcome continues to be satisfactory and satisfying.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗