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Biomedical subjects

L Schnaufer

Publications and source records attributed to L Schnaufer.

At least 37 records · Page 2Linked to original sources
PubMed1987

Microdiverticulitis: a rare but distinct cause of small bowel obstruction in early infancy.

During a 20-year period, eight term infants admitted to a large children's hospital for treatment of dehydration associated with diarrhea (6) and vomiting (2), and who ranged in age from 10 to 60 days at onset of symptoms and 18 to 75 days at presentation, developed distal small bowel obstruction following apparently successful rehydration. With the exception of a single infant who was dead on arrival, and another whose obstruction went unrecognized, all infants came to operation shortly after obstruction was confirmed. In each case, a severely inflamed area of distal ileum (7) or proximal colon (1) was found at autopsy (2) or operation (6) to be the cause; perforation was present in four of the cases. Resection of the diseased segments of intestine, and primary anastomosis, were performed in all six operated cases; reoperation was required in four of the six for leaks (3) and adhesions (1). All but two survived. Pathologically, the resected intestinal segments showed a unique pattern of injury: numerous punctate ulcers were apparent, which undermined the muscularis mucosae, without evidence of necrosis. Regenerating epithelium extended through these defects, resulting in the presence of glandular invaginations, which were surrounded by a brisk inflammatory response: hence the term "microdiverticulitis." We believe this lesion represents a beginning or furtive attempt at repair of severely inflamed, but viable intestine, and that it is a rare but true cause of small bowel obstruction in early infancy, separate and distinct from necrotizing enterocolitis.

Diverticulitis↗
PubMed1987

Omphalocele, cryptorchidism, and brain malformations.

Nineteen male infants died with a large omphalocele and 52% had associated cryptorchidism. However, two different groups with both omphalocele and cryptorchidism were recognized: (1) Eleven patients with omphalocele without brain malformation and an incidence of undescended testes not significantly different from the normal population; (2) Eight patients with omphalocele and brain malformation all having cryptorchidism. A comparison of the groups indicated that intact intraabdominal pressure during intrauterine life is not a main driving force of testicular descent, whereas normal testicular descent may occur only when the brain is normally developed. Whenever a child with omphalocele and cryptorchidism is examined, careful evaluation of the central nervous system is indicated. This triad of malformations may have prognostic and therapeutic implications.

Brain↗
PubMed1987

Recent experience with choledochal cyst.

This report details an 11-year experience with 17 patients ranging from newborn to 17 years with choledochal cyst. Two distinct groups were noted: an infantile group (mean age: 3 months) with obstructive jaundice identical to biliary atresia and a late onset group (mean age: 9 years) with various combinations of pain, mass, and jaundice. Two patients had cystoduodenostomy performed and both required revision. One of six patients who had Roux-Y cystojejunostomy required revision. All seven patients who had primary cyst excision and two patients who had secondary cyst excision with Roux-Y hepaticojejunostomy have been followed prospectively and have done well. The follow-up period ranges from 1-11 years with an average of 5.8 years. Cyst excision should be performed as a primary or secondary procedure whenever feasible. The rare patients with intrahepatic ductal dilatation (Caroli's disease) are best approached by hepatic lobectomy when possible, and those with choledochocele should be treated by unroofing the cyst as indicated by the anatomy encountered.

Adolescent↗
PubMed1987

Nonsurgical management of children with recurrent or unresectable fibromatosis.

At The Children's Hospital of Philadelphia, since 1971, six children 3 months to 17 years of age with fibromatosis have been treated with a combination of vincristine, actinomycin D, and cyclophosphamide (VAC). The first three patients also received radiation therapy (5,500 rads). Locally recurrent tumors developed in four of the children after previous operative removal; the other two had tumors that could not be removed initially. The tumors arose in the neck (three patients), pelvis (two patients), or foot (one patient). In the three patients treated with VAC alone, complete disappearance of tumor was confirmed at second operation in two, and greater than 75% shrinkage on CT scans occurred in the third, all at 4 to 6 months after VAC was started. In two of the three patients who received VAC plus radiation therapy, complete disappearance of tumor occurred at 13 and 16 months; the third had no response. Five of the six patients are free of recurrent fibromatosis at 1, 2, 4, and 11 years after VAC was begun; the sixth has required multiple operations during the last 6 years. We conclude that combination chemotherapy with VAC can produce regression of fibromatosis in some children with recurrent or unresectable lesions. The administration of VAC should be considered for children with fibromatosis in whom operative removal is not feasible, would prove mutilating, or is unlikely to produce long-term control of the disease.

Adolescent↗
PubMed1986

Coexistant gastric duplication and accessory pancreas: clinical manifestations, embryogenesis, and treatment.

Combined gastric and pancreatic duplications are uncommon. Although patients usually present with symptoms of intestinal obstruction, gastrointestinal (GI) bleeding may occur if the duplication ulcerates and erodes into a neighboring hollow viscus. An upper GI series and barium enema are helpful in making a diagnosis. Combined duplications probably are produced during embryologic development by traction along a neuroenteric band between the stomach and pancreas. Simple surgical excision is the treatment of choice.

Female↗
PubMed1986

Primary site as a prognostic variable for children with pelvic soft tissue sarcomas.

From 1974 to 1983 we treated 16 children between 1 and 16 years old for soft tissue sarcoma arising in the pelvis, bladder or prostate. An incisional biopsy was obtained in every patient. Each child then was treated with a combination chemotherapy program, incorporating vincristine, actinomycin D and cyclophosphamide with or without doxorubicin, cis-platinum and etoposide. Of the 16 patients 13 (81 per cent) also received radiation therapy. In 8 children with urinary obstruction or hematuria sarcomas arose in the bladder or bladder-prostate region, including 7 who had localized tumors and 1 who had lung metastases at diagnosis. The median tumor diameter in these patients was 5 cm. Of these 8 patients 3 eventually required total cystectomy and prostatectomy to eradicate persistent local tumor, and 6 are alive and remain free of recurrent sarcoma for 1 to 9 years after initiation of therapy. The 8 other children had a pelvic mass at diagnosis, which arose adjacent to but outside of the bladder or prostate, and 2 had lung metastases at diagnosis. The median tumor diameter in these patients was 15 cm. Only 3 of these 8 children are alive and remain free of sarcoma for 1 to 8 years after initiation of therapy. In only 1 of these children was complete tumor excision ever possible despite the use of local radiation therapy and aggressive chemotherapy. Sarcomas arising in the bladder-prostate region are found when relatively small, perhaps because they soon produce overt signs, and they appear to have a better prognosis than those arising in the retroperitoneum-pelvis outside the bladder. Better treatment strategies are needed for the latter group of tumors that often are locally uncontrollable.

Adolescent↗
PubMed1985

Management of esophageal atresia and tracheoesophageal fistula in the neonate with severe respiratory distress syndrome.

In a 10-year period, 22 neonates with esophageal atresia (EA) and tracheoesophageal fistula (TEF) required high pressure ventilatory support soon after birth because of respiratory distress syndrome (RDS). Eleven of the 22 or 50% survived overall, but if the 5 patients who died before definitive surgical repair could be attempted are excluded, 11 of 17 or 65% survived. More importantly, 4 of 7 (57%) patients who had gastrostomy performed first survived while 7 of 10 (70%) who had fistula ligation performed first survived. The difficulties with intraoperative management of those who had gastrostomy performed first were even more impressive. Our experience leads us to conclude that patients with EA and TEF with severe RDS who require high pressure ventilation preoperatively represent a group of patients who require special consideration. The danger to such patients with increased pulmonary resistance is not gastric distention but sudden loss of intragastric pressure. In the presence of poor lung compliance, the upper gastrointestinal tract functions in continuity with the tracheobronchial tree. A sudden loss of intragastric pressure, as with placement of a gastrostomy tube, results in an acute loss of effective ventilating pressure. Resuscitation of such a patient is not possible until leakage from the esophagus is controlled by ligation of the fistula or transabdominal occlusion of the distal esophagus. Placement of a Fogarty catheter into the fistula via a bronchoscope is effective but may not be feasible in every case. Early thoracotomy and ligation of the fistula in patients with progressive RDS provides immediate improvement in ventilatory efficiency and relief of gastric distention.

Airway Resistance↗
PubMed1982

Extraskeletal osteogenic sarcoma after treatment for Wilms' tumor.

A large proportion of children with Wilms' tumor will become long-term disease-free survivors. A small number of these children are at risk of developing second malignant neoplasms. There have been no previous reports of osteogenic sarcoma of the chest wall following treatment of Wilms' tumor. Our patient was age seven years when he received surgery, radiation therapy and chemotherapy for Wilms' tumor, eight years when he received radiation and chemotherapy for pulmonary metastases of Wilms' tumor, and 13 years when he developed osteogenic sarcoma of the chest wall.

Child↗
PubMed1982

Surgical management of reflux strictures of the esophagus in childhood.

The etiology of gastroesophageal reflux (GER) in infancy is related to developmental factors, and there is a high incidence of associated conditions such as neurologic syndromes and esophageal atresia (60%). This is different from the situation in adults. Experience with 18 consecutive children with peptic esophageal strictures is reviewed to determine if conservative surgical management is effective. Eighteen children 14 months to 13 years (mean 6.3 years) of age took an average of 3.5 years from the time of onset of symptoms of GER to develop tight strictures diagnosed by esophagography and esophagoscopy. The incidence of stricture in patients with GER was approximately 15%. Preoperative dilation or direct surgical management prior to correction of reflux is ineffective. All 18 children were managed by intraoperative dilatation, Nissen fundoplication, and guided dilatation after operation. More aggressive surgical procedures were not required nor were associated operations such as pyloroplasty; they are rarely necessary. An average three-year follow-up indicates that this conservative surgical approach is effective in the management of peptic esophageal strictures in childhood with relief of symptoms and gratifying improvement in growth.

Adolescent↗
PubMed1981

Treatment strategies for infants with malignant sacrococcygeal teratoma.

Twelve children with malignant sacrococcygeal teratoma containing embryonal carcinoma, were treated at the Children's Hospital of Philadelphia between 1971 and 1980. Their ages at diagnosis ranged from 2 days to 23 mo; 8 of the 12 (67%) were girls. Five of the 12 patients presented with localized tumors which were grossly completely excised. Four received no further therapy, and all 4 recurred with histologically documented embryonal carcinoma. Despite subsequent treatment with radiation therapy (RT) and chemotherapy (vincristine, actinomycin D, and cyclophosphamide, collectively called VAC, in all 4 plus adriamycin in 3, only 1 has survived free of disease, 8 yr from diagnosis. The other 3 died of tumor (2) or pneumonia after pulmonary RT (1). The fifth patient in this group received VAC with adriamycin (total dose, 350 mg/sq m) and died of autopsy-proven cardiotoxicity without tumor. The remaining 7 patients presented with either unresectable local tumor (3) or distant metastases (4). One of these 7 died 6 days after biopsy with massive liver metastases. The other 6 children were treated after operation with VAC (5) or vincristine and actinomycin D (1) chemotherapy, and 5 also received RT to the pelvis. One has survived free of disease at 6 yr after treatment with VAC and adriamycin and pelvic RT, but the other 5 died of tumor (2) or of pneumonia after pulmonary RT (3). Surgery alone is inadequate for successful management of children with malignant sacrococcygeal teratoma. Chemotherapy with VAC, with or without adriamycin, can be effective when combined with radiation, but fatal pulmonary toxicity can result from such combined therapy. The optimal therapeutic program for children with malignant sacrococcygeal teratoma is still evolving.

Coccyx↗
PubMed1981

Congenital right diaphragmatic hernia. A case report and review of the literature.

Congenital right diaphragmatic hernia can present a difficult diagnostic problem. An illustrative case is reviewed in which the diagnosis was not considered during the initial hospitalization because chest radiographs were interpreted as showing an atelectatic and pneumonic process. Once suspected, the diagnosis was made by fluoroscopy, which showed an immobile right hemidiaphragm, and by liver scan, which demonstrated herniation of the liver into the right thorax. The tendency of congenital right diaphragmatic hernias to present after the neonatal period and to mimic pulmonary parenchymal disease is discussed. Several alternative means of diagnosis of right diaphragmatic hernias are reviewed.

Female↗
PubMed1980

A review of 17 IV-S neuroblastoma patients at the children's hospital of philadelphia.

The records of 207 neuroblastoma patients seen at the Children's Hospital of Philadelphia between 1944 and 1977 were reviewed to study some of the features associated with the unusually good prognosis found in patients with Stage IV-S neuroblastoma. Initially, 22 patients appeared to fit the criteria of small primary tumor and distant disease in liver, skin, and/or marrow without evidence of bone metastases; 5 patients were subsequently rejected as being incorrectly staged. The remaining 17 patients had abdominal primary tumors and hepatic disease; in 12 of the 17, an enlarged liver was the presenting sign. Six patients had skin lesions, 4 had disease in the marrow on routine smear, and additional sites of spread were pancreas and bowel serosa. The treatment given was not systematic, and it was not possible to correlate any specific form of therapy with a satisfactory outcome. Eleven of 17 patients survived; 6 of 11 survivors had spontaneous regression of all or part of their diseases, 5 of 6 who died received irradiation, chemotherapy, or both. Death usually occurred in the first month as a complication of the local disease; 1 patient succumbed to radiation nephritis. This study establishes that the special pattern of widespread neuroblastoma termed Stage IV-S does exist, and that is associated with a good prognosis. Careful consideration should be given before selecting treatment for the Stage IV-S child because spontaneous regression is likely to occur in most of them. In patients with rapidly enlarging livers, renal or pulmonary complications may develop because of liver bulk or coagulopathies. Treatment should be directed to the liver in these cases because distant metastases seldom supervene. Low-dose irradiation, mild chemotherapy, and possibly surgical release of intraabdominal pressure using a silastic patch have all been effective. Unfortunately, patients occasionally succumb to local disease in spite of these and more aggressive measures.

Adrenal Gland Neoplasms↗
PubMed1980

Agenesis of the left diaphragm: surgical repair and physiologic consequences.

Agenesis of the hemidiaphragm is an unusual congenital anomaly associated with a high mortality. This paper presents the fourth patient to survive the neonatal period with agenesis of the hemidiaphragm. He was an identical twin, weighing 1.5 kg and his clinical course was characterized by ipsilateral pulmonary hypoplasia, large alveolar-arterial gradient for oxygen, persistent fetal circulatory pattern and ventilator dependence. These abnormalities suggest a pathophysiology similar to that observed in patients with Bochdalek hernia. The surgical correction, postoperative care and observation of pulmonary function following repair of agenesis of the left diaphragm are described.

Diaphragm↗
PubMed1979

The barium-enema examination and rectal manometry in Hirschsprung disease.

Barium-enema studies and rectal manometry were clearly shown complementary in diagnosing Hirschsprung disease. Of 27 patients studied, 19 had positive barium-enema and rectal manometric studies and were proved histologically to have Hirschsprung disease. Three had positive barium-enemas but normal rectal manometric studies in the first 10 days of life; later manometric studies were positive, and all 3 were subsequently proved to have Hirschsprung disease. Three patients had negative barium-enema and positive manometric studies and were proved to have low segment Hirschsprung disease. If both studies indicate the disease, rectal biopsy is not necessary before surgery.

Barium Sulfate↗
PubMed1978

Treatment of advanced Hodgkin's disease in pediatric patients.

From 1970 to 1976, twenty patients with stage II E or II B to IV B Hodgkin's disease were treated at Children's Hospital of Philadelphia. Initially, four of the stage II or III patients received planned total nodal irradiation (TNI) alone; three patients developed progressive disease during irradiation, and one relapsed after 18 months. These results with TNI led to the use of combined modality therapy. Sixteen patients (4, stage II E or B; 8, stage III; 4, stage IV) were treated with COPP (cyclophosphamide, Oncovin, prednisone, and procarbazine) and radiation therapy. In 14 patients treatment was started with COPP. Patients with disease below L2 received TNI; the rest received involved field (IF) or extended field (EF) irradiation. No patient treated with combination therapy encountered life-threatening toxicity. Relapse-free survival in 12 stage II or III patients is 100% with a median follow-up of 28 months (range, 24 to 91 months). Only one of four stage IV patients is alive. Combined modality therapy is effective, tolerable therapy for children with stage II B--III Hodgkin's disease. No relapses occurred in 10 patients given less that potentially curative radiation. Smaller radiation fields and lower doses are planned for the future.

Adolescent↗