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Biomedical subjects

M Zer

Publications and source records attributed to M Zer.

At least 37 records · Page 2Linked to original sources

Should repeated partial splenectomy be attempted in patients with hematological diseases? Technical pitfalls and causes of failure in Gaucher's disease.

BACKGROUND/PURPOSE: The awareness of the risk of overwhelming sepsis after splenectomy prompted surgeons to attempt splenic preservation in patients who had hematologic diseases for which splenectomy was the conventional treatment. Partial splenectomy for Gaucher's disease was widely performed before the introduction of alglucerase. In sporadic cases a second partial splenectomy had also been attempted. METHODS: The authors present three cases of failed repeated partial splenectomy attempted before alglucerase was available. The role of angiography in planning operative strategy and the surgical pitfalls of this unusual reintervention are discussed. CONCLUSION: New indications for partial splenectomy in other hematologic diseases makes the experience gained with Gaucher's disease valuable for management decisions.

Adolescent↗

Oesophageal plastic repair for symptomatic ballooning following circular oesophageal myotomy and correction of oesophageal atresia.

Two patients with refractory anastomotic stenosis and symptomatic ballooning of the upper oesophageal pouch following repair of long gap oesophageal atresia are described. In both cases a circular myotomy had been used to elongate the proximal oesophageal segment at the time of primary repair. Both patients were successfully treated by Y-V plasty of the oesophageal stenosis and tailoring of the dilated segment.

Esophageal Atresia↗

Necrotizing enterocolitis: the impact of the establishment of a Department of Pediatric Surgery on the Beilinson Medical Campus.

Necrotizing enterocolitis (NEC) is the most commonly acquired gastrointestinal emergency in neonates. We retrospectively surveyed all cases of confirmed NEC treated at the Beilinson Medical Center (BMC) (now Rabin Medical Center, Beilinson Campus) during a 12 year period to determine whether the establishment of a department of pediatric surgery has influenced the treatment and outcome of NEC patients. Of the 48 patients, 23 were patients treated in the "early period", 1982-87, when surgery was performed in the Department of General Surgery by staff trained in pediatric surgery (group I), and 25 were treated in the "later period" 1988-93, in the same neonatal intensive care unit, with surgical supervision by the new Department of Pediatric Surgery (group II). We found that 32% of the group II patients were of extremely low birthweight (<1,000 g) compared with 13% of group I. They also had lower Apgar scores, and a higher percentage needed respiratory assistance (56 vs. 26%, P = 0.02). All those infants of group II who had been born in other hospitals and were transferred to BMC for treatment were in an advanced state of disease and all required surgery. Total mortality decreased from 22% in group I to 8% in group II. This decrease may be attributed to improved supportive treatment in the neonatal intensive care unit, earlier surgery for NEC based on relative rather than absolute indications, higher rates of primary resection, and better postoperative care. Our review indicates that the establishment of a Department of Pediatric Surgery at the BMC has contributed to the considerable improvement in NEC outcome in our center.

Academic Medical Centers↗

Vertical partial splenectomy for epidermoid cyst.

Benign lesions of the spleen usually can be surgically removed by partial splenectomy. When the lesion is longitudinal and occupies most or the entire length of the spleen, partial splenectomy using the common surgical technique, based on the distribution of the organ's blood supply, is impossible. A new approach for "vertical" partial splenectomy for epidermoid cyst is described, using splenic artery occlusion and straight suture needles.

Child↗

Left ovarian cyst and right streak ovary in a neonate with a normal karyotype. Report of a case of neonatal Slotnick-Goldfarb syndrome or recessive gonadal dysgenesis.

A female neonate with a streak ovary on one side and huge ovarian cyst on the other side is presented. Her karyotype was 46,XX. Adnexectomy was mandatory, leaving the patient with a single streak gonad. Whether this infant has the recessive form of gonadal dysgenesis with one ovary, streak or hypoplastic, replaced by a cyst or the Slotnick-Goldfarb syndrome is impossible to elucidate at present. The potential implications of a streak gonad, either related to gonadal dysgenesis or as a possible source of neoplasia, are reviewed. Current approaches to an ovarian cyst and streak ovary in the neonatal period are discussed.

Female↗

Management of urachal anomalies in children and adults.

During a period of fifteen years, 9 children and 5 adults with a variety of urachal anomalies were treated. Infected urachal cysts were more common in children whereas adults more frequently had infected urachal sinuses. After careful physical examination, a preoperative diagnosis could be made in most patients, with ultrasound examination decisive in doubtful cases. Whenever feasible, complete excision of the umbilicovesical tract is performed, but in very ill patients, a staged treatment becomes necessary. The preoperative injection of methylene blue is helpful in the identification of communicating tracts, all of which should be removed. All affected children should undergo investigation for associated genitourinary anomalies.

Adolescent↗

Malignant duodenocolic fistula. The role of extended surgery.

Malignant duodenocolic fistula is a seldom-seen complication of advanced right colon carcinoma. We describe four patients who illustrate the sometimes rewarding results of aggressive treatment. Because duodenocolic fistula is always associated with severe nutritional impairment, operation should be deferred until total parenteral nutrition (TPN) and blood transfusions have improved the clinical state. Whenever feasible, resection offers the best treatment; lesser techniques such as bypass and exclusion result only in minimal palliation. A direct approach and fistula disconnection are contraindicated. The benefit of exploration should almost always be offered, even in such secondary fistulas, as a better quality of life and long-term survival are realistic goals.

Aged↗

The value of precise preoperative localization of colonic arteriovenous malformation in childhood.

Massive hemorrhage from the gastrointestinal tract in a 12-yr-old boy caused by a congenital atypically located colonic arteriovenous malformation is described. Guided and "clean" resection of the involved colon was possible due to preoperative selective angiography, which proved to be the most efficient diagnostic tool. Histologic documentation of this rare pathology in childhood is presented, and the classification and features of the disease are briefly reviewed.

Angiography↗

Familial chronic recurrent pancreatitis in identical twins. Case report and review of the literature.

Familial presentation of chronic recurrent pancreatitis in childhood is rare. The etiology of this illness is obscure, and its hereditary properties are not well defined. Simultaneous occurrence of chronic recurrent pancreatitis in identical twins with the same clinical presentation and similar typical pancreatographic abnormalities is exceptional. Twin sisters, aged 9 years, were admitted to the hospital because of recurrent attacks of pancreatitis. Ultrasound examination revealed an enlarged irregular pancreatic duct in both girls, and endoscopic retrograde cholangiopancreatography showed a distorted duct with multiple strictures and dilatations similar to a "chain of lakes" pattern. Both patients underwent longitudinal pancreatojejunostomy within a month. The therapeutic regimen and preoperative and surgical treatment of such patients are discussed, as is the optimal timing of intervention.

Child↗

Subtotal splenectomy in Gaucher's disease: towards a definition of critical splenic mass.

Partial splenectomy is currently the favoured surgical approach for Gaucher's disease. Preservation of splenic tissue is indicated to prevent susceptibility to overwhelming postsplenectomy sepsis, to delay the massive deposition of glucocerebroside in the liver and bones, and to relieve symptoms of hypersplenism. Controversial issues and technical problems related to partial splenectomy for Gaucher's disease are discussed and a definition of critical splenic mass considered.

Gaucher Disease↗

Neonatal intestinal perforation due to congenital defects in the intestinal muscularis.

Congenital defect of the muscular layer of the small intestine is a rare cause of spontaneous bowel perforation in premature infants. During the last 12 years we have observed four similar cases. We describe the most recent one, a premature infant who developed two abdominal events. On her 2nd day of life, spontaneous perforation of the distal ileum due to focal absence of the muscular layer occurred. Several weeks later she developed the typical clinical and histological picture of necrotizing enterocolitis. The clinical and histological characteristics of the two different conditions are compared, and the 24 cases reported in the literature are discussed. We conclude that focal absence of intestinal musculature may be not such a rare entity as is commonly believed.

Female↗

Long-term follow-up of partial splenectomy in Gaucher's disease.

Seven children with Gaucher's disease who underwent partial splenectomy were followed for 7 to 8 years. None of the children had systemic sepsis or symptoms related to liver enlargement. Bone crisis occurred in only two children who had experienced bone crisis prior to partial splenectomy. This contrasts with the development of bone crises in five of six children who underwent total splenectomy at a similar age and who had previously been free of bone symptoms. Partial splenectomy should be regarded as a temporary solution in the treatment of hypersplenism and the mechanical compression related to the huge spleen. In 71% of the patients, massive enlargement and a severe to moderate degree of pancytopenia occurred again after 3 to 8 years; total splenectomy was required in three patients.

Adolescent↗

Penetrating stab wound of the gluteus--a potentially life-threatening injury: case reports.

Presented are two cases of stab wounds through the gluteus in which serious damage was incurred by internal organs: in one case there was penetration of the rectum with the appearance of signs of acute abdomen; in the other there was tearing of retroperitoneal muscles and the internal iliac vessels with resultant hypovolemic shock. These observations suggest that there should be greater awareness of the life-threatening nature of stab wounds of the gluteus under similar circumstances.

Adolescent↗

[Ascaris lumbricoides--a cause of acute abdomen].

Ascaris lumbricoides infestation is the most common helminthic disease in many developing countries and may cause severe surgical complications, especially in children. We present a 3-year-old Ethiopian immigrant brought to hospital directly from the airport because of signs of acute abdomen. It was found to be caused by volvulus and necrosis of a loop of bowel impacted with worms. The necrotic bowel loop was resected and a temporary ileostomy was formed; recovery was uneventful. This disease and its severe complications are rare in Israel and the western world. Awareness of its occurrence is important in view of the present world-wide large-scale immigration from developing countries.

Abdomen, Acute↗

Inflammatory pseudotumor in childhood. A diagnostic and therapeutic dilemma.

We describe a case of inflammatory pseudotumor of the lower abdomen in a young child. The urinary bladder and abdominal wall were involved, and the pseudotumor simulated a malignant sarcomatous tumor. Surgical excision was undertaken, and the outcome was favorable. This rare tumor simulates a true neoplasm both clinically and morphologically and presents a diagnostic and therapeutic dilemma. Our case and a review of the literature indicate that an effort should be made to perform a radical excision. This is considered the treatment of choice except if it requires a mutilating procedure.

Abdominal Muscles↗