Torsion of uterine adnexa in neonates and children: a report of 20 cases.

Twenty children under the age of 14 years with torsion of the uterine adnexa are discussed. Of the 14 postnatal cases, 8 were associated with additional ovarian pathology whereas in 6 cases the torsion was of normal uterine adnexa. Six additional cases had antenatal torsion with "pseudocyst" formation. All 14 postnatal cases presented as acute abdominal emergency requiring immediate surgery. Only 6 of the 14 had been correctly diagnosed preoperatively, the rest being mostly diagnosed as acute appendicitis. Sonographic studies can improve the preoperative diagnosis. Hemorrhagic necrosis of the adnexa (secondary to the torsion) was found in all cases except one, and necessitated adnexal resection. In only one case preservation of the adnexa was possible. Fixation of the contralateral ovary is recommended in cases of torsion of normal uterine adnexa. All six cases of antenatal torsion were diagnosed by routine ultrasonography during pregnancy between the 29th and 40th week of gestation. The "fluid-fluid" level sign is characteristic of this lesion. These neonates were born in no acute distress and were found to be completely asymptomatic. Hence, they did not require urgent surgery and underwent operation electively at the age of 2 weeks. Resection of the pseudocyst with fixation of the contralateral ovary is the treatment of choice in these cases.

Erythrocyte acetylcholinesterase activity in Hirschsprung's disease in Israel.

Hirschsprung's disease is a relatively prevalent disorder in newborn babies and young children. Acetylcholinesterase (AChE) activity is augmented locally, in the neural plexus of the affected gut segment. It has been suggested that this enzyme will also be increased in red blood cells (RBCs) of affected children. Thus, we studied AChE activity in newborn babies and children, as well as age-adjusted data. This Israeli study consisted of healthy and affected Bedouins as well as Ashkenazi and Sephardic Jews. This study supports the Japanese study in that the RBC AChE is not augmented in Hirschsprung's disease. However, final conclusions cannot yet be drawn, because of the small sample.

Unusual presentation of omphalomesenteric duct remnant: a variant of mesodiverticular band causing intestinal obstruction.

Two unusual cases of small bowel obstruction associated with an omphalomesenteric duct remnant are described in 2 girls aged 14 years. The causative factor was a stenotic area in the terminal ileum caused by a ringlike lipovascular mesenteric band encroaching externally on the lumen. A phytobezoar was lodged proximally. This is as yet an undescribed variant of a mesodiverticular band.

Isolated torsion of the fallopian tube in premenarcheal girls.

The authors report on two premenarcheal girls, ages 4 and 13 years, with isolated torsion of the fallopian tube, an extremely rare condition. The fallopian tube was salvaged by detorsion in the younger girl, the first salvage described in the English literature on children. The mechanism of the isolated torsion and the clinical implications are discussed.

Transaxillary upper thoracic sympathectomy for primary palmar hyperhidrosis in children and adolescents.

Primary palmar hyperhidrosis is part of a triad of palmar, plantar, and axillary hyperhidrosis of unknown etiology, affecting children, adolescents, and young adults. Sixty-seven children and young adolescents were operated on during a 10-year period. A total of 103 transaxillary upper thoracic sympathectomies (36 bilateral) were performed, with no mortality. The immediate postoperative course was uneventful in 90%; the other 10% had mostly minor problems. The average hospitalization period was 3 to 4 days. Total abolition of palmar sweating was achieved in all but two patients in whom some residual moisture remained. Long-term extreme satisfaction was reported by 64 of 67 patients (94%). One was moderately satisfied, and two were not satisfied because of excessive "compensatory" sweating elsewhere. Compensatory sweating of some degree was reported by 45% of patients but did not alter satisfaction. By further limiting ganglionectomy to just one ganglion (T2 or T3), compensatory sweating possibly may be reduced further. Early surgery for severe palmar hyperhidrosis will save a child many years of agony and social discomfort because all types of conservative therapy are ineffective and cause unnecessary delay. A limited transaxillary upper thoracic sympathectomy is presently the authors' preferred approach, although ablation via thoracoscopy should not be excluded as further experience is gained with this modality.

Bridging of esophageal defects with lyophilized dura mater: an experimental study.

The aim of this study was to investigate the use of a prosthetic biologic material-lyophilized dura mater (Lyodura) in patching esophageal defects in dogs, having in view its potential use in bridging long gap congenital esophageal atresia and patching acquired esophageal defects. To follow the incorporation process, 20 mongrel dogs had full-thickness, 6 cm2 cervical esophageal defects, patched with Lyodura. The dogs were sacrificed at different postoperative periods and the patched esophagus removed for gross and microscopic examination. In a second group of 10 dogs, a segment of the esophagus was excised and replaced by 3 cm in length and 2 cm in diameter, Lyodura tubes. In this group, prolonged follow-up was undertaken including radiologic, endoscopic, and histological assessment. Special attention was given to the swallowing function and to growth and development. This study shows that: (1) the area of prosthetic replacement was characterized by a narrowing of the esophageal wall and the histopathologic study showed that the epithelialization process was complete in about 2 months; and (2) muscle reconstruction did not take place at the patched areas. We conclude that, pending further studies and improvement, lyophilized dura mater can be considered as a successful alternative for bridging esophageal defects.

Near total intestinal aganglionosis with extreme short-bowel syndrome--a difficult surgical dilemma.

Forty cases of total or near-total intestinal agangliosis (NTIA) were described to date in the English literature. Most cases had a lethal outcome. We describe the 41st case--a Beduin male neonate--who had only 30 cm of proximal hypoganglionic jejunum. He is presently almost one-year-old and thriving on home TPN, receiving one quarter of his caloric requirements orally using pregestamil, an MCT formula. The initial intricate course, diagnosis and several operative procedures, are elaborated. A review of the scant literature is discussed. The elusiveness of the correct diagnosis is pointed out and means to overcome these errors are described. Various surgical procedures have been suggested, none of which offer the perfect solution to the severe basic problem of short bowel. Long-term parenteral hyperalimentation is still the main modality of treatment. Based on our modest experience, we suggest saving every possible length of jejunum, even if hypoganglionic, since this bowel, following a few weeks of adaptation, starts to function fairly well, suggesting perhaps some neuro-muscular maturation. The best surgical approach is still pending. We present a report of a child with this disease and discuss the therapeutic dilemma.

[Traumatic rupture of the diaphragm in an infant].

Traumatic rupture of the diaphragm can be caused by both penetrating and blunt trauma. High kinetic energy applied to the abdomen during blunt abdominal trauma creates pressure differences between the abdominal and chest cavities. These sudden changes in pressure rupture the diaphragm. We present a 13-month-old infant admitted after being injured by a car. In addition to rupture of the diaphragm there were pelvic fractures. Chest x-ray on admission was diagnostic for diaphragmatic rupture. The possibility of congenital diaphragmatic hernia was rejected because of increasing ventilatory embarrassment.

[Psoas abscess: diagnostic dilemma in childhood].

A series of 16 children diagnosed as having a psoas abscess or deep iliac lymphadenitis seen over a 25-year period (1970-1994) is presented. 10 were treated conservatively while 5 were drained surgically and 1 percutaneously under imaging guidance. The presenting signs and symptoms may mimic the frequently seen entities, acute appendicitis and acute hip arthritis. Lower abdominal and inguinal pain, limp, fever and increased white count are common in all of these conditions. Accurate differential diagnosis is necessary to avoid unnecessary surgery due to a wrong diagnosis. Ultrasonography is preferred for diagnosis. Antibiotic therapy should be instituted immediately, aimed primarily at Staphylococcus aureus, the most common causative agent, although other organisms may be implicated. When a psoas abscess has been diagnosed, surgery and drainage are indicated. One of the preferred approaches is percutaneous drainage under imaging guidance. Convalescence is usually rapid and without late sequelae.

[Hirschprung's disease in the Negev].

We treated 65 children with proven Hirschprung's disease between 1970-1992. After definitive surgery, 35 were over 10 years of age and 13 were over 18. The male:female ratio was 4:1. All but 3 were born full-term. 44% were of Bedouin origin, with a higher prevalence in 3 families of 2 tribes. 38 (58%) were diagnosed in the neonatal period: by barium enema and rectal muscle biopsy in 42 (65%), and by barium enema alone in 23 (35%). In the latter the diagnosis was verified by intra-operative biopsy. Severe constipation, intestinal obstruction or enterocolitis were the presenting features. 19 associated anomalies were found in 12 children, but none was life-threatening; 5 (8%) had cardiac anomalies; none had Down's syndrome. The rectosigmoid colon was the most common aganglionic segment involved (only 1 had total colonic aganglionosis). 7 of the 8 with short segment involvement responded well to posterior rectal myectomy. 55 patients had an abdominoperineal pull-through: 48 by Swenson's procedure and 5 by the Soave and 2 by the Duhamel modifications. In 43 a protective colostomy was performed at the end of the procedure. 53 had complete diversion colostomy at the time of initial diagnosis (neonatal and early infancy). There was no intra- or immediate post-operative death. 1 patient died 2 months after operation of complications following enterocolitis and total parenteral nutrition. 2 died a few hours after admission of severe sepsis due to enterocolitis before operation was possible. There were early postoperative complications in 11% of the 151 operations, mostly minor wound infections.(ABSTRACT TRUNCATED AT 250 WORDS)

[Is there justification for limited percutaneous surgical drainage in severe neonatal necrotizing enterocolitis in low birth weight premature infants?].

The usual treatment of complicated neonatal necrotizing enterocolitis (NEC) is resection of the necrotic bowel, lavage of the peritoneal cavity and diversion enterostomy. Low-birth-weight premature neonates with this condition are in special danger if general anesthesia and full exploratory surgery is contemplated. A relatively simple alternate procedure is percutaneous insertion under local anesthesia of a soft abdominal drain, most often in the right lower quadrant. The procedure is done in the neonatal intensive care unit without moving the whole set-up to the operating room. 4 such cases have been treated within the past year. 3 were discharged home as they did not require additional surgical treatment, not having developed intestinal stenosis or obstruction. 1 recovered from the acute episode, but succumbed to a severe intraventricular hemorrhage and respiratory failure 7 days after the procedure. Our limited but most gratifying experience, in addition to similar experience of others, encourages us to recommend this simple surgical approach in the very sick low-birth-weight premature with fulminant NEC.

"Pantaloon" phytobezoar: an unusual cause of intestinal obstruction associated with Meckel's diverticulum.

An unusual cause of small bowel obstruction associated with Meckel's diverticulum is described in two boys, aged 10 and 11 years. The causal factor was a phytobezoar lodged in the diverticulum in a Y-shaped "pantaloon" fashion.

The use of collagen-coated vicryl mesh for reconstruction of the canine cervical esophagus.

Collagen-coated vicryl mesh (CCVM) was used experimentally to patch partial and total segmental defects in the canine cervical esophagus. The esophageal healing process over a 6-month period with successful incorporation of the CCVM and esophageal wall regeneration are described. Twenty-two (91.7%) of 24 dogs survived the procedure without any evidence of esophageal stricture or dysphagia. CCVM has proved to be a suitable material for substitution of esophageal tissue in the canine model. Its clinical use in congenital and acquired esophageal lesions should be considered only pending further experimental laboratory work.

Laparoscopic and thoracoscopic surgery in children and adolescents: a 3-year experience.

Our initial experience over the last 3 years with laparoscopic and thoracoscopic surgery in children and adolescents is reported. Between September 1992 and August 1995, a total of 215 laparoscopic and thoracoscopic procedures were performed: 32 appendectomies for acute appendicitis, 10 cholecystectomies for symptomatic gallstones, 11 procedures for adnexal pathology, 6 laparoscopies in children with nonpalpable testes, 3 diagnostic laparoscopies, and 153 thoracoscopic sympathectomies in children suffering from primary palmar hyperhidrosis. The post-operative course was uneventful in all cases. In 2 children with acute appendicitis we converted to the open technique due to technical difficulties. We are encouraged by the results of our initial experience. There is no doubt that laparoscopic cholecystectomy, laparoscopic surgery of adnexal pathology, and thoracoscopic sympathectomy, because of their numerous benefits - shorter operative time, hospitalization, and convalescence as well as less postoperative pain and improved cosmetic results - are replacing the open techniques. We are not convinced as yet of the advantages of laparoscopic appendectomy in children; we are presently performing both laparoscopic and conventional techniques and studying the various parameters in order to reach a more definite conclusion. Various other endoscopic surgical procedures will be carefully considered in the near future.

Lymphangioma circumscriptum.

Two children with lymphangioma circumscriptum (LC), a rare cutaneous lymphangioma, are described. One was diagnosed antenatally as having a right axillary cystic hygroma; in addition, postnatally LC was noted on the right forearm. A few years later a mediastinal lymphangioma was diagnosed. This combination of LC associated with additional malformations of the lymphatic system has not been reported previously, and may point to a generalized developmental disorder of the lymphatic system. The pathophysiology of the lesion is discussed with a review of the pertinent literature. It appears that LC primarily affects the subcutaneous tissue in the form of cystic dilatation of lymphatic channels without systemic lymphatic communication. The skin lesions are probably secondary to the increased intraluminal pressure. The typical clinical appearance is manifested by multiple skin vesicles and lymphorrhea. The main treatment modality is surgical excision. Recurrences are not unusual and require re-excision.