The thoracoabdominal syndrome (TAS): a new X-linked dominant disorder.

We report on a family with a previously undescribed malformation syndrome. The syndrome is composed of diaphragmatic and ventral herniae, hypoplastic lung, and associated cardiac anomalies. The relative number of affected males and females and appreciably severer manifestations in males suggest an X-linked dominant inheritance.

Omental herniation. An unusual fetal complication of intrauterine transfusion.

Amniocenteses in an Rh-sensitized woman revealed the need for intrauterine transfusion to improve fetal status. Following delivery of the infant by cesarean section 7 weeks later, herniation of a segment of omentum through the anterior abdominal wall was noted. The injury was due to needle puncture during the intrauterine transfusion. The injury was repaired, and the infant recovered.

The aberrant cervical thymus. Embryology, Pathology, and clinical implications.

Cervical thymic anomalies are not as rare as previously suggested. Six cases were encountered over a relatively short period of time, prompting us to report them and emphasize the importance of this entity. Five of the six patients were children, two of them infants less than one year old. The occurrence of thymic remnants in the neck of young children is not surprising, considering the nature and behavior of the thymus at different stages of life. After a brief embryologic survey, the various types of cervical thymus (solitary ectopic, cystic, or partially arrested descent) and their pathogenesis are discussed. The rare occurrence of thyroid and parathyroid tissue within the mass of a large cervical thymic cyst is reported and evaluated. Cervical thymic lesions can either be symptomless or cause severe dyspnea and dysphagia, especially in the young infant. Accurate diagnosis and an intelligent surgical approach in the child with a cervical mass can avoid the obvious parental apprehension and lead to the correct treatment. Symptoms due to pressure on neighoring structures are promptly eliminated after excision, and prognosis is excellent.

Diverticula of Meckel's diverticulum.

Diverticula, or multiple sacculations, of a Meckel's diverticulum are a relatively unknown and undocumented pathologic entity. A case is reported, and another case recalled. In both cases, the sacculations were in the fundus. There was partial or complete absence of muscular layers in the saccular wall. A congenital origin of these muscular defects is more likely than is an origin from acquired hydrostatic pressure.

Congenital cysts of the head of the pancreas.

A very rare case of an infant with a huge multilocular congenital cyst of the head of the pancreas is presented. The symptomatology, diagnosis and surgical approach are discussed, while reviewing the few scattered reports in the English literature. Selective celiac arteriography is considered to be of definite aid in the differential diagnosis of cystic lesions of the head of the pancreas and has important implications on the choice of surgical procedure. We believe this to be the first case of a congenital cyst of the head of pancreas to be diagnosed accurately preoperatively by means of selective celiac and gastroduodenal angiography. Cystoduodenostomy proved to be a simple and effective procedure.

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.

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

After 20 years of experience with the "open" transaxillary approach, the authors are presently performing the thoracoscopic technique for upper thoracic sympathectomy in severe primary hyperhidrosis. During a period of 14 months, 23 operations were performed and 22 patients had immediate and permanent relief of palmar sweating. The immediate postoperative course was uneventful in all cases. Hospitalization was short, and all patients returned to school and full activity 3 to 5 days after operation. These initial results compare favorably to the "open" method and, pending further experience, are actually better in terms of less pain, early discharge, quicker return to normal activity, and a smaller, less conspicuous scar.

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.

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.

Surgical management of the cleft sternum.

Cleft sternum is a rare disturbance of unknown origin in the normal central midline fusion. Three cases of upper cleft sternum are reported here, one associated with a pericardium to the skin sinus tract. Surgical correction of this anomaly in the neonatal period is advised, whether the infant is symptomatic or not. Simple closure of the defect during the first month of life avoids the more complex reconstruction necessary in older children.

The laparoscopic approach to uterine adnexal torsion in childhood.

Seven girls with adnexal torsion (of varying degrees) were treated laparoscopically during a 6-month period. All had associated adnexal pathology. Six had simple follicular cysts (> 4 cm in diameter), and one had a 10-cm dermoid cyst. Detorsion with resection of the cyst and preservation of ovarian tissue was performed in all cases. No intraoperative or postoperative complications were encountered. Video-assisted minimally invasive surgery is suitable for the treatment of adnexal pathology in children; the hospitalization period is short, the cosmetic results are excellent, and the return to normal activity is rapid.

Tracheal rupture after blunt chest trauma in a child.

Blunt traumatic tracheal rupture is a life-threatening injury. The authors report on a 14-year-old boy who suffered such an injury in a road accident, underwent surgery immediately, and survived. The relevant literature is reviewed.

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.

Aberrant (ectopic) adrenocortical tissue along the spermatic cord.

Aberrant adrenocortical tissue along the spermatic cord is a far more common finding than has previously been reported. During a period of 12 consecutive months, 360 children, aged 13 days to 13 yr, underwent 386 operations for common inguinoscrotal pathology at the Soroka Medical Center. In 12 cases ectopic adreno-cortical tissue was found along the spermatic cord, an overall incidence of 3.8%. None was found in females. Considering the group operated upon for undescended testis, in which by nature of the procedure the spermatic cord was dissected throughout its length, the frequency of ectopic adrenal tissue found rose to 9.3%, the highest figure cited for this location. The nature of aberrant adrenocortical tissue is briefly discussed, including its embryology, histology, and clinical implications. It usually appears as an incidental finding during surgery. Its removal is indicated whenever found although no special attempt should be made to search for it.

Peritoneal encapsulation: a rare cause of bowel obstruction in children.

Two rare cases of peritoneal encapsulation causing partial intestinal obstruction are described. Both were operated on, and one of them had a "cocoonlike" appearance. The characteristics and differences of peritoneal encapsulation and abdominal cocoon are discussed.

Discordant Hirschsprung's disease in monozygotic twins.

Two pairs of twins are being reported where monozygocity was supported by the observed sharing of a single and common placenta and by ABO and HLA identity. Only one of each pair of twins was affected by long-segment Hirschsprung's disease; the other twins were entirely normal. Discordant Hirschsprung's disease in monozygotic twins is very rare. The etiology in such cases is influenced by genetic or by environmental intrauterine factors rather than by postnatal influences, as suggested elsewhere.

Familial idiopathic intussusception: a report of two families.

The authors report on two families with 5 out of 10 and two of two siblings who presented with idiopathic ileocolic intussusception. This may suggest hereditary predisposition as an etiologic factor.

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.

Granular cell tumor (myoblastoma) of the labia major: a rare benign tumor in childhood.

The clinical presentation, surgical findings, and histology of a granular cell tumor (myoblastoma) of the vulva in a 9-year-old girl are presented. Although rare, this benign lesion must be considered in the differential diagnosis of the labia major masses, such as Bartholin's duct cyst, lipoma, papilloma, hydradenoma, and fibroma.