F.M. Guttman

Splenic cysts: aspiration, sclerosis, or resection.

Percutaneous aspiration and tetracycline sclerosis is a safe but temporary therapy of large splenic cysts in children. Between 1985 and 1987, three girls with splenic cysts were seen. Their ages ranged from 5 to 14 years, and the cysts were from 8 to 16 cm in diameter. Despite their large size, all were asymptomatic and were discovered upon physical examination or ultrasound for unrelated conditions. All cysts were avascular by scan and had irregular crenated or smooth walls by ultrasound. Further investigation excluded infectious or parasitic causes. Each cyst was aspirated for diagnosis, and a pigtail catheter was inserted for drainage and sclerotherapy. All needle aspirations resulted in cyst collapse, but in one patient the pigtail catheter insertion was unsuccessful, and in the other two cases, multiple attempts of tetracycline sclerosis failed to obliterate the cysts. There were no other complications. Surgery for the recurrent splenic cysts was performed 3 months to 2 years following the percutaneous procedures. The two patients operated on with 3 months of aspiration underwent successful partial splenectomy and have normal splenic function by ultrasound scan, and absence of RBCs. The third patient had progression of the cystic disease throughout the spleen, and required splenectomy. Pathology confirmed multiseptate congenital mesothelial cysts in the first two patients and massive lymphangiomatosis in the third. In all three cases, percutaneous therapy was safe but did not result in long-term control. In one patient, the cystic disease progressed following sclerotherapy and may have influenced the need for complete splenectomy. Prior manipulation did not adversely affect the dissection and mobilization of the spleens.(ABSTRACT TRUNCATED AT 250 WORDS)

Appendicitis in children: Current therapeutic recommendations

Wound infection is the most common source of morbidity in appendicitis. Most recent pediatric series use protocols of preoperative antibiotics with aerobic and anaerobic coverage, intraoperative lavage, no peritoneal or wound drains, and continuation of antibiotics postoperatively with complicated appendicitis. There still remains controversy concerning skin closure and the duration of antibiotic therapy. We report the results of a prospective protocol followed over 2 years with 420 children. The protocol was designed to determine whether the skin could be closed primarily in all patients undergoing appendectomy. Preoperatively all patients received triple antibiotics (ampicillin, gentamicin, and clindamycin) that were continued postoperatively for two doses if there was a normal appendix or simple acute appendicitis, for at least 3 days with gangrenous appendicitis, and at least 5 days with perforated appendicitis. Antibiotics were continued if the patient remained febrile or had a white count greater than 10,000. No drains were used and the skin was closed primarily. The overall infectious complication rate was 1.0% (4/420). Among those with a normal appendix or simple acute appendicitis there were no infectious complications. Among those with gangrenous or perforated appendicitis there were 1.7% wound infections (2/117) and 1.7% intraabdominal abscesses (2/117). Duration of hospitalization was 2.1 days (range, 1 to 5 days) after simple acute appendicitis and 6.9 days (range, 3 to 40 days) after gangrenous or perforated appendicitis. These results set new standards in terms of wound management, infectious complications, and length of hospital stay.

Congenital adenomatoid malformation of the lung: current management and prognosis.

A retrospective 10-year review of congenital adenomatoid malformation (CAM) included 10 cases diagnosed in utero by ultrasound and 13 cases that presented postnatally. Two prenatally diagnosed cases were aborted because of associated lethal anomalies. All remaining patients underwent resection. Up to one third of all cases, whether diagnosed prenatally or postnatally, were asymptomatic. Resection is recommended to avoid respiratory distress, infection, or associated malignancy. There were 5 nonsurvivors, including 2 therapeutic abortions and 3 who died postoperatively. All had either polyhydramnios or ascites. All patients who died postoperatively had a respiratory arrest at birth and underwent immediate lobectomy. All died on the first day of life after a brief period and were found to have associated pulmonary hypoplasia. One had undergone a prenatal transthoracic cyst aspiration at 34 weeks gestation in an attempt to allow lung growth and prevent premature labor. Prenatal ultrasound findings suggestive of poor prognosis included polyhydramnios, ascites, mediastinal shift, and noncystic type III CAM. However, there is a spectrum of severity of CAM. The lesion can either regress and be asymptomatic at birth, or it can progress to cause either fetal death from hydrops or neonatal death caused by associated pulmonary hypoplasia. These findings should be considered in prenatal counseling for CAM.

Management of complex ovarian cysts presenting in the first year of life

With more frequent antenatal and postnatal diagnosis, the management of ovarian cysts has become somewhat controversial. Management protocols for simple ovarian cysts have been proposed. The purpose of this study was to establish a management protocol for complex ovarian cysts presenting antenatally and in the first year of life. We reviewed the records of nine infants who underwent surgical treatment for ovarian cysts over a 10-year period (1980 through 1989). Antenatal ultrasound performed between 24 and 34 weeks of gestation showed ovarian cysts in six infants. All six infants had complex cysts ranging in size from 3 to 10 cm on postnatal ultrasound. Exploration with oophorectomy or salpingooophorectomy was carried out at 2 days to 3 months of age for ovarian torsion. Ovarian cysts measuring up to 7 cm were diagnosed postnatally in three infants from 1 day to 7 months of age with ultrasound confirmation of complex, cystic intraabdominal masses. All patients underwent salpingooophorectomy, two for tuboovarian torsion and the third patient for a juvenile granulosa cell tumor. We recommend that all complex ovarian cysts, regardless of size, be surgically removed because they represent torsion, neoplasm, or alternate diagnoses, and removal can prevent possible complications.

Laparoscopic cholecystectomy : a treatment option for gallbladder disease in children

Laparoscopic cholecystectomy was performed safely in two children ages 7 and 15 years. Special considerations were required in the younger child because of the discrepancy between the length of the instruments and the size of the peritoneal cavity. Both children remained in hospital less than 24 hours and were able to resume normal diet and activity quickly. Pediatric surgeons with special training in laparoscopic surgery may want to use this treatment modality for children with biliary tract disease.

Recurrent intussusception: Safe use of hydrostatic enema

Recurrent intussusception (RI) occurs in 5% to 8% of patients with intussusception. The authors reviewed their 15-year experience to better define the management of RI. Among 258 patients, 28 (10.8%) had episodes of RI (37 episodes altogether). Twenty-two patients had a single recurrence, four had double recurrence, one had triple recurrence, and one had quadruple recurrence. RI and non-RI patients were compared with respect to symptoms. The incidence of vomiting and bloody stools was significantly lower in the RI group. Ten percent of RI patients had lead points. Thirty percent of RI episodes occurred within 24 hours, and 74% occurred within 6 months. The success rate of hydrostatic enema reduction for recurrent episodes was 62.8%, which is comparable to that for the initial episode (68.9%). Among patients with previous surgery, 36.3% of enemas were successful; after previous enema reduction, 75% were successful. There was no complication related to the enema. Enema reduction is safe and has a good success rate in cases of RI, even after previous surgical reduction.

Aortoesophageal fistula : congenital and acquired causes

Aortoesophageal fistula (AEF) is a rare but frequently lethal cause of upper gastrointestinal bleeding. This is a report of the authors experience with four cases--two from congenital and two from acquired causes. A review of the pediatric cases reported in the literature is included. The mortality from AEF is high, and can be decreased in two ways. AEF can be prevented by avoiding prolonged nasogastric intubation in patients with known vascular rings, and by promptly removing esophageal foreign bodies and promptly treating periesophageal abscesses. Once an AEF occurs, it presents with typical large-herald upper gastrointestinal bleeding of bright red blood, and cardiovascular collapse. Recognition of this pattern, with vigorous resuscitation and aggressive surgical management, will improve the survival rate.

Distal congenital esophageal stenosis associated with esophageal atresia

A series of six patients with congenital esophageal stenosis associated with esophageal atresia (EA) and distal tracheoesophageal fistula is presented. Three patients required only repeated dilatations, and have had good results. Two patients required limited resections of the distal esophagus, with excellent results. One patient died following a Heller myotomy. Tracheobronchial rests were present in the distal esophagus in the latter three patients. Diagnosis of congenital distal esophageal stenosis following repair of EA requires a high index of suspicion and a careful review of previous esophagrams. It is important to exclude anastomotic stricture and stenosis associated with gastroesophageal reflux. This requires barium esophagram, esophagoscopy with biopsy, and esophageal pH monitoring. Once a congenital basis for distal esophageal stenosis is suspected, management consists of dilatation by bouginage followed by balloon dilatation. Resection is reserved for persistent stenoses from tracheobronchial rests, which usually do not respond to dilatations.

Spontaneous deep vein thrombosis in childhood and adolescence

Deep vein thrombophlebitis (DVT), unrelated to intravenous catheters, is unusual in children. During the period between 1970 to 1984, 15 cases of spontaneous DVT were seen at The Montreal Childrens Hospital. The female to male ratio was 2:1. Age ranged from 10 to 17 years. Venogram was positive in the 14 patients in whom it was done. Significant factors were related to the DVT in 12 patients: oral contraceptives, pelvic fracture, Ewings sarcoma and chemotherapy, brain tumor, nephrotic syndrome, and ulcerative colitis (2, one of whom developed the DVT 3 months before the onset of gastrointestinal symptoms). The sites of thrombophlebitis encountered were left iliofemoral vein, right iliofemoral, right tibial, and left subclavian. Three patients developed pulmonary emboli, which were recurrent in two and required iliac vein ligation or inferior vena cava clipping. Treatment usually consisted of intravenous heparin followed by oral anticoagulants for 3 to 6 months. Two patients received streptokinase with good results. All the patients recovered. With a limited follow-up (6 months to 5 years), we have not seen any postphlebitic sequelae.

Diaphragmatic pacing in children with congenital central alveolar hypoventilation syndrome

BACKGROUNDnThe purposes of this study were to study congenital central alveolar hypoventilation syndrome (CCAHS), to report the experience at our institution with this disorder, and to describe the surgical technique of diaphragmatic pacing. Three patients are in our diaphragmatic pacing program. They all have CCAHS, documented by means of lack of respiratory drive to hypercapnia and normal peripheral nerve and muscle studies. Two patients have associated Hirschsprungs disease.nnnMETHODSnThe ages of patients at insertion of bilateral phrenic nerve pacemakers were 1, 2, and 5 years. A bilateral axillary thoracotomy in the third interspace was used in two patients, and anterior thoracotomy was used in one for insertion of electrodes on the phrenic nerves. Counterincisions in both flanks were used in all patients for insertion of the receivers in subcutaneous pockets.nnnRESULTSnIn all patients pacing was commenced within 1 week of the surgical procedure, because no primary pacemaker failures occurred. One patient has experienced no failure of the equipment and no infectious complications at 4 years. A second patient has had the pacemakers for 6 months without complications. The third patient underwent placement of bilateral pacemakers in 1984. In this patient a 10-year experience has resulted in subcutaneous implant failure on two separate occasions; also the presence of a staphylococcal empyema necessitated the temporary removal of one phrenic nerve electrode for 6 months, with successful reinsertion. All patients now receive mechanical assisted ventilation when sleeping and phrenic nerve pacing when awake.nnnCONCLUSIONSnPediatric surgeons should be aware of CCAHS because it may be treated with surgically implanted electrodes that allow for pacing of the diaphragm. The technique has an acceptable complication rate, and it can greatly decrease the impact of the disease on the lifestyle and activity of the patient. CCAHS also may be associated with Hirschsprungs disease.