Perforated appendicitis in children: is there a best treatment?

PURPOSE: This study was performed to provide outcome data for the development of evidenced-based management techniques for children with appendicitis in the authors' hospital. METHODS: This is a retrospective analysis of 1,196 consecutive children with appendicitis over a 5-year period (1996 to 2001) at a metropolitan hospital. RESULTS: The median age was 9 years (7 months to 18 years). The perforation rate was 38.9%, and the nonappendicitis rate was 5.6%. Predictors of perforation included age less than 8 years, Hispanic ethnicity, generalized abdominal tenderness, rebound tenderness, and increased number of bands. In perforated cases, the median length of stay was 5 days, and the complication rate was 13.5%. There was no difference in complication rates related to type or timing of antibiotics or related to the individual surgeon. There was no difference in infection rates related to type of wound management. CONCLUSIONS: Children with perforated appendicitis are treated effectively by a less expensive broad-spectrum antibiotic regimen, expeditious operation by open or laparoscopic technique, primary wound closure, and postoperative intravenous antibiotics until they are afebrile for 24 hours and have a white blood cell count of less than 12,000/mm3. This approach is to be used in our prospective, randomized analysis of children treated on or off a clinical pathway.

Chronic subcutaneous octreotide decreases gastrointestinal blood loss in blue rubber-bleb nevus syndrome.

BACKGROUND: A patient affected by blue rubber-bleb nevus syndrome had chronic gastrointestinal bleeding requiring weekly blood transfusions. Despite multiple surgical and endoscopic procedures to treat the venous malformations, the patient continued to bleed primarily from lesions in the small bowel. Therefore, this patient was treated with octreotide, a somatostatin analog known to decrease splanchnic blood flow and that is used for acute and chronic gastrointestinal bleeding. METHODS: Octreotide therapy, 5.7 microg/kg subcutaneously twice daily, was initiated, and the patient was followed up clinically. Complete blood counts, blood glucose concentration, pancreatic enzyme concentration, liver function tests, and growth hormone concentration were monitored during treatments. RESULTS: During the 4 weeks after initiation of octreotide therapy, hemoglobin concentration was maintained without the need for transfusions. Octreotide decreased the patient's monthly need for blood transfusion from 52 +/- 7 mL. kg-1. mo-1 of packed red blood cells to 23 +/- 7 mL. kg-1. mo-1. She had no detectable side effects or growth inhibition. Other medical interventions including -epsilonaminocaproic acid, nadolol, and total parenteral nutrition with bowel rest were not as effective as octreotide alone. CONCLUSION: Octreotide decreased the patient's need for blood transfusions. Possible mechanisms include altering blood flow to the gastrointestinal tract and direct effects on the venous malformations.

Early operation in children with pancreas transection.

BACKGROUND/PURPOSE: The preferred treatment for children with major pancreatic ductal injury remains controversial. This report compares our results using early operation with previously reported series using both operative and nonoperative management. METHODS: This is a retrospective analysis of all children with pancreatic transection identified at Children's Medical Center of Dallas, Texas, from 1995 through 1999. RESULTS: There were 11 children with pancreatic transection. There was a delay of 2.3 days before presentation to a hospital in 6. Transection was diagnosed within 12 hours of presentation in 5 children. In the other 6 there was a mean delay of 36 hours. Nine children underwent operation within 72 hours of injury. Two late presenters initially were treated nonoperatively, and both developed a pseudocyst. The length of hospital stay in patients undergoing early operation averaged 11 days. Early postoperative morbidity occurred in 4 children and late morbidity in 1. CONCLUSIONS: Major pancreatic injuries are uncommon in children, and the diagnosis often is delayed. A high index of suspicion and repeated computed tomography scans should lead to earlier diagnosis. When compared with nonoperative management, early pancreatic resection more expeditiously returns the child to good health and lessens the inconvenience and emotional stress associated with prolonged hospitalization.

Management of parapneumonic collections in infants and children.

BACKGROUND/PURPOSE: Video-assisted thoracoscopic surgery (VATS) has a recognized role in treatment of empyema thoracis. The purpose of this report is to show the value of initial VATS as the primary treatment of parapneumonic collections. METHODS: A retrospective review was done of 139 children who required surgical consultation for parapneumonic collections between January 1992 and July 1998. Management options were (M1) thoracentesis, chest tube drainage, or fibrinolytic therapy and delayed thoracotomy for unresolved collections; (M2) thoracentesis, chest tube drainage, fibrinolytic therapy with delayed VATS if the child remained ill; or (M3) primary VATS. Comparative data included age, duration of prehospital illness, oxygen requirements, white blood cell count, bacterial culture results, number of procedures performed per patient, duration of chest tube drainage, complications, and length of stay. Kruskal-Wallis 1-way analysis was used, with significance at P less than .05. RESULTS: A total of 60 children were treated by M1, 38 by M2, and 41 by M3. Age, duration of prehospital illness, oxygen requirements, white blood cell count, bacterial culture results, and complication rates were comparable. The median length of stay was 12 days for M1, 11 days for M2, and 7 days for M3, with M3 significantly shorter at P<.001. The number of procedures was a median of 2 in M1, 2 in M2, and 1 in M3, with M3 significantly fewer at P<.001. Duration of chest tube drainage was a median 5 days for M1 and 3 days for M2 and M3, with M1 significantly longer at P<.001. There were 9 thoracotomies in the M1 group, 3 in the M2 group, and none in the M3 group. One child in M3 required a second VATS. CONCLUSIONS: Primary VATS has significantly decreased the number of procedures, duration of chest tube drainage and length of stay for children with parapneumonic effusions. Primary VATS appears to be of value in management of bacterial pneumonia with effusion.

Posterior sagittal anorectoplasty in adults: secondary repair for persistent incontinence in patients with anorectal malformations.

PURPOSE: Posterior sagittal anorectoplasty, regarded as a standard surgical primary repair for anorectal malformations in infancy, was evaluated for effectiveness when performed as a secondary operation for establishing continence in the adult. The purpose of this review was to evaluate our results of performing posterior sagittal anorectoplasty in adult patients and to emphasize the extensive evaluation required to perform proper patient selection. METHOD: From January 1, 1992, to December 31, 1996, eight patients with Grade 3 incontinence underwent posterior sagittal anorectoplasty. The ages ranged from 13 to 40 (mean, 26) years. RESULTS: All patients had diverting stomas at the time of repair and all but one had restoration of intestinal continuity. Of eight patients who underwent posterior sagittal anorectoplasty, one failed secondary to rectal ischemia and retained a diverting stoma. Six patients had restoration of continuity. Five patients were continent and one had incontinence only to gas. DISCUSSION: We have established that posterior sagittal anorectoplasty can effectively be used to establish continence as a secondary procedure for a select group of adult patients.

Parathyroid carcinoma in a child.

Parathyroid carcinoma is a rare cause of hypercalcemia in children but should be considered in a child presenting with an extremely elevated serum calcium level. The authors report the fifth case of parathyroid carcinoma in a child less than 16 years of age.

Antegrade continent enema management of chronic fecal incontinence in children.

BACKGROUND/PURPOSE: Fecal incontinence impedes social and psychological development in children. Conventional bowel management with rectal enemas, medication, and biofeedback frequently will provide intervals free of fecal soiling sufficient for children to be socially continent. METHODS: This report details the improvement achieved by antegrade irrigation of the colon when conventional bowel management programs have failed to provide satisfactory intervals free of fecal soiling. Twenty-three children had conduits performed to administer antegrade continent enemas in the colon from December 1993 to May 1997. RESULTS: Twenty of the children were available for evaluation. One child was lost to follow-up, and two were noncompliant with the irrigation program. Fourteen of the 20 children are out of diapers. Four of the six wearing diapers, do so for urinary, not fecal incontinence. Two patients (10%) still require diapers for fecal incontinence. Parents were pleased with the efficacy of antegrade irrigation and the decrease in family stress with the elimination of fecal soiling. CONCLUSIONS: Ninety percent of the children available for follow-up benefited from antegrade irrigations of the colon. The antegrade irrigations were an improvement over conventional bowel management programs because a predictable interval free of soiling could be established. This allowed families to plan activities and improved the self-esteem of their children.

Radical excision of invasive thymoma with intracaval and intracardial extension: a successful case report.

A case of invasive thymoma with intracaval and intracardiac extension is reported. The use of cardiopulmonary bypass was necessary to achieve a radical excision of the tumor thus avoiding early death due to cardiovascular complications. This highly unusual mode of tumor presentation makes this particular case worth reporting.

Comparison of laparoscopic and open splenectomy in children with hematologic disorders.

OBJECTIVE: To compare laparoscopic and traditional open splenectomy in children with nonmalignant hematologic disorders. STUDY DESIGN: Retrospective review of 36 consecutive nonrandomized splenectomies (16 laparoscopic and 20 open) performed for hematologic disorders at a single pediatric institution during the past 3 years. The two-sided Mann-Whitney U test for non-parametric variables was used for statistical analysis. RESULTS: An open procedure was performed on 20 patients (mean age, 9.7 years), five of whom had a concomitant cholecystectomy. A laparoscopic splenectomy was performed on 16 children (mean age, 10.3 years), seven of whom had a concomitant cholecystectomy. The mean anesthesia and operative times were longer in the laparoscopic than in the open group (p < 0.001). However, the mean number of hours of postoperative analgesia was less in the laparoscopic group (p < 0.005). Patients who had laparoscopic splenectomy were also discharged home earlier (p < 0.01) and resumed a regular diet sooner. Mean operating room charges were higher in the laparoscopic group (p < 0.001), but total hospitalization costs were not significantly different. Postoperative complication rates were similar. The hematologic response was comparable. CONCLUSIONS: laparoscopic splenectomy is feasible and safe in children with hematologic disorders. Although it currently requires more operative time than the open approach, it is superior with regard to duration of postoperative analgesia, duration of hospital stay, and recovery of bowel function.

Effect of dexamethasone on rat plasma platelet activating factor acetylhydrolase during the perinatal period.

It has been previously reported that the administration of dexamethasone (DEX) to adult rats increases the activity of plasma platelet-activating factor acetylhydrolase (PAF-AH) and prevents the development of intestinal necrosis caused by platelet activating factor (PAF) injection. In this report, we examined the effect of DEX administration on plasma PAF-AH activity during the perinatal period. Timed-pregnant rats received DEX (0.2-1.0 mg/kg/d) or normal saline (controls) on days 16-18 (early group) or days 18-20 (late group) of gestation. Maternal plasma PAF-AH activity was lower in late gestation than in postpartum period (P < 0.001). Fetal and neonatal plasma PAF-AH activity was higher than maternal values (P < 0.05). No changes of PAF-AH activity were seen in maternal, fetal or neonatal plasma after prenatal DEX administration at the aforementioned doses. A higher dose of DEX (1.3 mg/kg/d x 4d) or cortisone (200 mg/kg/d) produced an elevation of maternal plasma PAF-AH activity (DEX 79.2+/-3.0, cortisone 70.5+/-1.9 vs. controls 49.4+/-2.3 nmol/min/ml, P < 0.01), but resulted in a high fetal mortality. Treatment of newborn rats with DEX (0.5 mg/kg/d) on days 1-3 after birth, increased plasma PAF-AH activity on day 4 (DEX 292+/-5 versus controls 140+/-9 nmol/min/ml, P < 0.001) and day 6 (DEX 302+/-12 versus controls 136+/-6 nmol/min/ml, P < 0.001). Postnatal administration of DEX increases the plasma PAF-AH activity in the rat. Only high doses of prenatal corticosteroids that cause fetal death can elevate maternal plasma PAF-AH activity.

Laparoscopic Nissen fundoplication in the infant.

Twenty-five children weighing less than 8 kg underwent laparoscopic Nissen fundoplication for treatment of refractory gastroesophageal reflux (GER) between January 1993 and April 1995. Mean patient age was 6 months and weight was 5.1 kg. Mean operative time was 114 min, including gastrostomy in 20 patients. Nineteen patients are alive without evidence of GER with a mean follow-up of 359 days. Perioperative complications were unusual and mild. There were 6 late deaths due to progression of underlying disease and not attributable to the procedure. Laparoscopic Nissen fundoplication provides effective antireflux protection to very small children with acceptable morbidity and mortality through short-term follow-up.

Laparoscopic splenectomy in childhood hematologic disorders.

Laparoscopic splenectomy is a new technique that is being utilized in patients with a variety of mostly hematologic disorders. Its application in children has not been extensively documented. Between January 1994 and February 1995, 11 children less than 15 years of age underwent elective laparoscopic splenectomy. Data collected from this treatment group were compared to that from the ten most recent open splenectomy patients with comparable hematologic disorders. All procedures in both groups were successful in relief of symptoms, increase in platelet count, and/or increase in hematocrit. Operative times averaged 147 mm in the laparoscopic group, compared to 112 mm in the open group. Estimated blood loss was 32 ml in the laparoscopic group and 86 ml in the open group. Days to laparoscopic patient discharge were 3.6, compared to 5.3 days in the open group. There were no wound complications or need for perioperative platelet transfusions in the laparoscopic patients. Patient response has been uniformly positive in the laparoscopic group. Reusable access trocars are utilized for two of the four working ports. Stapling devices and special tissue morselizers are not required. There are no additional operating room or surgeons fees incurred in the laparoscopic procedures. This series demonstrates that laparoscopic splenectomy is a safe, cost-efficient alternative to open splenectomy in children with a variety of hematologic disorders.

Outcome of partial splenectomy for type I Gaucher disease.

Partial splenectomy was introduced to achieve the benefits of splenectomy and to avoid the risk of overwhelming infection in patients with symptomatic Gaucher disease. We observed regrowth of the splenic remnant, reemergence of preoperative symptoms, and new bone involvement among most of our patients who had undergone partial splenectomy. Enzyme replacement therapy has markedly limited indications for splenectomy, partial or total, for Gaucher disease.

Congenital and acquired aneurysmal disease.

Finding an aneurysm in a child is a rare event that should prompt careful evaluation for a secondary disease or a history of trauma or serious infection. Although congenital and idiopathic aneurysms do occur, almost all aneurysms seen in children are acquired. The majority of true aneurysms (including mycotic aneurysms) occur in the aorta, with the primary branches of the aorta (such as the renal or iliac arteries) the next most common site. Most false aneurysms are caused by trauma and usually present in the extremities. Treatment is surgical resection and vascular reconstruction except in patients with arteritis and small stable aneurysms.

Development of tolerance to class II-mismatched renal transplants after a short course of cyclosporine therapy in miniature swine.

Our laboratory has reported previously spontaneous acceptance of class II-matched, single haplotype (but not 2 haplotype), class I-mismatched renal allografts in miniature swine. All class II-mismatched animals rejected acutely regardless of class I matching. We have also demonstrated recently that a short course of high dose (10 mg/kg/day for 12 days) CsA uniformly induces donor-specific tolerance to 2-haplotype, class I-mismatched renal allografts. The survival of 2-haplotype, fully MHC mismatched renal allografts was prolonged by the same treatment, but tolerance was not induced, as all animals rejected eventually. We have now tested this short course of immunosuppressive therapy for its effect on renal allografts mismatched selectively for 2 haplotypes at class II. We have observed long-term graft survival in 5 of 7 animals under these conditions. Each of the 5 acceptor animals was demonstrated to be specifically tolerant by its response either to donor-matched skin grafts or to a second donor-matched kidney transplant without further immunosuppression. These data suggest the existence of a common pathway for induction of specific transplantation tolerance to MHC antigens when these antigens are recognized on vascular endothelium under conditions of altered cytokine production. They also suggest that tolerance induction under these conditions requires matching for either class I or class II antigens, which may have implications for the mechanism by which peripheral tolerance is induced, as well as practical implications for the extension of these results to potential clinical practice.

Retransplantation in miniature swine. Lack of a requirement for graft adaptation for maintenance of specific renal allograft tolerance.

In miniature swine, one-haplotype class I disparate renal allografts are accepted without exogenous immunosuppression by approximately 35% of recipients. Alternatively, transplants bearing a two-haplotype class I mismatch are always rejected acutely. However, long-term acceptance in the latter animals can be achieved uniformly with a 12-day course of cyclosporine. In vitro studies of recipient cell-mediated lymphocytotoxicity responses have shown donor-specific cytotoxic T lymphocyte clones in tolerant animals, suggesting that tolerance may be a local phenomenon or a central phenomenon activated in the milieu of the graft. Six animals were retransplanted with kidneys MHC-matched to their original allograft to determine whether (1) tolerance is a central phenomenon; (2) host tolerance can be broken with a fresh challenge of donor antigen and antigen-presenting cells; and (3) graft adaptation is required for maintenance of tolerance. Four of the retransplanted animals had been spontaneous acceptors of one-haplotype class I-disparate grafts and two had been rendered tolerant to two-haplotype class I-mismatched kidneys with CsA induction. All six explanted allografts showed no histological evidence of rejection and all six retransplants were accepted without exogenous immunosuppression. These findings suggest that in miniature swine tolerance of class I-disparate kidneys is a stable, centrally mediated phenomenon that cannot be broken with a challenge of fresh donor antigen and donor-type APCs. Furthermore, successful retransplantation without immunosuppression in animals receiving CsA induction therapy for their first transplant suggests that graft adaptation is not necessary for the maintenance of tolerance.

Bone marrow transplantation in miniature swine: IV. Development of myeloablative regimens that allow engraftment across major histocompatibility barriers.

Studies of the myeloablative regimens capable of permitting successful BMT across MHC barriers in miniature swine have been performed. To minimize graft-versus-host disease (GVHD), engraftment was studied in the F1-->P combination (i.e., MHC homozygous ["parental"] swine receiving bone marrow from one-haplotype matched MHC heterozygous ["F1"] donors). Animals given total body irradiation (TBI) up to 1100 cGy, 10 cGy/min, in a single dose failed to engraft. Increasing the dose rate led to unacceptable extramedullary toxicity without improving engraftment. Eleven different fractionated TBI regimens were tested in this F1-->parent model. At all of the dose rates tested, a total dose of less than 1000 cGy was insufficient for engraftment, and a total dose of 1400 cGy led to unacceptable toxicity. Between these extremes, a window was defined in which engraftment could be obtained without unacceptable extramedullary toxicity utilizing 2 equally divided fractions of TBI delivered 24 hr apart. The addition of 50 mg/kg cyclophosphamide i.v. to fractionated TBI (1150 cGy total dose [500 + 650]) also permitted engraftment, with decreased incidence of interstitial pneumonitis as compared to fractionated TBI (1300 cGy total dose [650 x 2]). Both of these regimens were also confirmed to permit engraftment between heterozygous donors and recipients sharing a single common haplotype ("F1-->F1"). The regimen of 1300 cGy (650 x 2) also permitted engraftment in completely MHC mismatched BMT, but with subsequent death from GVHD. These studies of the myeloablative regimens permitting engraftment across defined MHC barriers in miniature swine provide a basis for further studies of allogenic BMT and GVHD in this large animal preclinical model.

Management of pediatric postoperative chylothorax.

Questions persist about the management of postoperative chylothorax in infants and children. Our experience with postoperative chylothorax over the most recent decade (1980 to 1990) has been reviewed. The type and amount of drainage, data from cardiac catheterization and echocardiography, operative decisions and details, and eventual outcomes have been cataloged. All patients were initially treated with total gut rest, with operation reserved for unabated drainage. Chylothorax developed postoperatively in 15 infants and 11 children (18 with a cardiac procedure and 8 with a noncardiac procedure). The average age was 3.1 years. Spontaneous cessation and cure occurred in 19 (73.1%) of these 26 patients, with an average drainage duration of 11.9 days (range, 4 to 30 days). Those for whom operation was chosen drained preoperatively for an average of 29.2 days (range, 25 to 40 days). There were no deaths in either group. Complications were lymphopenia (2 patients) and fungal sepsis (1 patient). The amount of drainage per day was not significantly different between patients treated operatively and those treated nonoperatively. Failure of nonoperative management was associated with venous hypertension from increased right-sided cardiac pressures or central venous thrombosis (p < 0.05, Fisher's exact test). Presumably this increased pressure is transmitted to the lymphatic system. These patients should be identified early and considered for thoracic duct suture or pleuroperitoneal shunting.

Primary (retractile) mesenteritis in a child.

We report a case of primary retractile mesenteritis presenting as acute abdominal pain requiring surgery in a 3-year-old boy. At laparotomy, a hemoperitoneum was discovered and the diagnosis of primary mesenteritis was made with the aid of frozen section biopsy of the small bowel mesentery. No resection was necessary, and he made an uneventful recovery and remains well on follow-up.