A single-institution review of portosystemic shunts in children: an ongoing discussion.

PURPOSE: Review the safety and long-term success with portosystemic shunts in children at a single institution. METHODS: An IRB-approved, retrospective chart review of all children ages 19 and undergoing surgical portosystemic shunt from January 1990-September 2008. RESULTS: Ten patients were identified, 8 females and 2 males, with a mean age of 15 years (range 5-19 years). Primary diagnoses were congenital hepatic fibrosis (5), hepatic vein thrombosis (2), portal vein thrombosis (2), and cystic fibrosis (1). Primary indications were repeated variceal bleeding (6), symptomatic hypersplenism (2), and significant liver dysfunction (2). Procedures performed were distal splenorenal bypass (4), side-to-side portocaval shunt (3), proximal splenorenal shunt (2), and an interposition H-graft portocaval shunt (1). There was no perioperative mortality and only minor morbidity. Seventy percent of patients had improvement of their symptoms. Eighty percent of shunts remained patent. Two were occluded at a median follow-up of 50 months (range 0.5-13.16 years). Two patients underwent subsequent liver transplantation. Two patients died at 0.5 and 12.8 years postoperatively, one from multisystem failure with cystic fibrosis and one from post-operative transplant complications. CONCLUSIONS: The need for portosystemic shunts in children is rare. However, in the era of liver transplantation, portosystemic shunts in selected patients with well-preserved liver function remains important. We conclude that portosystemic shunts are safe and efficacious in the control of variceal hemorrhage and symptoms related to hypersplenism.

Management of chronic pancreatitis in the pediatric patient: endoscopic retrograde cholangiopancreatography vs operative therapy.

PURPOSE: The management of chronic pancreatitis (CP) in children is challenging. We compare endoscopic retrograde cholangiopancreatography (ERCP) to operative therapy (OR). METHODS: The study involved review of patients younger than 18 years with CP who underwent ERCP or OR from 1973 to 2007. Follow-up was complete in 95% of patients (median, 6 years; range, 1-23 years). RESULTS: We identified 37 children with CP; 25 (68%) were managed by OR with 20 of these previously failing ERCP. Twelve (32%) were managed by ERCP alone. Mean follow-up was longer in the OR group (5.1 vs 2.1 years; P = .02). Patients with idiopathic pancreatitis (58% vs 13%; P = .04) and patients with a later onset of pancreatitis (12.0 vs 7.4 years; P = .002) were more likely to be managed with ERCP alone. The patients who underwent OR had a lower rate of recurrent pancreatitis (39% vs 75%; P < .0001), although this did not correlate to fewer hospitalizations or less narcotic use compared to ERCP alone. When patients who failed ERCP and progressed to OR were included in the ERCP alone group, ERCP was worse in recurrence (90% vs 39%; P < .0001) and rate of hospitalization (55% vs 33%; P = .04) compared to OR. CONCLUSION: Patients with CP managed by OR have a lower rate of recurrent pancreatitis and hospitalization compared to ERCP.

Surgical management of intestinal malrotation in adults: comparative results for open and laparoscopic Ladd procedures.

BACKGROUND: This study aimed to characterize the clinical features of intestinal malrotation in adults, and to compare the results for the open and laparoscopic Ladd procedures. METHODS: Between 1984 and 2003, 21 adult patients with a mean age of 36 years (range, 14-89 years) were surgically treated for intestinal malrotation. The clinical data collected included age, gender, presenting symptoms, diagnostic tests, type of operation, operative time, narcotic requirement, time to oral intake, length of hospital stay, and outcome. The groups (open vs laparoscopic) were comparatively analyzed using two-sample t-tests and Wilcoxon rank sum tests. RESULTS: The two groups were similar in terms of age, clinical presentation, and diagnostic tests performed. The most common presenting symptoms were chronic abdominal pain, nausea, and repeated vomiting. Upper gastrointestinal barium studies (UGI/SBFT) were diagnostic for all patients with malrotation as compared with computed tomography (CT) scanning, which was falsely negative in 25% of patients. A total of 21 patients underwent the Ladd procedure, either open (n = 10) or laparoscopic (n = 11). Three laparoscopic procedures were converted to open. Overall, the laparoscopic group resumed oral intake earlier than the open group (1.8 vs 2.7 days; p = 0.092), had a shorter hospital stay (4.0 vs. 6.1 days; p = 0.050), and required less intravenous narcotics on postoperative day 1 (4.9 vs 48.5 mg; p = 0.002). The laparoscopic group underwent a longer operation (194 vs 143 min; p = 0.053). Sixteen of eighteen patients available for follow-up reported complete resolution of symptoms, 2 felt greatly improved. No patient required a second operation related to volvulus or recurrent symptoms. CONCLUSIONS: The laparoscopic Ladd procedure is feasible, safe, and as effective as the standard open Ladd procedure for the treatment of adults who have intestinal malrotation without midgut volvulus. Patients also benefit from this minimally invasive approach, as manifested by an earlier oral intake, a decreased need for intravenous narcotics, and an earlier discharge from the hospital.

Intraoperative electron irradiation in the management of pediatric malignancies.

BACKGROUND: External beam irradiation (PBRT), especially in children, is limited by the radiosensitivity of normal tissues. Local control remains a problem in abdominopelvic childhood malignancies. Intraoperative electron irradiation (IOERT) has the potential to increase the dose to the tumor, thereby improving local control, without increasing treatment morbidity. METHODS: Between February 1983 and October 1990, 11 children received IOERT as part of a multidisciplinary treatment program for locally advanced primary or recurrent abdominopelvic malignancies. The 7 boys and 4 girls ranged in age from 2-18 years. Tumor histologies included four neuroblastomas, two desmoid tumors, and one each of the following: embryonal rhabdomyosarcoma, synovial cell sarcoma, neurofibrosarcoma, malignant fibrous histiocytoma, and paraganglioma. Single radiation doses of 10-25 grays were delivered using 6-15-megaelectron volt electron beams to 1-5 IOERT fields. All patients also underwent EBRT and six received chemotherapy. RESULTS: Eight patients (73%) were alive and disease free at a median follow-up of 99 months (range, 37-126 months). All eight patients who underwent gross total resection were locally controlled. Three patients required surgical intervention for IOERT-related complications and two patients developed neuropathies. CONCLUSIONS: IOERT as part of a multidisciplinary treatment approach in patients with locally advanced pediatric malignancies appears to enhance local control in those patients in whom a gross total resection is possible. The long term survival rate was encouraging. Further study, with a larger number of patients, appears warranted to more carefully delineate the efficacy and tolerance of IOERT in the pediatric population.

Abdominal pain in infants and children.

The challenges of childhood abdominal pain are to treat the majority of children with self-limited conditions of obscure but benign causes and to identify the child with a rare, life-threatening cause of pain. The diagnostic algorithm of abdominal pain during childhood is best compartmentalized into three groups: infants, preschoolers, and school-age children. In infants, bilious vomiting heralds a life-threatening or surgically indicated disorder. Most nonsurgical conditions are related to gastroenteritis, constipation, and reflux. The majority of children with abdominal pain experience spontaneous resolution of their symptoms without specific management. For every 15 school-age children with abdominal pain, 1, at most, will have a serious condition such as appendicitis. Parents and children appreciate a careful appraisal of the symptoms, and the physician should listen to their concerns and perform a thorough examination. Laboratory or radiologic studies are rarely indicated.

Familial recurrence of tracheoesophageal fistula and associated malformations.

Tracheoesophageal fistula (TEF) may occur as an isolated malformation or together with other malformations. To determine the recurrence risk of TEF or associated malformations in children and sibs, and to determine the frequency of associated malformations in index patients, we reviewed the Mayo Clinic records of 204 patients with TEF. Also, questionnaires were sent to patients or relatives. Questions were designed to determine whether the patient and relatives had TEF and/or related organ system (including VACTERL) malformations. The VACTERL association is a disorder characterized by 3 or more of the following: vertebral, anal, cardiac, renal, or radial anomalies, and TEF. One hundred twenty-eight families returned a completed questionnaire, and 140 index patients were ascertained based on complete medical records, questionnaire, and/or autopsy. Forty-one (29.3%) of 140 index patients had TEF with one other VACTERL malformation, and twenty-four (17.1%) of 140 index patients had TEF with at least two other VACTERL malformations. Of the 347 sibs of index patients, 5 (1.4%) had one VACTERL malformation each, including 1 sib with esophageal atresia (EA) without TEF. Of the 41 children of index patients, 1 (2.4%) had TEF plus two other VACTERL malformations; another had one non-TEF VACTERL malformation. From our study, the largest reported population of TEF patients to date, we conclude that: 1) nearly half (46%) of patients with tracheoesophageal fistula will exhibit other VACTERL malformations; 2) the recurrence risk for individuals with TEF to have affected children is 2-3%; and 3) there is an increased risk to relatives of TEF patients to exhibit other VACTERL malformations.

Predictive factors of the outcome of traumatic cervical spine fracture in children.

The uncommon traumatic cervical spine fractures and dislocations were studied in 227 consecutively treated children (1 to 17 years of age). Lower cervical spine injuries (C-3 to C-7) affected 73% of our patients. However, among the 38 patients younger than 8 years of age, 87% had an injury at the C-3 level or higher (P < .0001). There were 19 fatalities (8.4%), all of which were associated with injuries at the C-4 level or higher. Of the 11 patients with atlanto-axial fracture or dislocation, all died soon after the injury. All had an unstable fracture and cord transection that resulted in cardiorespiratory collapse. Injuries at the vertebral levels C-1, C-2, C-3, and C-4 were associated with fatality rates of 17%, 9%, 4.3%, and 3.7%, respectively. No fatalities were encountered among patients with lesions lower than C-4. The authors conclude that the younger the age, the higher the cervical spine injury, and that the fatality rate correlates directly with the level of cervical spine fracture.

Surgical management of Meckel's diverticulum. An epidemiologic, population-based study.

OBJECTIVE: The authors determined whether Meckel's diverticulum, discovered incidentally at operation, should be removed. SUMMARY BACKGROUND DATA: It is not clear from the medical literature whether the risk of an incidental Meckel's diverticulectomy is greater than the risk of leaving the diverticulum in place. METHODS: The authors used the medical experience of Olmsted County, Minnesota residents for the period 1950 to 1992 to answer the question. RESULTS: During the period, 58 residents developed Meckel's complications that required diverticulectomies. The incidence of complications was 87 per 100,000 person-years, and the lifetime risk (to 80 years of age) of developing them was 6.4%. The risks were similar throughout the period and at all ages of life, but were greater among men (124 per 100,000 person-years) than women (50 per 100,000 person-years, p < 0.05). Diverticulectomies for complications carried an operative mortality and morbidity of 2% and 12% and a cumulative risk of long-term postoperative complications of 7%, whereas incidental diverticulectomies done in 87 residents during the period carried corresponding rates of only 1%, 2%, and 2%, respectively. CONCLUSIONS: Meckel's diverticula discovered incidentally at operation should be removed for most patients, regardless of age.

Papillary carcinoma of the thyroid in children.

Papillary carcinoma of the thyroid is the most common thyroid cancer in children. It usually occurs in the teenage female and presents as a 3-cm mass within the thyroid itself. Although the tumor is well differentiated, almost 80% of all children will present with local lymph node metastasis. Despite this metastasis, most children have a life expectancy that parallels those of their peers for 30 years. Treatment for well-differentiated papillary carcinoma includes surgical excision of all cancerous tissue and removal of the affected lymph nodes. Radioablation of residual disease has been effective.

Medullary thyroid carcinoma in children.

Medullary cancer of the thyroid (MCT) in children, although uncommon, represents not only a most intriguing problem but also one that is a model of early cancer detection that results in cure. MCT in children is usually a part of the multiple endocrine neoplasia (MEN) syndrome (MCT, pheochromocytoma, and hyperparathyroidism) and is detected by screening studies. MCT arises from C cells that secrete calcitonin, with C-cell hyperplasia representing the precancerous state. Both are diagnosed by measuring calcitonin levels both basally and after stimulation with pentagastrin. Screening studies should begin shortly after birth in infants at risk for MEN IIb and by age 1 year in children at risk for MEN IIa. Application of this approach should result in near elimination of the spread of cancer in children with MCT.

Diagnostic laparoscopy and laparoscopic equipment.

The ability to perform laparoscopic procedures in children is due to the remarkable advances in endoscopic technology and laparoscopic equipment. Although laparoscopy was first reported in 1901, poor exposure and inadequate visualization hampered advances in this area until fiberoptic technology was developed in the 1960s. Recently, the improved endoscope was combined with a xenon light source and computer chip camera to provide the visual images necessary for laparoscopic surgery. This article discusses the operative technique for creation of the pneumoperitoneum and the equipment necessary for the procedures, and reviews the complications from diagnostic laparoscopy. Further advances in technology surely will allow more procedures to be performed endoscopically.

Laparoscopy and thoracostomy in children.

The laparoscopic and thoracoscopic revolution is sweeping through pediatric surgery. The current literature has detailed numerous reports of laparoscopic approaches to traditional operations. The most commonly performed and currently most accepted operations are diagnostic laparoscopy, gynecologic laparoscopy, and laparoscopic cholecystectomy and appendectomy. Few, if any, of these procedures have been subjected to a prospective, randomized trial, but initial reports indicate improved postoperative outcome by decreasing hospital stay and an earlier return to normal activities. Thoracoscopic lung biopsy, tumor excision, and treatment of empyema are well-established procedures. Other operations soon to be introduced are laparoscopic fundoplication, laparoscopic-aided intestinal surgery, thoracoscopic excision of mediastinal masses, spinal surgery, and chest wall surgery. The early enthusiasm for minimally invasive surgery must be tempered by an appreciation for its limitations and complications. How surgeons handle this new innovation will determine whether the promise of laparoscopy is another step towards eliminating the negative factors that have made surgery such a dichotomy of fear and hope.

Laparoscopic cholecystectomy in children: initial experience and recommendations.

Our initial experience with laparoscopic cholecystectomy in children and the modification of techniques required for pediatric surgical patients is reported. Six children aged 6 to 17 years underwent laparoscopic cholecystectomy for symptomatic cholelithiasis. All patients had biliary colic and did not require concomitant intraabdominal surgical procedures. Average operative time was 89 minutes; none were converted to open cholecystectomy. One patient returned home the evening of surgery, two patients were discharged the following morning, and two patients were discharged 2 days following the procedure because of their distance from home. There were no complications. All patients have remained asymptomatic on follow-up of 1 to 5 months. Based on this initial experience, several changes in the technique as reported in adults are recommended. Due to the softness and laxity of the anterior abdominal wall, an infraumbilical incision for the 10-mm camera trocar is more cosmetically acceptable and just as efficient. There is a smaller intraabdominal space with which to work, tempting one to overinflate the abdomen for better visualization. Care should be taken to ensure that the intraabdominal pressure does not exceed 15 mm Hg despite the small volumes required to do so. Placement of the second epigastric incision is more inferior and lateral than the standard recommendation for adults. The cystic duct must be controlled as early as possible in the operative course to prevent egress of stones from the gallbladder into the common duct. In one patient, these migrating stones were milked back into the gallbladder and a second clip placed.

Surgical therapy and long-term follow-up of childhood hereditary pancreatitis.

Treatment and a 15-year follow-up survey of 42 patients with hereditary pancreatitis (HP) were compared with 28 patients with idiopathic recurrent pancreatitis (RP) of childhood. There was no difference between the two groups except for pancreatic ductal dilatation and stones in patients with HP. Longitudinal pancreaticojejunostomy (20) or resection and drainage procedures (7) were more commonly required in patients with HP than RP (55% v 14%). There was no surgical mortality. Postoperatively, immediate and complete relief of symptoms was obtained in 43% of patients with HP and 25% of patients with RP. In the remainder, recurrent attacks of pancreatitis abated over 2 years such that 81% of the surgical patients were in good or excellent health. Occasional symptoms persisted in 52% of HP patients and 25% of RP patients. Of the 20 patients with HP or RP undergoing longitudinal pancreaticojejunostomy extending from the head to the tail, 75% were symptom-free on follow-up. However, 3 of 6 patients with poor results had also undergone this procedure. At long-term follow-up of patients who did not undergo operation, 75% of HP patients and 90% of RP patients reported excellent or good health despite the persistent symptoms in 68% and 42%, respectively. Surgery for childhood HP is dependent on the complications present. Longitudinal pancreaticojejunostomy is beneficial for ductal dilatation and associated pseudocysts or pancreatic ascites. The performance of this procedure in the absence of consistent pancreatic duct dilatation will give poor results. Patients without ductal dilatation and the majority of patients with RP may eventually lead near normal lives without resorting to surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

Rhabdomyolysis and renal failure following a wolf attack: case report.

Trauma-induced rhabdomyolysis and myoglobinuric renal failure are well recognized. The mechanism of trauma in reported cases has been burns, crush injuries, or compression syndromes. We report a case of myoglobinuric renal failure in a 5-year-old boy who suffered primarily lacerations and penetrating wounds from a wolf attack. We are unaware of any other reports of nonvenomous bites or penetrating trauma causing rhabdomyolysis and myoglobinuric renal failure. Early suspicion, diagnosis, and treatment of this condition result in an excellent prognosis. Pathogenesis, treatment, and a review of the literature are presented.

Pathogenic Escherichia coli: a new etiology for acute ileitis in children.

Acute ileitis in children is frequently diagnosed at the time of laparotomy for acute abdominal pain and is usually due to inflammatory bowel disease, Yersinia, or Campylobacter infections. We report a case in which a pathogenic strain of Escherichia coli was responsible for the disease. The diagnosis, microbiology, and epidemiology are discussed.

The emerging pattern of pediatric day-care surgery.

The utilization of short stay surgical facilities is increasing and indications for day-care surgery for children are becoming more diverse. These trends were observed in a review of day-care surgery performed at British Columbia Children's Hospital during the years 1982 to 1986. During 1984 and 1985, 688 hernia repairs were undertaken, as were 76 anorectal procedures such as anal fistulotomy, drainage of abscesses, etc. In addition, 127 orchidopexies were performed, which indicated a 40% increase when compared with earlier years. Further procedures included the excision of 38 branchial cleft anomalies and 17 salivary gland lesions, as well as the performance of 95 tracheobronchial endoscopies including the removal of 25 foreign bodies. Head and neck and endoscopic procedures now represent 21% of all general surgery day care cases, and have increased by 50% since 1982. Thirty-six children (1.6%) initially brought in for day care surgery required actual admission to the hospital for such reasons as (1) the procedure being more complex than initially anticipated (15 patients); (2) hemorrhage (5 patients); or (3) postanesthetic concern (11 patients). Two thirds of the anesthetic complications were in infants less than 3 months of age. A special subgroup of high-risk infants were identified--the previous premature less than 52 weeks conceptual age. In our opinion, infants who are less than 3 months of age, especially if born prematurely, should be admitted to the hospital for surgery.

Emergency management of choledochal cysts in adult patients.

Congenital cystic dilation of the biliary tree is rarely considered as cause of cholangitis in the adult patient. Emergency operation in seven adults with unsuspected choledochal cysts resulted in reoperation in all seven. Each patient presented with right upper quadrant pain, a mass, and cholangitis or jaundice. Abdominal ultrasonography incorrectly identified the cyst as a dilated gallbladder in three of the patients. All initial emergency drainage procedures required subsequent modification to cyst excision and Roux-Y reconstruction. From review of the experience presented and the literature to date, we recommend that otherwise stable patients be managed nonsurgically and undergo endoscopic retrograde cholangiopancreatography or percutaneous transhepatic cholangiography to plan primary single-stage excisional operation. Patients requiring emergency decompression should have cholecystostomy or choledochostomy for good control without compromising subsequent operation. At the time of excision, a technical consideration not previously reported is the presence of small daughter cysts in Calot's triangle which must be distinguished from the hepatic bile ducts. The surgical literature has frequently addressed the problems of elective surgery for choledochal cysts; however, emergency complications requiring urgent operative intervention are seldom addressed. We believe emergency intervention should correct the urgent complication without compromising the definitive surgical treatment.

Typhlitis: selective surgical management.

Typhlitis is a neutropenic enterocolitis of varying severity. Its incidence is increasing, particularly in patients with acute myelogenous leukemia undergoing high dose cytosine arabinoside chemotherapy. The onset is heralded by prodromal fever, watery or bloody diarrhea, abdominal distension, and nausea during the phase of severe neutropenia. The symptoms may then localize to the right lower quadrant with an associated increase in systemic toxicity. The diagnosis can be confirmed in these and other less specific cases by serial reexamination and abdominal radiographs, ultrasonography, computerized tomograms, or radionucleotide scans. The mainstay of management is complete bowel rest with nasogastric suction and total parenteral nutrition. Broad-spectrum combination antibiotics are essential, as is the avoidance of laxatives or antidiarrheal agents. Granulocyte support may be helpful. Patients with a history of nonspecific gastrointestinal complaints or of true typhlitis, successfully managed nonoperatively, should have prophylactic bowel rest and total parenteral nutrition instituted at the beginning of further chemotherapy. Patients with ongoing severe systemic sepsis who do not respond to chemotherapy and those with overt perforation, obstruction, massive hemorrhage, or abscess formation require surgical intervention. All necrotic material must be resected, usually by a right hemicolectomy, ileostomy, and mucous fistula. Divided ileostomy for less severe cases may be useful. Failure to remove the necrotic focus in these severely immunocompromised patients is fatal. With adequate recognition of typhlitis and its precipitating factors, the incidence of complications can be reduced through prevention and timely surgical intervention. Although typhlitis developed in a quarter of our acute myeloblastic leukemia patients, use of this combined approach was successful in all cases.