Routine intraoperative cholangiography during laparoscopic cholecystectomy minimizes unnecessary endoscopic retrograde cholangiopancreatography in children.

PURPOSE: The aim of this study was to determine the necessity for intraoperative cholangiography (IOC) during pediatric laparoscopic cholecystectomy (LC). METHODS: A retrospective review of 100 consecutive patients undergoing LC was conducted. RESULTS: Ninety-eight children underwent successful LC. The average age was 11.3 years. IOC was successful in 55 of 63 studies. Operating time for patients with IOC averaged 91 minutes, and without IOC, 67 minutes. Twenty children had preoperative ultrasound, laboratory, or clinical evidence of common bile duct (CBD) stones. Fifteen of these 20 children actually had CBD stones. Three additional children who lacked any ultrasound, clinical, or laboratory evidence of choledocholithiasis had unsuspected CBD stones. Eight children, therefore, had ultrasound, clinical, or laboratory findings not predictive of the actual state of the CBD. Sixteen children underwent endoscopic retrograde cholangiopancreatography (ERCP), 9 preoperatively and 7 postoperatively. Four preoperative ERCP studies showed no CBD stones. There were no complications from performing IOC. CONCLUSIONS: (1) CBD stones are common in children with gallstones, (18 of 100 patients). (2) Preoperative studies and clinical findings may not predict accurately the presence or absence of CBD stones. (3) IOC should be routinely performed in children before the use of ERCP to avoid unnecessary ERCP unless CBD stones are specifically visualized by ultrasound scan. J Pediatr Surg 36:881-884.

Transplantation of adult living donor kidneys into infants and small children.

HYPOTHESIS: We hypothesized that improved outcomes following renal transplantation in high-risk infants and small children primarily are due to advances in immunosuppression and accurate diagnosis of rejection. Optimizing renal allograft perfusion is critical to achieving good early graft function and decreasing early graft loss. DESIGN: Twenty-eight consecutive recipients (weighing <20 kg) of adult living donor kidneys transplanted at our center from 1984 to 1999 were reviewed. Two groups were identified based on differing immunosuppression protocols and clinical surveillance. Actuarial graft and patient survival reported at 1, 3, and 5 years were compared for group 1 (1984-1991) and group 2 (1992-1999). Graft losses, categorized as immunologic or nonimmunologic, and the incidences of delayed graft function, vascular thrombosis, and rejection were compared. RESULTS: Graft and patient survival in group 1 (n = 13) at 1, 3, and 5 years was 77% and 92%, 54% and 85%, and 54% and 85%, respectively. In group 2, all 15 patients are alive with functioning grafts to date. Immunologic graft loss occurred in 5 of 13 patients in group 1 who developed chronic rejection. Nonimmunologic causes (vascular thrombosis [2 patients]) and patient death [1]) resulted in early graft failure within 2 weeks in 3 of 13 patients in group 1. The overall incidences of delayed graft function (10.7%) and thrombosis (7.1%) were low and did not differ between groups. Percutaneous renal biopsy was used more frequently in group 2 to evaluate graft dysfunction and guide treatment. CONCLUSIONS: We conclude that improved overall graft and patient survival in group 2 is owing to advances in immunosuppression and better treatment of rejection. Percutaneous renal biopsy allows prompt and accurate histological diagnosis of graft dysfunction. Surgical technique and aggressive fluid management aimed at maximizing renal allograft perfusion is critical in optimizing early graft function and decreasing vascular complications.

Asymptomatic inferior vena cava abnormalities in three children with end-stage renal disease: risk factors and screening guidelines for pretransplant diagnosis.

We report two children with end-stage renal disease (ESRD) found to have inferior vena cava (IVC) thrombosis at the time of renal transplantation. The children suffered from renal diseases that included congenital hepatic fibrosis and portal hypertension as part of their pathophysiology. Neither child had evidence of hypercoaguability or clinical symptoms of IVC thrombosis. Prior to transplantation, the renal replacement therapy consisted primarily of peritoneal dialysis. During their hospital courses, these children had central venous catheters placed for temporary hemodialysis, episodes of peritonitis and numerous abdominal surgeries. The medical literature to date has not identified a link between IVC thrombosis and portal hypertension, nor has an association between the patients' primary renal disease and IVC thrombosis been found. We also report the finding of asymptomatic IVC narrowing in a third patient with obstructive uropathy, colonic dysmotility and numerous abdominal surgeries. IVC narrowing was diagnosed by CT scan during his pretransplant evaluation. In this paper, we consider similarities between these three patients that may have predisposed each of them to asymptomatic IVC pathology, including large-bore central venous access as young children and/or recurrent scarring abdominal processes. A discussion regarding appropriate screening of the 'high-risk patient' for IVC pathology prior to kidney transplantation and surgical options for children with this rare complication are presented.

Minimally invasive surgery and clinical decision-making for pediatric malignancy.

BACKGROUND: Minimally invasive surgery (MIS) is an ideal way to obtain biopsy specimens in children with cancer. We examined the safety, reliability and outcome of decisions made based on tissue obtained using MIS. METHODS: Fifty-nine oncology patients underwent 62 MIS procedures between January 1994 and July 1998. Complications, biopsy results, and outcomes were reviewed. RESULTS: The study population comprised 32 boys and 27 girls, with an average age of 8.8 years. There were 47 thoracoscopic and 15 laparoscopic operations. Laparoscopic procedures included initial biopsy, determination of resectability, and second-look exam. Thoracoscopic procedures included 40 lung biopsies and seven biopsies/resections of mediastinal masses. Diagnostic accuracy was 100% in all cases. No patient was found retrospectively to have been inadequately treated based on decisions made from tissue obtained by MIS. CONCLUSION: MIS is a safe and accurate means of obtaining tissue in pediatric oncology patients. Treatment decisions can be made accurately and with confidence using these techniques.

Delayed primary repair of esophageal atresia with tracheoesophageal fistula: is it worth the wait?

OBJECTIVE: To characterize a successful approach to the management of infants with long-gap esophageal atresia (EA) with tracheoesophageal fistula (TEF), significant prematurity with respiratory distress syndrome (RDS), or both, so as to preserve the native esophagus. DESIGN: A review of the medical records and office charts of a cohort of patients with EA and TEF. SETTING: A tertiary care children's hospital affiliated with a major university. PATIENTS: A total of 118 children with EA and TEF admitted from February 1986 through December 1996. All of the patients diagnosed as having EA and TEF during this period were included. INTERVENTION: Of the 118 infants, 88 received primary repair of EA and TEF within 48 hours of birth. An additional 23 children had the TEF divided and a gastrostomy placed secondary to (1) severe RDS and prematurity (n = 6), (2) long-gap EA (gap length > 4 cm or the upper pouch above the thoracic inlet (n = 10), or (3) associated cardiac defects (n = 7). Delayed primary EA repair was done when the RDS resolved or the gap length was 2 cm or less. MAIN OUTCOME MEASURES: Successful anastomosis of native esophagus. Comparison of incidence of gastroesophageal reflux, anastomotic complications, or survival between groups undergoing primary or delayed repair. RESULTS: Primary EA was accomplished in 88 patients. Delayed EA was successfully accomplished in 18 of the 19 surviving patients within 5 months, thereby preserving the native esophagus in all surviving infants. There was no difference in anastomotic complications, gastroesophageal reflux, or survival when the delayed group was compared with those who had a primary repair. CONCLUSIONS: Using delayed EA repair, all children with EA and TEF, regardless of gap length, can have their esophagus preserved. The primary cause of mortality was the association of a severe cardiac anomaly with EA and TEF.

Aortic arch anomalies associated with long gap esophageal atresia and tracheoesophageal fistula.

PURPOSE: The purpose of this study was to determine whether aortic arch anomalies are associated with long gap esophageal atresia and tracheoesophageal fistula (EA-TEF). METHODS: The authors performed a retrospective review of all infants who had EA-TEF from 1980 to 1996 at two pediatric surgery centers. Two hundred three infants who had EA-TEF were identified. RESULTS: Twelve infants were noted to have both long gap EA-TEF defined as a gap length greater than 3 cm and aortic arch anomalies. Of these 12, 7 had aberrant right subclavian arteries originating from the descending aorta. Four of the seven infants who had aberrant right subclavian artery (SCA) had gap lengths greater than 4 cm. All four had their fistulae divided initially through a right thoracotomy with primary repair performed at a later date. The remaining five infants who had long gap EA-TEF had right-sided aortic arch with aberrant left subclavian arteries. All five initially underwent exploration through the right chest. On discovery of the long gap EA and concurrent vascular anomaly, the thoracotomies were closed, and the infants underwent definitive repair of both their EA-TEF and their vascular anomaly through a left thoracotomy. CONCLUSIONS: The authors find that aortic arch anomalies are associated with long gap EA-TEF. Patients who have these two anomalies tend to have a long gap. Preoperative diagnosis of these anomalies may alter the timing and technique of surgical intervention. The embryogenesis of these vascular lesions may account for this more severe form of esophageal atresia.

Posterior sagittal anorectoplasty: primary repair of a rectovaginal fistula in an adult: report of a case.

PURPOSE: A case of an adult patient with the anorectal malformation of a rectovestibular fistula successfully repaired by performing a posterior sagittal anorectoplasty is reported. This case should increase the awareness of both primary and secondary anorectal malformations in the adult patient. METHODS: Management and outcome of an adult patient who presented with a rectovaginal fistula and underwent primary operative correction of her anorectal malformation using posterior sagittal anorectoplasty is reviewed. RESULTS: Total continence was achieved in an adult patient undergoing primary repair of a rectovaginal fistula using posterior sagittal anorectoplasty. CONCLUSION: Posterior sagittal anorectoplasty can be successfully performed in the adult patient for a primary repair of anorectal malformations. This operation should be considered in patients who have undergone another prior operative procedure with less than optimum function and now desire a secondary corrective procedure.

Is pediatric laparoscopic splenectomy safe and cost-effective?

OBJECTIVE: To determine whether laparoscopic splenectomy (LS) is a safe, cost-effective alternative to open splenectomy (OS). DESIGN: Retrospective comparison and economic evaluation. SETTING: Tertiary care children's hospital. PATIENTS: Children with hematologic disease involving the spleen. INTERVENTIONS: Laparoscopic splenectomy (10 patients); OS (10 patients). MAIN OUTCOME MEASURES: Safety of LS and cost differential between LS and OS. RESULTS: Operative time was longer for LS. Discharge occurred 64 hours postoperatively for LS and 79 hours for OS (P < .03). Patients who underwent LS returned to activity 1 to 5 weeks faster than patients who underwent OS. The average (+/-SD) operative charges for LS was $7176 +/- $2064 and for OS, $1977 +/- $344 (P < .001). Total hospital charges averaged $13,033 +/- $2976 for LS and $7106 +/- $1923 for OS (P < .001). CONCLUSIONS: Laparoscopic splenectomy can be performed safely in children. In our hospital, LS is more expensive than OS. The faster return to school and normal activity warrants the continued use of this procedure despite the increased cost.

Video-assisted thoracoscopic closure of postpneumonectomy bronchopleural fistulas.

Despite improved surgical techniques and advances in medical-surgical treatment, postpneumonectomy bronchopleural fistulas remain an important cause of morbidity and a therapeutic challenge. Video-assisted thoracoscopic closure of these fistulas reinforced by transposition of bulky chest wall muscles or omentum to obliterate the residual space may lessen risks and complications of repeated thoracotomy in these often frail, debilitated, and compromised patients. We report our initial experience with video-assisted thoracoscopic debridement of the empyema cavity and closure of the postpneumonectomy bronchopleural fistula by transposing an entire pectoralis major muscle in one patient and by transposing a pedicled omentum in another patient.

The surgical management of giant cervicofacial lymphatic malformations.

Giant cervicofacial lymphatic malformation, a potentially life-threatening congenital malformation, historically has been removed in staged resections. However, complete surgical extirpation is desirable and can be achieved with the aid of a multidisciplinary team. The authors present the case of a 12-year-old girl who had an extensively enlarged cervicofacial lymphatic malformation. Effective treatment involved aggressive surgical excision, facilitated by mandibular osteotomies to provide access to the floor of the mouth, the tongue, and the pharyngeal wall. In addition, total removal of involved facial and neck skin was performed. The remaining soft tissue defect was reconstructed primarily with an abdominal musculocutaneous free flap. In the 5 years since the procedure, there has been no recurrence. Based on this experience, excision of involved skin and mandibular access osteotomies are important techniques to help completely remove cervicofacial lymphatic malformations with oral involvement, thus potentially reducing the number of recurrences. In addition, these cases require the best efforts of a multidisciplinary team to achieve a successful result so that complete and radical excision can be performed with the hope of avoiding the pitfalls of multiple resections.

Does posterior sagittal anorectoplasty in patients with high imperforate anus provide superior fecal continence?

The posterior sagittal anorectoplasty (PSARP) has become the primary surgical procedure for patients with high imperforate anus. Very few careful long-term follow-up studies have established the superiority of the PSARP procedure over other surgical repair techniques. The authors' goal was to evaluate the operation and to identify the factors associated with improved continence. The medical records, operative reports, and radiographs of 53 patients (46 male, 7 female) who underwent PSARP for high imperforate anus at Children's Hospital and Medical Center (CHMC) between 1982 and 1990 were reviewed retrospectively. Subjective follow-up data were collected by telephone questionnaire, assessing habits indicative of stool continence, and a "fecal continence score" (FCS) was calculated for each patient. A prospective, 7-day diary assessing similar patterns of fecal continence was completed by each patient's family. The mean age of the patients studied was 8.0 years, and the mean follow-up period was 6.8 years. The telephone questionnaire was completed for 48 patients (94%). Toilet training for bowel continence was successful in 20 patients (42%) and occasionally successful in another 20 patients (42%), but 8 patients (16%) had no awareness of impending stool. Forty-five (94%) were physically active, but 16 (33%) reported social problems related to offending odor. The mean fecal continence score for all patients was 3.0 +/- 1.4 (5=excellent, completely continent), which was nearly identical to published scores for the other types of surgical repair. The FCS did not improve with age. The parents' responses to the telephone questionnaire matched the results obtained from the prospectively collected continence diary data. The authors' results for PSARP in patients with high imperforate anus do not differ substantially from those achieved by other repair techniques. Previously cited prognostic factors such as fistula anatomy and sacral anomalies did not appear to alter the functional results in the authors' series. Aggressive postoperative bowel management should be anticipated in all patients who have high imperforate anus and may benefit those who otherwise would achieve less satisfactory continence.

Identification and surgical management of cystic retroperitoneal lymphangioma in children.

Retroperitoneal lymphangiomas are rare, benign cystic structures that are best evaluated with computed tomography and ultrasound. Preoperative diagnosis is often difficult, in part because there is little to distinguish them from other cystic masses and because the lesion is often not considered on the differential diagnosis. Surgery may be required as both a diagnostic and therapeutic measure. The cysts may be asymptomatic for years and then present because of compression of surrounding structures or pain. The treatment is as complete surgical excision as is possible. Bowel cleansing should be done preoperatively. The long-term results are excellent when total excision or near-total excision with marsupialization is accomplished.

Management of transected pancreas in children.

The pancreas is the fourth most commonly injured intra-abdominal organ in children who sustain blunt abdominal trauma. Appropriate management of the injured pancreas has been controversial. With the advent of the computerized tomography scan, paediatric surgeons have tended to manage pancreatic injuries non-operatively. However, if pseudocysts develop, non-operative management may necessarily entail a long hospital course involving total parenteral nutrition, drainage procedures and attendant morbidity. The critical element in planning therapy is to determine the status of the pancreatic duct. We have recently encountered five children who suffered blunt pancreatic injury where the main pancreatic duct was determined to have been transected. These children underwent spleen preserving distal pancreatectomy with resultant shorter hospital stays and minimal long-term morbidity. We suggest that in children with pancreatic injury where the main pancreatic duct has been transected early operative management rather than non-operative therapy is the procedure of choice. Endoscopic retrograde cholangiopancreatography should be used to determine the status of the pancreatic duct. This modality can be both diagnostic and therapeutic in appropriate circumstances.

Lessons learned in the management of hemolytic uremic syndrome in children.

Escherichia coli O.157:H7 is a serious and common human pathogen that can cause diarrhea, hemorrhagic colitis, and the hemolytic uremic syndrome (HUS). During a massive outbreak of infection with E coli O157:H7 in January 1993 in Washington State, more than 600 people, mostly children, acquired symptomatic infection, and 37 were hospitalized with HUS at Children's Hospital and Medical Center in Seattle, and six at other hospitals in Washington. Twenty-one (57%) required dialysis. Nineteen (51%) had significant extrarenal pathology: gastrointestinal in 14 patients (38%), cardiovascular in 13 (35%), pulmonary in 9 (24%), and neurological in 6 (16%). Most patients were managed nonoperatively, but three required total abdominal colectomy and one a left colectomy. No child had perforation. Three patients died, all of whom had multisystem disease. The authors recommend (1) that all patients with bloody diarrhea undergo microbiological evaluation for E coli O157:H7 before any surgical intervention; (2) avoidance of antibiotics and antimotility agents in patients with proven or suspected infection with E coli O157:H7 until the safety and efficacy of such interventions have been established in controlled trials; (3) that patients with E coli O157:H7 infections be evaluated for microangiopathic changes consistent with HUS in the week after onset of diarrhea; (4) nasogastric suction for severe symptoms, and frequent abdominal evaluations, tests (electrolytes/amylase), and roentgenograms to exclude treatable abdominal disorders; and (5) institution of hemodialysis for oliguria/anuria, acidosis, or rising creatinine. The authors recommend surgical exploration for toxic megacolon, colonic perforation, acidosis unresponsive to dialysis, or recurrent signs of obstruction or colonic stricture.

Videothoracoscopic wedge resection for peripheral pulmonary nodules.

BACKGROUND: This study was done to evaluate the use of the endoscopic multifire linear stapler for videothoracoscopic wedge resection (VTWR) of peripheral pulmonary nodules and to define the indications, advantages, and drawbacks of this minimally invasive technique. STUDY DESIGN: A case study review of 57 consecutive video-assisted thoracic operations for wedge resection of peripheral pulmonary nodules performed upon 55 patients admitted to a community hospital from June 1991 through July 1993 is presented. RESULTS: There were 44 malignant and 13 benign lesions. Of the malignant peripheral pulmonary nodules (PPN), there were 19 adenocarcinomas, ten squamous cell carcinomas, two undifferentiated large-cell carcinomas, three bronchoalveolar carcinomas, two carcinoid tumors, one neuroendocrine tumor, and seven metastatic carcinomas. The benign nodules included five hamartomas, two granulomas, one aspergilloma, one nodular amyloidosis, one Wegener's granulomatosis, one focal pulmonary infarct, and two interstitial fibroses. Videothoracoscopic wedge resection alone was performed upon 37 patients, 17 of whom had primary carcinoma of the lung; seven had metastatic lesions, and the remainder had benign disease. Of the 17 patients with primary carcinoma of the lung who had VTWR alone, eight patients had marked impairment of pulmonary function, six had significant co-morbid disease, two had peripheral carcinoid tumors, and one had bilateral metachronous carcinomas. Videothoracoscopic wedge resections with concomitant lobectomies were performed upon 20 patients with primary carcinoma of the lung, including one patient with bilateral synchronous carcinomas. Five of the patients with nodules ranging from 2 to 3 cm in diameter were found to have metastasis to regional nodes. None of the patients who had lobectomies for peripheral carcinomas less than 2 cm in diameter had regional nodal metastases. There was no perioperative mortality and no significant morbidity. CONCLUSIONS: Videothoracoscopic wedge resection is a useful alternative to traditional transthoracic resection for suspicious, undiagnosed PPN, for low grade malignant neoplasms, such as carcinoid, for peripheral metastatic lesions, for bilateral synchronous or metachronous tumors, for the occasional clinically localized peripheral small-cell carcinoma as a surgical adjunct to chemotherapy, and for small, peripheral, T-1, N-0, M-0 bronchial carcinomas in compromised patients at high risk with marginal pulmonary reserve. The procedure is effective, minimally invasive, and has potential advantages over conventional thoracotomy, including less postoperative pain and morbidity, shorter hospitalization period and convalescence, and an earlier return to work and normal activities.

Limited surgery for lower-segment Hirschsprung's disease.

OBJECTIVE: To determine if rectal myectomy is an effective treatment for a specific group of patients with Hirschsprung's disease. DESIGN: Retrospective series with follow-up of 6 to 132 months (mean follow-up, 37.1 months). SETTING: Children's hospital. PATIENTS: Fourteen children aged 2 to 14 years with chronic constipation and nondiagnostic barium enema contrast study findings. INTERVENTION: Posterior rectal myectomy. MAIN OUTCOME MEASURES: Laxative use, frequency of stools, incidence of soiling or incontinence, and patient's assessment of improvement. RESULTS: The conditions of all 14 patients improved regardless of the presence of ganglion cells in the myectomy specimen; there were six excellent, six good, and two fair assessments. laxatives were used intermittently by seven of 14 patients. No patients complained of incontinence or soiling. CONCLUSIONS: Rectal myectomy is an effective, less-extensive operation that should be considered for older children with Hirschsprung's disease.

Modulation of the excitability of avian peripheral nerves by vitamin D: relation to calbindin-D28k, calcium status and lipid composition.

Calbindin-D28k (CaBP), previously localized in some of the cell bodies of ganglia of the avian intestinal (Remark's) nerve, was shown to be vitamin D-dependent. In the present studies, the effect of vitamin D3 on electrophysiological properties of this nerve was examined in vitro. Electrical stimulation of the nerve yielded a compound action potential with two primary components, Peaks I and II. Peak II, suppressed by hexamethonium bromide or Ca(2+)-free buffer, is synaptically mediated. The transit time between the two peaks was unaffected by vitamin D3. The apparent conduction velocity, defined as [(activation time + transit time)/nerve length], was increased by vitamin D-deficiency and decreased by vitamin D3 repletion, the latter decrease due entirely to an increase in activation time. Activation time after vitamin D-repletion was correlated with an increase in CaBP and plasma Ca2+ levels. However, normalization of plasma Ca2+ by supplementation of vitamin D-deficient diets with excess calcium (2.5 and 4.0%) also resulted in an increase in activation time, without affecting neuronal CaBP levels. Vitamin D3 also decreased the conduction velocity and increased CaBP of the vagus nerve and, by lipid analysis, was shown to increase and decrease its phosphatidylcholine and phosphatidylethanolamine content, respectively, and to decrease its phospholipid/cholesterol ratio. Modulation of peripheral nerve activity by vitamin D3 is related to calcium status and perhaps to changes in lipid composition. The functional role of CaBP in the behaviour of this complex nerve remains unknown.

Early recognition of neonatal abdominal wall necrotizing fasciitis.

Necrotizing fasciitis (NF) of the abdominal wall occurring in newborns is associated with a 50% mortality rate. Improved survival requires early diagnosis followed by aggressive surgical débridement. During a 10-year period, we treated 7 infants who developed NF. During the same period, 32 infants were admitted with omphalitis that did not progress to NF. The patients with omphalitis and those with NF were compared. Tachycardia, abnormal white blood cell counts, induration, and violaceous skin discoloration were seen exclusively in the NF patients. Polymicrobial infections were documented in 28% of the omphalitis patients and 86% of the NF patients. All omphalitis patients survived, whereas 5 of 7 (71%) NF patients died. Adjuvant hyperbaric oxygen therapy was used for 4 infants with NF, 2 of whom survived (50%). NF is a highly morbid disease, that can be distinguished from other infant abdominal wall infections by the skin changes, white blood cell counts, heart rate, and microbiologic results. Prompt diagnosis of NF improves survival when combined with aggressive surgical débridement.

Growth factor profiles in breast cyst fluid identify women with increased breast cancer risk.

Gross cystic disease and breast cancer are hormonally induced diseases that may share a common biochemical environment conducive to abnormal proliferative responses. Breast cyst fluid samples were analyzed for specific growth factors and levels were compared with breast cancer risk. Growth factor profiles identified both women at increased breast cancer risk and subgroups of women with distinct clinical manifestations of gross cystic disease. Women at increased risk for breast cancer demonstrated in their breast cyst fluid lower levels of platelet-derived growth factor and transforming growth factor (TGF)-beta, compared to women at lower risk. The presence of multiple cysts was associated with increased mitogenic activity, increased epidermal growth factor (EGF) and TGF-beta breast cyst fluid levels, and recurrent cysts were associated with higher levels of EGF. Unique growth factor profiles were associated with each risk group or clinical state, suggesting that distinct proliferative environments, associated with different clinical outcomes, are present in the breast tissue of women with gross cystic disease.