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Biomedical subjects

R R Ricketts

Publications and source records attributed to R R Ricketts.

At least 37 records · Page 2Linked to original sources

Choledochal cysts: a ten year experience.

Choledochal cyst (CC) is a rare disorder that usually presents in childhood. Prognosis depends on early diagnosis, complete excision of the cyst, and reconstruction by hepaticojejunostomy. This report details our 10-year experience and emphasizes innovations in our management. Sixteen patients presented with CC at a mean age of 3 years. (Range, newborn to 21 years, with a M:F ratio 1:4). Two groups could be identified on the basis of age at presentation. Group I (N = 7), presented in the neonatal period, three with obstructive jaundice and four without symptoms. In Group II (N = 9), all patients presented with ascending cholangitis at a mean age of 6 years. Thirteen patients had a type 1 CC, one patient had a type 3 CC, and two had type 4 CC. The patients with type 1 and type 4 CC underwent primary cyst excision with Roux-en-Y hepaticojejunostomy, whereas the patient with type 3 CC underwent cyst excision with sphincteroplasty of the ampulla. There was only one complication of postop cholangitis that cleared with antibiotic therapy. All patients have remained free from symptoms in the follow-up period (6 months-10 years). Our four most recent cases were diagnosed in utero by prenatal ultrasonography. This led to appropriate antenatal counseling and prospectively planned neonatal surgery. These infants were asymptomatic, with no clinical signs at birth, and antenatal ultrasonography prevented diagnostic delay. Excision of the choledochal cyst and primary hepatico-enteric anastomosis is confirmed as the therapy of choice. Antenatal sonography is a sensitive method in the diagnosis of CC and offers the opportunity for early diagnosis and planned surgery before the onset of complications.

Adolescent↗

Idiopathic fibrosing pancreatitis: a rare cause of obstructive jaundice in children.

Idiopathic fibrosing pancreatitis is a rare cause of obstructive jaundice in children and young adults. Only 30 cases have been previously reported. Four children with this entity have been treated at Egleston Children's Hospital at Emory University since 1970. Diagnosis was established by imaging studies and open pancreatic biopsy. Treatment was by bilioenteric bypass, and excellent results have been achieved with follow-up periods up to 25 years.

Adolescent↗

Thoracoscopy in the management of pediatric empyema.

The surgical management of empyema consists of (1) aggressive therapy with thoracotomy and decortication or (2) conservative treatment with chest tube drainage and intravenous antibiotics. Recently, Kern and Rodgers introduced thoracoscopic debridement as an adjunct to the management of children with empyema, with promising results. Hence, the authors report their experience with thoracoscopy in the management of pediatric patients with empyema. In the last years, 10 children have undergone thoracoscopic debridement (TD) for empyema. The average age was 6.9 years (range, 2 to 16). Children underwent TD an average of 14 days (range, 8 to 16) after initial presentation and 4 days (range, 2 to 6) after admission to the authors' hospital. Indications for TD were persistent requirement of supplemental oxygen and failure of conservative medical management that consisted of antibiotics and tube thoracostomy. Three children had positive pleural fluid cultures for Streptococcus pneumoniae. In all cases, preoperative ultrasound or chest computed tomography examination showed dense pleural fluid with septation. During surgery, TD allowed for lung expansion and precise chest tube placement in all patients except one who required conversion to minithoracotomy and decortication for persistent encasement with a thick pleural peel. There were no postoperative complications related to the procedure. After TD, all children had prompt clinical improvement. The patients were weaned from supplemental oxygen by postoperative day 2, and following early chest tube removal, nine children were discharged home by postoperative day 7 (range, 3 to 10). One child required further hospitalization for underlying renal failure. In the authors' hands, TD was effective in producing prompt clinical improvement in children with empyema.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Interferon-alpha-2a for the treatment of complex hemangiomas of infancy and childhood.

OBJECTIVE: The authors describe the use of interferon-alpha-2a (IFN-alpha-2a) in the treatment of complex hemangiomas and review the role of interferon (IFN) in this example of an angiogenic disease. SUMMARY BACKGROUND DATA: Hemangiomas are the most frequent tumors of infants and children. They grow rapidly for 6 to 8 months and then resolve over a period of years. Approximately 5% produce life-, sight-, or limb-threatening complications, with mortality rates between 20% and 50%. Aggressive therapy with steroids, arterial ligation or embolization, or surgery has been used in these situations with variable results and high morbidity. Recently, IFN-alpha was found to be effective treatment in these complex hemangiomas. METHODS: Four infants and one child were treated with IFN-alpha-2a at an initial subcutaneous dose of 1 million units/m2/day and a sustained dose of 3 million units/m2/day for 5 to 11 months. Appropriate laboratory values were monitored and adverse reactions and ultimate response to therapy were recorded. RESULTS: Two patients experienced minor complications that were managed easily. Three patients had total or near-total regression of the hemangioma, one had partial (50%) regression, and one had stabilization but no regression after an average of 7.1 months of IFN therapy. CONCLUSION: Interferon-alpha inhibits angiogenesis and endothelial cell migration and proliferation in vitro. The patients in this study add to the growing number who have benefited from IFN therapy. As such, IFN-alpha should be considered as a first-line agent in treating complex hemangiomas of infants and children.

Child, Preschool↗

Surgical treatment of necrotizing enterocolitis and the short bowel syndrome.

Necrotizing enterocolitis (NEC) is the most common surgical emergency of newborns and the most common cause of short bowel syndrome in infancy. The surgical treatment of NEC is reviewed, including indications for operation, surgical procedures, postoperative management and complications, and survival. The causes, pathophysiology, and treatment of short bowel syndrome are also discussed.

Enterocolitis, Pseudomembranous↗

Pediatric liver transplantation in Georgia: a paradigm for the health care crisis in the United States?

The United States health care system, felt by many to be the most technologically advanced program in the world, has many critics. Two indisputable facts that drive such criticism are 1) inequitable access and 2) rising costs out of proportion to other countries. Although Georgia is a poor state and ranks nationally near the bottom in most measures of child and adolescent care, we decided to start a pediatric liver transplant program at Egleston Children's Hospital at Emory, Atlanta. Over the past 2 1/2 years, 18 transplants have been performed in 14 patients; 10 children are presently surviving. Looking carefully at the expenses of the first 10 patients, the average cost of orthotopic liver transplantation for the eight survivors was $206,375. The hospital costs for providing care to these 10 children were over $2 million. In a state that ranks 49th out of 50 states in infant mortality and with nearly one-third of its pre-school children not immunized against preventable diseases, is this a fair and equitable distribution of our resources?

Child↗

Malignant risk in juvenile polyposis coli: increasing documentation in the pediatric age group.

The presence of juvenile polyps with resulting bleeding and abdominal pain has traditionally been considered a benign, self-limiting process which would resolve with age. The dictum that these polyps were usually solitary, were found predominantly in the rectosigmoid area, and were without malignant potential has been reconsidered in recent years with the increased use of colonoscopy. Several case reports in both adults and children have documented the presence of adenomatous changes in this syndrome. We report 3 cases of children, ages 3, 11, and 11 who were found to have adenomatous polyps in the midst of fields of juvenile polyps on evaluation for rectal bleeding. All three were treated definitively with endorectal pull-through. Two of these patients had atypia on histological evaluation, one of which was severe. We recommend a more aggressive approach to patients found to have multiple juvenile polyps on barium enema, including colonoscopic biopsies at several sites to determine the presence of adenomatous changes, with colectomy and endorectal pull-through should these be found.

Adenomatous Polyps↗

Extracorporeal membrane oxygenation service at Egleston: two years' experience.

Extracorporeal membrane oxygenation (ECMO) is a perfusion support procedure that has been used to treat more than 7,000 patients with life threatening cardiac and/or respiratory failure. After 6 months of training and preparation, an ECMO service was opened on January 2, 1991, in Egleston Children's Hospital at Emory University. During the first 2 years, 96 neonatal, 31 pediatric, and 8 cardiac patients have been referred for possible ECMO. Of these 135 patients, 21 had disqualifying conditions. Sixty-four were considered candidates for ECMO but were able to be supported using less invasive therapies; only one of these died. Fifty patients were treated with ECMO of whom 39 survived (78%). Survival rates for neonatal, pediatric, and cardiac cases as separate groups as well as for each diagnostic category within these groups compare favorably with those reported by the international ELSO Registry. Notable in this series is the fact that 26/35 neonatal patients and 7/10 pediatric patients were successfully supported using venovenous (VV) rather than venoarterial (VA) perfusion, with the major indication for venoarterial ECMO being inability to introduce the 14F venovenous catheter into the patient's internal jugular vein. No patient initially managed with VV ECMO required conversion to VA. It is anticipated that avoidance of carotid ligation along with other innovations, such as the impending commercial availability of heparin-coated ECMO circuits, will make ECMO a highly attractive and appropriate therapy for an increasing number of high risk neonatal and pediatric patients in our state and region.

Adolescent↗

An unusual case of meconium peritonitis associated with perforated hydrocolpos.

Meconium peritonitis results from intestinal wall perforation with meconium spillage into the peritoneum during intrauterine life. Anal atresia is associated with meconium peritonitis and is also associated with cloacal malformation. A female neonate presented with a distended abdomen, anal atresia, and meconium peritonitis as diagnosed by calcification in the peritoneal cavity on abdominal radiograph. Sonography and cystography demonstrated a cloacal malformation with perforation of the hydrocolpos and resultant spillage of meconium into the peritoneum. There was no evidence of further perforation of bowel at surgery. This unusual case of meconium peritonitis is reported.

Anus, Imperforate↗

Rupture of the airways from blunt trauma: treatment of complex injuries.

Tracheobronchial rupture from blunt trauma is usually single and transverse but may be longitudinal or complex, a combination of various sites and forms of rupture. From 1970 to 1990, 183 cases of rupture of the airways were reported in the literature: 136 (74%) transverse, 33 (18%) longitudinal, and 14 (8%) complex. During the same 20 years at Grady Memorial Hospital, 6 patients with such injuries were treated. One had complex injury consisting of rupture of the distal trachea and both main bronchi, 1 had a longitudinal tracheal rupture and rupture of the innominate artery, and 4 had a transverse rupture, 1 of whom also had a traumatic false aneurysm of the left pulmonary artery. Cardiopulmonary bypass was used only for the repair of the complex injury, whereas the repair of the left main bronchial rupture associated with a false aneurysm of the left pulmonary artery was done with standby cardiopulmonary bypass. All 6 patients had satisfactory results from the correction of their lesions except 1 child in whom stenosis developed at the rupture site. This study suggests that complex injuries are rarely seen, and their repair is often quite involved. In some of these cases, the use of cardiopulmonary bypass increases the margin of safety during operation and may encourage repair rather than resection of the affected lung.

Adolescent↗

Surgery for achalasia of the cardia in children: the Dor-Gavriliu procedure.

The Dor-Gavriliu procedure was utilized in six children with achalasia of the cardia from 1983 to 1991. Four boys and two girls (mean age, 10.1 years; range, 3 months to 16 years) presented with symptoms of weight loss (83%), emesis (83%), dysphagia (67%), recurrent respiratory infections (67%), and nocturnal regurgitation (33%). The diagnosis of achalasia was established by barium swallow in all patients; esophageal manometry was used in four patients to confirm the diagnosis. Follow-up ranged from 10 months to 8 years (mean, 3.5 years). Four neurologically normal patients had excellent results with complete resolution of their preoperative symptoms. Two neurologically impaired children, both afflicted with Down's syndrome, had less than excellent results. One moderately impaired child had a good result (required three postoperative bougie dilations over 8 years without demonstration of gastroesophageal reflux); the second, more severely impaired child, had only a fair result (persistent failure to thrive with the development of grade II gastroesophageal reflux). The Dor-Gavriliu procedure uses a transabdominal, anterior esophageal myotomy with incorporation of an effective, nonobstructing, antireflux mechanism that should prevent myotomy reapposition.

Adolescent↗

Infantile gastrointestinal hemangioma as a cause of chronic anemia.

Gastrointestinal hemangiomas, though rare, can be an important source of GI bleeding in infants. In the past, diagnosis has been hampered by a lack of reliable methods. With the recent advent of selective mesenteric angiography, the suspected diagnosis can be readily confirmed. Surgical resection remains the definitive therapy; steroid therapy has produced conflicting results.

Anemia↗

The J-pouch Swenson procedure for ulcerative colitis and familial polyposis.

Total colectomy with mucosal proctectomy and ileal pouch-anal anastomosis has proven to be a favorable option in the treatment of ulcerative colitis and familial polyposis coli. The main advantages of this procedure are that it obviates the need for a permanent stoma, it preserves anal continence, and it removes all disease-prone mucosa. As an alternative to this procedure, the authors have found success with the Swenson pull-through following proctocolectomy in children. This technique involves resection of the rectum at the dentate line, thus, eliminating the need for mucosal proctectomy. This may be particularly advantageous in patients with severely diseased rectal mucosa. Ileoanal anastomosis is performed after creation of an ileal J-pouch using the terminal ileum. The procedure has been used in two children with familial polyposis coli and in three with ulcerative colitis. Median follow-up after closure of the diverting ileostomy is 13 months (5-33 months). Continence has been preserved in all five patients. There have been no complications involving bladder or sexual dysfunction. This technique provides a reliable alternative for the definitive treatment of ulcerative colitis and familial polyposis coli.

Adenomatous Polyposis Coli↗

Modern treatment of cloacal exstrophy.

We have treated 12 newborns with cloacal exstrophy (10 classical and 2 variants) since 1980. Eleven infants underwent repair and they are all surviving; the one nonoperated infant died of pulmonary hypoplasia. There are six genetic females and six genetic males, five of whom are being raised as females. The initial operation consisted of separating the bowel from the bladder to create an intestinal stoma; closing the omphalocele; and reapproximating (5), closing (4), or leaving the exstrophied bladder undisturbed (2). The importance of creating a "tailgutostomy" instead of an ileostomy to prevent problems with diarrhea, dehydration, and acidosis is emphasized. There have been 71 subsequent operations in these 11 patients (28 general surgical, 25 urological, 9 neurosurgical, and 9 orthopedic). Quality of life, rather than survival, is now the major issue facing patients with cloacal exstrophy. All of these patients except one are neurologically normal. The defect is closed in all patients. Three patients wear no appliances, 7 wear one (gastrointestinal [GI]), and one wears two (GI and genitourinary [GU]). We developed a scoring system to analyze bowel and bladder continence: voluntary control = 3; control with an enema program or intermittent catheterization = 2; incontinence with a well-functioning stoma = 1; and incontinence without a stoma = 0. The best continence score is 6 (GU + GI). We currently have 7 patients with a continence score of 1 (colostomy + incontinent bladder); 1 with a score of 2 (ileostomy + incontinent ureteroenterostomy); 2 with a score of 4 (enema program + continent urinary diversion); and 1 with a score of 5 (enema program + continent bladder).(ABSTRACT TRUNCATED AT 250 WORDS)

Bladder Exstrophy↗

Management of Hirschsprung's disease in adolescents.

It is unusual for Hirschsprung's disease to go undiagnosed until late childhood. Adolescents presenting with Hirschsprung's disease offer unique challenges to the surgeon because of the massively dilated and hypertrophied colon that is invariably present. Five adolescents (four males and one female; ages, 11 1/2 to 16 1/4 years) with newly diagnosed Hirschsprung's disease were managed by slightly different methods between January 1981 and March 1987. Three patients had a "leveling" colostomy, followed by a Swenson procedure in two and a Duhamel procedure with a temporary diverting colostomy in one. One patient had a primary Duhamel procedure with a temporary diverting ileostomy. One patient had a primary Swenson procedure without diversion. Long-term results were uniformly excellent. Based on the experience with these patients the following principles of management emerge: 1) Avoid loop "leveling" colostomies because of their grotesque size and their tendency to retract or prolapse. 2) Rectal tube decompression can often adequately prepare a patient for a primary pull-through procedure. 3) When a diverting ostomy is required, it should be an ileostomy rather than a colostomy because its subsequent closure will not endanger the marginal artery, which if divided, could compromise the blood supply to the pulled-through colon. 4) The Swenson procedure, although difficult in adolescents, can be done with excellent long-term results. 5) Several applications of the stapling instrument are required in the Duhamel procedure to fully divide the septum between the aganglionic rectum anteriorly and the normal colon posteriorly.

Adolescent↗

Hepatico-jejuno-duodenostomy reconstruction following excision of choledochal cysts in children.

Ten children with choledochal cysts were treated at Children's Memorial Hospital in Chicago between 1979 and 1980 and at Henrietta Egleston Hospital for Children or Grady Memorial Hospital in Atlanta between 1980 and 1987. Age at the time of operation ranged from one to 46 months. There were eight girls and two boys with Type I choledochal cysts. One child with a dilated but histologically normal common bile duct underwent a choledochoduodenostomy. The remaining nine patients had excision of the choledochal cyst. Continuity with the GI tract was then re-established with a valved isolated jejunal segment interposed between the common hepatic duct and the duodenum. The valve serves to prevent reflux of enteric contents up into the biliary tract. The short jejunal segment allows bile to drain into the duodenum with minimal stasis; this more closely approximates the natural physiologic state than does a Roux-en-Y reconstruction. There was no mortality, with follow up ranging from six months to over eight years. Two post-operative complications (one anastomotic stricture and one small bowel obstruction) occurred, both in the same patient. There were no anastomotic leaks and no episodes of post-operative cholangitis. All patients are currently free of jaundice and none have signs of portal hypertension. The treatment of choledochal cysts is excision. Biliary tract reconstruction with the valved common hepatico-jejuno-duodenostomy is safe, effective, and physiologically appealing.

Child, Preschool↗