T-tube ileostomy for meconium ileus: four decades of experience.

BACKGROUND/PURPOSE: The T-tube ileostomy was first used at Texas Children's Hospital in 1959. The purpose of this study is to update the experience since the initial report of this technique in 1981. METHODS: A database of 448 patients with cystic fibrosis (CF) seen in the authors' institution was used to identify 83 patients (18.5%) who presented with meconium ileus. The clinic and hospital charts of these patients were reviewed retrospectively to identify patients who had undergone placement of a T-tube ileostomy. RESULTS: Surgery was performed in 60 of 83 patients for complications of meconium ileus or failure to evacuate the meconium after a contrast enema. Of these patients, 21 of 60 (35%) underwent placement of a T-tube ileostomy. An additional 8 patients were identified who underwent placement of a T-tube ileostomy but were not included in the CF database, for a total of 29 patients who have been treated with T-tube ileostomy since 1959 at Texas Children's Hospital. Five patients were excluded from analysis because of insufficient data or misdiagnosis. One of the 24 patients in the series died of complications of prematurity. A total of 20 of 23 patients had resolution of their meconium ileus after T-tube irrigation with n-acetylcysteine or pancreatic enzymes. Three patients required additional surgery to relieve persistent bowel obstruction. All patients had the T-tube removed within the first 8 weeks after surgery. Two patients required subsequent repair of an incisional hernia. There were otherwise no complications of this procedure, with an average follow-up of 11.5 years. CONCLUSION: In patients with uncomplicated meconium ileus unrelieved by contrast enema, the T-tube ileostomy is an effective and safe treatment.

Postoperative ad lib feeding for hypertrophic pyloric stenosis.

PURPOSE: The aim of this study was to compare three methods of postoperative feeding after pyloromyotomy for hypertrophic pyloric stenosis (HPS). METHODS: The authors reviewed retrospectively the charts of 308 patients who underwent pyloromyotomy for HPS from 1984 to 1997. Nineteen patients had prolonged hospitalization for other reasons and were excluded from the study, leaving 289 patients for analysis. All procedures were performed by a single group of pediatric surgeons. The individual preferences of these surgeons resulted in three different feeding schedules: R, strictly regimented (>12 hours nothing by mouth, then incremental feeding over > or =24 hours), I, intermediate (>8 hours nothing by mouth, then incremental feeding over <24 hours), or A, ad lib (< or =4 hours nothing by mouth, with or without a single small feeding, then ad lib feedings). RESULTS: Of the 289 patients, 248 (80.5%) were boys. The average age of the patients was 5.64 weeks (range, 1 to 21 weeks). A total of 265 of 289 (92%) were full term. Thirty-nine of 289 (13.5%) had a family history positive for pyloric stenosis. A total of 104 of 289 (36%) were first-born infants, 89 of 289 (31%) were second born. The diagnosis of pyloric stenosis was made by a combination of physical examination findings and diagnostic image for most patients. An "olive" was palpated in 60.6% of the patients. Sixty percent (60.4%) of patients had an upper gastrointestinal series performed, and 42.5% were examined by ultrasonography. Overall, 53% of the patients had postoperative emesis. Only 3.5% had emesis that persisted greater than 48 hours after surgery. Patients fed ad lib after pyloromyotomy had slightly more emesis (2.2 A v. 1.2 R, and 0.7 I episodes, P = .002), but tolerated full feedings sooner than patients fed with a regimented or intermediate schedule. No patient required additional therapy or readmission after tolerating two consecutive full feedings, suggesting that this might be a suitable discharge criterion for most patients with HPS.

The role of surgery in the management of pediatric pelvic rhabdomyosarcoma.

PURPOSE: We assessed the role of surgery, particularly exenteration, in the treatment of children with lower urinary tract and pelvic rhabdomyosarcoma. MATERIALS AND METHODS: We treated 23 children with bladder and/or prostate (11), or pelvic retroperitoneal tumors (12). Initial management was tumor resection in 6 cases, anterior pelvic exenteration in 5 and biopsy only in 12, combined with chemotherapy in 23 and radiotherapy in 20. RESULTS: The bladder salvage rate for surviving patients with pelvic tumors was 92% versus 27% for those with prostate/bladder tumors. Estimated 5 and 10-year survival probability for patients with pelvic retroperitoneal tumors was 49 +/- 50% compared to 81 +/- 24% for those with bladder and/or prostate tumors (log rank test, p = 0.11). CONCLUSIONS: Exenterative surgery is frequently required to achieve a durable complete response.

Congenital lung cysts.

Congenital cystic adenomatoid malformation (CCAM), pulmonary sequestration, congenital lobar emphysema (CLE), and bronchogenic cysts are all congenital malformations of the lung that present in imaging studies as abnormal air, air/fluid, or fluid-filled cysts. The embryology, histology, clinical presentation, and treatment of these lesions are discussed based on world literature and our experience with 22 operative resections of congenital lung cysts over the past 10 years. The roles of prenatal diagnosis and fetal surgery in the management of certain lung cysts are considered. Computed tomography has emerged as an extremely useful tool in the differential diagnosis of these lesions. Surgical excision by segmentectomy or lobectomy for intrapulmonary lesions and simple excision for extralobar sequestrations and bronchogenic cysts are safe and have a low incidence of complications. Asymptomatic patients with CLE may not require resection. Overall, with accurate diagnosis and preoperative planning these rare but fascinating anomalies can be treated safely and effectively, with excellent results.

Limy bile syndrome.

Limy bile syndrome (LBS) is a rare condition in which a radiopaque gallbladder and/or bile ducts are noted on plain roentgenograms. LBS is caused by calcium carbonate precipitation in the bile and is usually associated with distal biliary tract obstruction. The etiology of limy bile syndrome is unclear; however, it may be a long-term complication of total parenteral nutrition.

Emergency center arteriography in the evaluation of suspected peripheral vascular injuries in children.

During the 6-year period from 1983 to 1989, 109 children aged 3 to 18 years (mean, 16) with suspected peripheral vascular injuries underwent 113 emergency center arteriograms (ECA) performed by hand injection of contrast material using a single roentgenographic film. The most common indication for ECA was the proximity of the injury in 93 (82.3%) of the cases as penetrating injury accounted for 106 (94%) of the cases. There were 89 true-negative, 14 true-positive, 1 false-negative, and no false-positive arteriograms. The remaining 9 arteriograms were either equivocal or technically inadequate. The sensitivity, specificity, and diagnostic accuracy of this procedure were 98.9%, 100%, and 91.2%, respectively. Operative intervention was required for 9 (64.2%) injuries detected by emergency arteriography. The remaining 5 injuries were considered minor and were observed with no complications during a period of 21 months. Sixty-eight children (76.4%) with negative ECA were followed for a mean of 12 months with no vascular complications or growth abnormalities noted. ECA is a rapid, accurate, and cost-effective technique. It is of particular value in detecting the presence of occult arterial injuries that might have deleterious effects in the growing child.

Current management of laryngeal and laryngotracheoesophageal clefts.

Laryngeal and laryngotracheoesophageal clefts (L-LTEC) are uncommon anomalies in neonates that cause significant morbidity secondary to aspiration, pneumonia, and respiratory distress. Other anomalies of development, such as esophageal atresia and tracheoesophageal fistula (EA-TEF), are observed in 20% of patients with L-LTEC and often confuse the radiographic and clinical picture. Repair of L-LTEC depends on the length and location of the cleft, associated anomalies, and concurrent systemic illness. For type I L-LTEC, endoscopic repair occasionally is possible with microsurgical instrumentation. With types II to IV L-LTEC, an open approach must be used. Tracheotomy is a universal requirement, often for extended periods of time. Reported here is our experience with four cases of L-LTEC managed over the past 7 years at Texas Children's Hospital.

Ventriculoatrial shunting via the azygos vein.

The treatment of hydrocephalus has evolved through many stages but the "cure" is still elusive. It is not unusual for the neurosurgeon to find that the commonly used routes for catheter placement or sites for drainage of cerebrospinal fluid (CSF) cannot be employed. The azygos vein was used to gain access to the right atrium when the CSF could not be drained into the peritoneal cavity, nor could the neck veins be used to place the catheter into the right atrium. The azygos vein is a convenient and safe route to reach the right atrium in selected patients.

Thyroid nodules in children: current profile of a changing disease.

Pediatric thyroid disease has changed since the decline of widespread cervical irradiation. Our analysis of 32 recently resected childhood thyroid nodules showed that the incidence of malignancy (22%) is decreasing, though the incidence of nodules is stable. Risk factors for development of thyroid disease include female sex, age around puberty, family history of thyroid disease, previous or coexisting thyroid disease, and history of a medical condition that may be steroid- or endocrine-related. Age, sex, location and size of nodule, duration of symptoms, and family or patient history of thyroid disease did not distinguish between malignant and benign disease preoperatively. Of the seven children with thyroid cancer, six (86%) had unrelated concurrent medical conditions, compared with 9/25 (36%) with benign disease and 2/11 (18%) with adenoma. Fine needle biopsies provided correct preoperative diagnosis in 6/7 cases. As pediatric thyroid malignancy becomes rarer and the high-risk nodule becomes better defined, criteria for surgical versus nonsurgical modes of diagnosis and treatment should become more distinct.

Central venous catheters in older children.

This report discusses the safety of 89 cuffed, Silastic (Dow Corning, Midland, MI) catheters placed in children between the ages of 1 and 18, a group that may be considered high risk on the basis of age. The overall complication rate was once every 288 days, three times more often than in published results in adults, but less often than in other pediatric series. The sepsis rate of once each 1236 days was almost twice the adult rate. Thirty (35%) of the catheters were used at home, accounting for 85 per cent of the total catheter use. Among this group, the overall complication rate was only once every 815 days, three times less frequent than in comparable adult series. The complication rate due to sepsis was once each 2444 days, the same as noted in adults. Whereas these catheters may be more hazardous overall in this age group, home usage appears to be safer than expected. These catheters are well accepted by this age group and their use, particularly among outpatients, should not be restricted for fear of an unacceptably high complication rate.

Small bowel obstruction as a complication of Kawasaki disease.

A case of small bowel obstruction in an 8-month-old infant with Kawasaki disease is described. At laparotomy a discrete area of jejunal stricture with adhesions was noted. Microscopic examination revealed evidence of small artery thrombosis. Kawasaki disease results in a diffuse vasculitis, which may produce significant abnormalities in multiple organ systems. Serious abdominal complications can occur and should be considered when gastrointestinal symptoms develop in a patient with Kawasaki disease.

Bilateral Wilms' tumor.

Recent studies indicate a favorable outcome with bilateral Wilms' tumor. From 1971 to 1985, ten children between the ages of 6 months and 5 years were treated for this disease. During the early part of the series, five patients had nephrectomy on one side and partial nephrectomy on the other side, and one patient had bilateral partial nephrectomies at the same operation. More recently four patients initially had a biopsy of both tumors and lymph node sampling followed by chemotherapy. At the second-look procedure two patients had multiple biopsies because there was no obvious tumor. Histologically there was no tumor on the third-look procedure in these two patients. Nine patients had a favorable histology of the Wilms' tumor. One patient had a favorable histology on one side and an unfavorable type of histology on the other side. Eight patients are surviving between 6 months and 13 years. Two died of extensive disease within 16 months of diagnosis. One patient had an unfavorable histology. The good results following partial nephrectomies have led us to attempt to conserve additional tissue, as has been done in the last four patients. Our early results suggest biopsy of the tumor followed by chemotherapy, then a second look, and if necessary, third-look procedures may result in preservation of functioning renal tissue.

Major duodenal injuries in children: diagnosis, operative management, and outcome.

The diagnosis, treatment, and outcome of ten children less than 13 years old operated on for major duodenal injuries is reviewed. Three had gun shot wounds with perforation and seven had blunt trauma resulting in duodenal disruption. Of those with blunt trauma, three had massive injuries requiring immediate operation, and four had what appeared to be lesser injuries. The symptoms of those children with lesser injuries were initially vague and delayed in onset, resulting in a delay of 24 hours to 7 days from time of injury to admission. At the time of admission, all four had tenderness localized to the right lower quadrant and an unclear preoperative diagnosis. Eight had associated injuries, most commonly to the pancreas. The single death occurred within 48 hours of injury and was the result of an associated head injury. The surviving nine patients left the hospital between the 11th and 22nd postoperative day doing well. One patient with a transected pancreas and extensive duodenal injury underwent a pancreaticoduodenectomy with a Whipple type reconstruction. Two patients with an extensive blowout injury to the duodenum and one with a crush injury to the pancreas underwent a pyloric exclusion and gastrojejunostomy. These patients did well with no fistula formation. The remaining seven children underwent debridement and primary closure of the duodenal injury. Four developed fistulae; however, two were pancreatic, one was jejunal, and only one was duodenal. All drained less than 250 mL per 24 hours and all except the jejunal fistula were closed by the 17th postoperative day. All were supported nutritionally either intravenously or more recently with a jejunal catheter placed at operation.(ABSTRACT TRUNCATED AT 250 WORDS)

Late presentations of midgut malrotation in children.

Malrotation of the intestine may become symptomatic in the older child and may manifest itself in atypical presentations. Older children may present with symptoms of less than 72 hours duration which are typical of acute duodenal obstruction. More frequently, however, the older child with malrotation will present with chronic abdominal pain with or without vomiting or chronic diarrhea. The diagnosis of malrotation should be considered in any child with intermittent abdominal pain, vomiting, diarrhea, or malabsorption. Surgical intervention is curative and should be implemented as soon as possible after the diagnosis is made.

Psoas abscess in children.

In children, psoas abscess does not head the list in the differential diagnosis of the child who presents with a limp or lower abdominal pain. Therefore, the road to this diagnosis can be long and complicated leading to numerous studies and specialty consultations. Over a 7-year period, seven psoas abscesses have been drained surgically. All were Staphylococcal though one was mixed. In each case, the original admitting diagnosis was that of septic arthritis of the hip. In general, this diagnosis was ruled out by negative hip aspirations and bone scans. Often, the severity of symptoms led to persistent evaluation with noninvasive tests such as gallium scan, intravenous pyelogram, or barium enema. Though these tests were often suggestive, a positive ultrasound or CT scan was the key studies diagnostic enough to warrant surgical exploration and drainage. During this time period, there have been no negative explorations for psoas abscess. Upon surgical drainage, all patients improved, with subsequent recovery of hip function. The child who presents with a limp or painful hip should be considered for ultrasonography or computerized tomography once hip pathology is ruled out. We feel that the results of other tests such as gallium scan, IVP, or barium enema are not sufficiently specific to indicate surgery.

Cholelithiasis and cholecystitis in childhood.

In this review of 50 children with gallbladder disease in Houston, Texas, there were an equal number of patients with and without hemolytic disease and a slight predominance of males in both groups. Nearly all had been symptomatic for a prolonged period before diagnosis and cholecystectomy. Common early diagnoses were hemolytic crisis and appendicitis. Ultrasonography has become a very accurate tool for the preoperative evaluation of these children. Once the diagnosis of cholelithiasis is established, elective cholecystectomy is the treatment of choice.

Morgagni hernias during infancy: presentation and associated anomalies.

Morgagni hernias during infancy are associated with significant respiratory symptoms as well as other congenital anomalies, particularly congenital heart disease. During the past 6 years we have had the opportunity to treat five infants less than 1 year of age with Morgagni hernias. A review of the literature revealed an additional seventeen detailed case reports of infants with Morgagni hernias. Each of our patients was symptomatic as were all but two of those previously reported. These patients presented with tachypnea, dyspnea, and cyanosis which was very similar to the presentation of Bochdalek hernias seen during infancy. Thirteen of 16 patients in whom detailed descriptions of associated anomalies were given including four of our own patients, had significant congenital anomalies. Nine involved the heart, including three infants with dextracardia, three with ventricular septal defects, and two with anomalous pulmonary venous return. Five infants were retarded, including three with Trisomy 21. Two of the previously reported patients had large omphaloceles and other stigmata of Cantrell's syndrome. All but one of the patients reported, including ours, had a hernia sac. The liver, colon, and small bowel were most commonly found in the hernia sac; however, the presence of the stomach and spleen have been reported. While both the transabdominal and transthoracic approaches to Morgagni hernias have been advocated and, indeed, repair of the defect can be accomplished through both approaches, the transthoracic approach allows better exposure of incarcerated viscera, particularly the frequently found anomalous left lobe of the liver.

Resection with primary anastomosis for necrotizing enterocolitis.

Primary anastomosis following the resection of bowel for necrotizing enterocolitis (NEC) can be done with acceptable morbidity and mortality rates. Twenty-seven patients are reported with three deaths. Primary anastomosis will avoid problems seen with small-bowel stomas. The concomitant ligation of a significant patent ductus arteriosus (PDA) should be considered for patients undergoing abdominal procedures for NEC.