Surgical treatment of infantile achalasia: a case report and literature review.

Allgrove syndrome is a rare genetic disorder typically manifested by alacrima, achalasia, and adrenal insufficiency, and is one of the rare causes of achalasia in infants. While the gold standard for achalasia treatment in adults is an esophageal myotomy with fundoplication, a standard treatment for infantile achalasia remains undetermined due to the low number of reported cases and rarity of the disease in this age group. We report a 7.7 kg infant with achalasia secondary to Allgrove syndrome who was successfully treated by Heller myotomy and simultaneous Toupet fundoplication. This case represents one of the smallest patients to ever be treated for achalasia and highlights the role that primary surgical therapy may have for the infantile variant of the disease.

Needle core biopsy in the diagnosis of pediatric thyroid neoplasms: a single institution retrospective review.

PURPOSE: Our institution routinely utilizes needle core biopsy (NCB), instead of fine needle aspiration, in the evaluation of pediatric thyroid nodules. This practice initially arose from limited cytopathology services in our hospital. Given the lack of information regarding the utility of NCB in diagnosing pediatric thyroid neoplasms, we set out to review our institution's experience with this technique. METHODS: We performed a single institution retrospective chart review of all children who underwent thyroidectomy for primary thyroid pathology. RESULTS: Seventy-four patients, with a mean age of 12.9 ± 4.5 (SD) years, underwent partial or total thyroidectomy between 2002 and 2010. Seven of these patients had medically refractive hyperthyroidism. The remaining 67 patients had one or more thyroid nodules as identified by ultrasound. 24 (36 %) of these cases were malignant on final pathology. 14 (58 %) of the malignant cases were papillary thyroid carcinoma. 46 of the thyroid nodule cases underwent pre-operative NCB. Biopsy results for these patients were non-diagnostic in 6 (13 %), benign in 11 (24 %), atypical in 17 (37 %), and malignant in 12 (26 %). There were no complications arising from NCB. Sensitivity of NCB for diagnosing papillary carcinoma (PC) and follicular neoplasm was calculated at 0.88 (0.47-1.0, 95 % CI) and 0.84 (0.60-0.97, 95 % CI), respectively. Of the 28 patients not undergoing preoperative NCB, 12 underwent hemithyroidectomy, with one patient (8 %) requiring completion thyroidectomy for PC. Overall, the sensitivity of NCB in diagnosing PC and follicular thyroid neoplasms was 0.85 (0.55-0.99, 95 % CI), while the specificity was 0.63 (0.42-0.82, 95 % CI). CONCLUSIONS: Needle core biopsy appears to have a low rate of associated complications, and its sensitivity for diagnosing PC and follicular neoplasm is comparable to what has been reported for fine needle aspiration biopsy in a similar patient population.

Hepatocellular transplantation in acute hepatic failure and targeting genetic markers to hepatic cells.

Orthotopic liver transplantation (OLT) represents the only therapeutic option for many patients with end-stage liver disease as well as many inborn genetic errors of hepatic metabolism. Despite dramatic progress in methods for OLT, the utilization of this procedure is limited by its considerable morbidity and mortality, by a chronic shortage of organs for transplant, and by difficulty arranging funding for many patients. Many children with fulminant hepatic failure do not receive OLT because this technology is unavailable or unaffordable. Hepatocellular transplantation (HCT), in which isolated, heterologous hepatocytes from a donor liver would be infused into the diseased organ in order to provide essential hepatic functions, could provide a much needed therapeutic alternative to OLT in the treatment of some causes of hepatic insufficiency. Experiments in animals have demonstrated that several genetic deficiencies of hepatic metabolism as well as experimental induced hepatic failure in animals can be reversed by HCT. Despite this experience, HCT has never been attempted in human subjects. This protocol represents the first proposed clinical trial of HCT. We are proposing a clinical trial in which HCT would be attempted as a therapeutic intervention in children with acute hepatic failure who have no other medical or surgical options. This proposal is intended to establish surgical methods for HCT and to evaluate the feasibility of this procedure for treating hepatic disease in humans. It is our expectation that HCT may provide short-term support for patients awaiting organ availability, a "bridge to recovery" allowing patients with fulminant hepatic failure to recover, or a long-term repopulation of the patient's liver with healthy donor cells. One of the major limitations of many animal studies in HCT is that, since the donor hepatocytes are often indistinguishable from those of the host, it has often been difficult to demonstrate a clear correlation between engraftment and the therapeutic effect. In order to verify engraftment independent of the therapeutic response, we propose to "mark" the donor hepatocytes by transducing these cells with a recombinant retroviral vector (LNL6) carrying a marker gene (NEO-R, neomycin phosphoribosyl transferase). The presence of this marker will enhance the ability to identify transplanted cells in the host using assays for the NEO-R gene or transcribed NEO-R mRNA. The LNL6 vector has been approved for human use and has been used as a marker gene for transplanted cells in human subjects without any reported adverse effects. We would like to emphasize that this is a proposal with therapeutic intent.(ABSTRACT TRUNCATED AT 400 WORDS)

In situ hybridization utilizing a Y chromosome DNA probe. Use as a cell marker for hepatocellular transplantation.

Research in hepatocellular gene therapy requires a consistently reproducible cell marker to detect transplanted hepatocytes. We have used a Y-specific genomic DNA probe to accomplish this goal. This technique enables the identification of transplanted male cells in recipient female tissues. Donor hepatocytes from male mice were transplanted into female mice via splenic injection. Recipient mouse livers were harvested 1, 24, and 48 hr after transplant. Transplanted (male) hepatocytes were detected in liver biopsy sections using in situ hybridization with the Y chromosome probe.

Successful use of Tesio catheters in pediatric patients receiving chronic hemodialysis.

Semipermanent venous catheters remain the most commonly used access for chronic hemodialysis (HD) in pediatric patients. The recent availability of Tesio catheters in 7 and 10 F has expanded available HD catheter options for children and adolescents. We report our experience with Tesio catheter survival, complications, and effect on dialysis adequacy in comparison to standard dual-lumen (DL) catheters in our pediatric HD patients. Demographic data were similar between the two groups. Overall actuarial survival was significantly longer for Tesio versus DL catheters (46% versus 0% at 1 year; P = 0.003). A comparison of smaller catheters (7 F Tesio catheter, 8 or 10 F DL catheter) showed that smaller Tesio catheters had a significantly longer survival (median survival, 244 versus 13 catheter-days; P < 0.01). Tesio 10 F catheters also survived significantly longer than the larger 11.5 and 12 F DL catheters (P < 0.02). Catheter sepsis occurred less frequently with Tesio catheters (one episode/20 catheter-months) than DL catheters (one episode/5 catheter-months) despite the longer duration of Tesio catheters. Adequate dialysis (single-pool Kt/V > 1.2) was delivered with the same frequency, but for a longer duration with Tesio catheters (76% +/- 32% over 100 monthly measurements versus DL catheter, 57% +/- 45% over 54 monthly measurements). Our clinical practice was to replace cuffed catheters when adequate dialysis could not be delivered. We conclude that Tesio catheters provide superior performance compared with DL catheters in terms of catheter survival, infection rates, and duration of adequate performance.

The surgical implications of chronic granulomatous disease.

BACKGROUND: Chronic granulomatous disease (CGD) of childhood is a rare congenital abnormality of the phagocyte NADPH oxidase system. Affected neutrophils and macrophages have an ineffective respiratory burst and cannot destroy certain phagocytized bacteria and fungi. CGD patients usually present with recurrent pyogenic and fungal infections. Catalase-positive bacteria are frequently involved, since they metabolize the hydrogen peroxide they produce, making it unavailable for augmentation of microbicidal activity in CGD neutrophils. Afflicted patients also have a tendency to form granulomas, which can lead to obstruction of the gastrointestinal and genitourinary tracts. METHODS: Charts of 10 patients with CGD were reviewed for age at diagnosis, surgical procedures, complications of these procedures, and medical treatment. RESULTS: Eight of the 10 children were male. The average age at first presentation was 18 months (range 2 days to 9.8 years). Each child developed a mean of 9.9 infections and an average of 1.4 infections per year. All required surgical procedures, with an average of 2.9 procedures each. Five children had operative procedures for infections that preceded the diagnosis of CGD. The procedures performed most frequently were incision and drainage of soft-tissue abcesses (7) or perirectal abscess (3), thoracentesis (3), and bronchoscopy (3). Three children had poor wound healing following surgery. Two developed partial gastric outlet obstruction which resolved with antibiotic therapy. One developed granulomatous cystitis with obstruction which responded to antibiotics. CONCLUSIONS: Since patients with undiagnosed CGD may present with surgical problems, surgeons need to be familiar with this condition. The diagnosis should be suspected in children who have recurrent or unusual infections or unexplained problems with wound healing.

Meta-analysis of the risk of metachronous hernia in infants and children.

BACKGROUND: Inguinal herniorrhaphy is the most common general surgical procedure performed in children. The presence of a contralateral patent processus vaginalis forms the basis of the recommendation for contralateral exploration in patients undergoing unilateral herniorrhaphy. However, a patent processus vaginalis does not necessarily go on to become a clinically apparent inguinal hernia. METHODS: All published pediatric series, in which patients underwent unilateral inguinal hernia repair and were evaluated for the development of a metachronous hernia, were included. The incidence of and risk factors associated with development a metachronous hernia were evaluated with meta-analysis. RESULTS: There were 15,310 patients ranging in age from birth to 16 years, including premature infants. Of these, 1,062 patients (7%) developed a metachronous hernia. Gender and age were not risk factors. There was an 11% risk of metachronous hernia if the original hernia was on the left side, a risk that was 50% greater than if the original hernia was on the right. Of patients who developed a metachronous hernia, 90% did so within 5 years. The complication rate of metachronous hernia was 0.5%. CONCLUSION: There is no role for routine contralateral groin exploration. High-risk infants and children, especially those who undergo left inguinal herniorrhaphy, may benefit from contralateral groin exploration. If a patent processus vaginalis is found, it should be ligated. Patients who do not undergo contralateral groin exploration should be followed up for 5 years.

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.

Right lower quadrant pain in children caused by omental infarction.

BACKGROUND: Omental infarction is a rare cause of abdominal pain, with fewer than 300 cases reported in the literature. Only 15% of reported cases occur in the pediatric population. We present our experience with 18 children diagnosed with omental infarction admitted to Texas Children's Hospital over a 15-year period. STUDY DESIGN: A retrospective review of clinical records, diagnostic images, and pathologic findings was carried out for all children diagnosed with isolated omental infarction from 1986 to 2000 in order to analyze presenting signs and symptoms, preoperative imaging, surgical management, and postoperative outcomes. RESULTS: Eighteen children were treated for isolated omental infarction. There were 12 boys and 6 girls with an average age of 7.5 years (range 2 to 13). All patients presented with acute onset of right lower quadrant pain. Only 5 of 18 (24%) had associated gastrointestinal symptoms. The average temperature at presentation was 99.4 degrees F (+/- 0.78). The average white blood cell count was 11.4 (+/- 4.4). Fourteen patients had ultrasonographys performed preoperatively: 6 of 14 incorrectly diagnosed appendicitis, 4 of 14 were nondiagnostic and 4 of 14 correctly diagnosed omental infarction. Two of 18 patients underwent computed tomography scans, which were diagnostic for omental infarction. Resection of the infarcted omentum was performed in all patients and appendectomy in 16 of 18. Thirteen patients underwent an open procedure, 5 were performed laparoscopically. There were no postoperative complications. All patients had resolution of pain postoperatively and were discharged an average of 3.0 (+/- 0.9) days after admission. CONCLUSIONS: Omental infarction is an uncommon cause of right lower quadrant pain in children and is often diagnosed as appendicitis preoperatively. Ultrasonography and computed tomography can be diagnostic. Surgical resection of the infarcted omentum results in immediate resolution of pain with no morbidity.

Gene therapy in pediatric surgery.

Gene transfer has become a reality during the last decade. The basic principles of molecular biology, gene transfer, and the problems associated with this new technology have direct implications for the treatment of pediatric surgical patients. Research into applications of gene transfer in the treatment of disorders of the bone marrow, cancer, hemophilia, hepatic and metabolic disease, cystic fibrosis, and vascular disease is rapidly progressing. These areas of research may dramatically affect the care of pediatric surgical patients in the future.

The subcutaneous spleen: a new model for percutaneous access to the portal venous system.

Correction of hepatic failure or metabolic disease by hepatocellular transplantation may require replacement of up to 10% of enzyme function with normal or genetically corrected cells. Although single injections of hepatocytes have not been able to consistently achieve this level of functional replacement in experimental models, multiple injections should theoretically be able to approach this goal. Delivery of multiple doses of hepatocytes to the spleen in experimental animals is complicated by the need to perform multiple laparotomies. By relocating the spleen to a subcutaneous position, the authors have designed an animal model to facilitate multiple splenic injections without the need for repeated celiotomies. Because it is in the prefascial plane, multiple hepatocyte injections can be delivered to the spleen percutaneously using only minimal sedation. Bleeding secondary to needle puncture is contained by a pseudocapsule which develops around the spleen. No statistical difference in the degree of hepatocyte migration to the liver has been demonstrated in animals transplanted via the subcutaneous spleen compared with animals transplanted by laparotomy (0.51% vs. 0.56%, p = .785).

Transuterine puncture of the fetal stomach provides access to the small bowel in the rabbit.

The prevention of perinatal complications of congenital gastrointestinal (GI) diseases such as cystic fibrosis may require prenatal treatment. New Zealand White rabbits were evaluated as a potential animal model to study gastrointestinal anatomy and transit in the fetus. The lengths of the GI tract of fetuses at 21/31 and 28/31 days gestation were established, and gastric volume was measured. Gastric volume at 28 days gestation averaged 2.6 mL, adequate to permit instillation of a solution into the fetal stomach. A study was then carried out to establish gastric emptying and delivery of the solution into the small bowel. Using ultrasound guidance, the stomachs of 26 fetuses from 7 litters were punctured and 0.5 mL of dilute barium was injected. A cesarean section was performed 4 h later and the progression of barium though the GI tract was measured. In 18/26 (69.2%) of the fetuses barium was successfully delivered to the lumen of the stomach. In these 18 fetuses, barium progressed to the duodenum in 15 (58%), the jejunum in 13 (50%), and the ileum in 8 (31%). The stomach of the 3.5- to 4-week-old fetus is large enough to allow transuterine delivery of a solution of dilute barium. Gastric and intestinal motility in the 25-day-old rabbit fetus is coordinated and results in delivery of barium to the small bowel in 50% of animals successfully injected. The results suggest that the rabbit is an acceptable model for the study of gastrointestinal delivery of therapeutic drugs or genes to the fetus.

Malrotation presenting beyond the neonatal period.

The diagnosis of malrotation is easily made in the neonatal period, but is often delayed in older patients. Among 82 patients treated for malrotation in this institution, 45 patients presented with symptoms related to their malrotation, seven were diagnosed at exploration for concomitant intrinsic duodenal obstruction, and 30 patients had malrotations discovered as incidental findings at laparotomy or autopsy. Among the 45 symptomatic patients, 25 (56%) underwent surgery in the first month of life, whereas 20 patients (44%) underwent surgery at an older age. In this last group, the mean age at surgery was 51.5 months (range, 2 months to 16 years), the mean age of onset of symptoms was 2 years (range, 0 to 15 years) and the mean delay in diagnosis was 1.7 years. Although bilious vomiting was the presenting symptom among all patients undergoing surgery in the neonatal period, clinical features of older patients included intestinal obstruction (7), chronic abdominal pain (4), malabsorption/diarrhea (3), peritonitis/septic shock (2), solid food intolerance (1), common bile duct obstruction (1), abdominal distention (1), and delayed transit postappendectomy (1). The frequency of midgut volvulus was equal among both groups. Unusual forms of malrotation were more frequent in patients undergoing surgery beyond the neonatal period. In this group there was evidence of chronic venous and lymphatic obstruction with one case of superior mesenteric vein thrombosis and two cases of intestinal gangrene. A Ladd's procedure was performed in all cases and the most frequent postoperative complication was adhesive intestinal obstruction. There were no deaths. Awareness of the unusual presentation in patients who present beyond the neonatal period may help reduce delays in diagnosis and surgical treatment. We believe that laparotomy is indicated in all patients with malrotation, even if they are asymptomatic.

Sirenomelia in an identical twin: a case report.

Sirenomelia, or the mermaid syndrome, is the most extreme example of the caudal regression syndrome. It invariably presents with lower limb fusion, sacral and pelvic bony anomalies, absent external genitalia, anal imperforation, and renal agenesis or dysgenesis. Because of the resultant oligohydramnios, these infants most often have Potter's facies and pulmonary hypoplasia. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation. It has been suggested that the association of the most extreme form of caudal regression, sirenomelia, with monozygotic twinning may represent developmental arrest of the primitive streak, with creation of a second primitive streak that gives rise to the usually normal twin. The embryology of the various presentations of the caudal regression syndrome may be further delineated by studying infants with this dramatic and fatal syndrome.

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)

Yolk sac tumor identified at autopsy after surgical excision of immature sacrococcygeal teratoma.

Although the predictive value of immature elements in sacrococcygeal teratomas in unclear, there are reports of malignant recurrence after surgical resection of immature sacrococcygeal teratomas. The recurrent tumors are presumed to arise from small residual malignant foci not identified at the time of surgical resection. In this report a premature female infant was delivered at 29 week's gestation with a large sacrococcygeal teratoma. The tumor weighed 1,350 g. It was largely cystic with a focal nodular and variegated appearance. Histologically, the tumor was a grade 1 immature teratoma with a predominance of neuroglial elements. No malignant elements were identified in any of 26 sections examined. The infant died intraoperatively of cardiovascular complications related to the large vascular supply of the tumor but had a grossly complete resection of tumor. At autopsy, a small microscopic focus of yolk sac tumor was identified adjacent to the sacrum anteriorly. Had the infant survived, this focus might well have been a source for malignant recurrence.

Surgical complications of the hemolytic-uremic syndrome.

Hemolytic-uremic syndrome (HUS) of childhood is a triad of acute hemolytic anemia, thrombocytopenia, and acute renal failure associated with a gastrointestinal prodrome. From 1977 to 1988, 134 patients with HUS were admitted to this institution. All patients presented with abdominal pain and diarrhea, which was virtually always bloody. Seventy-eight patients (60%) required dialysis. Five patients died (4%). One patient died as a result of colon perforation, the other four patients died of other nonsurgical complications of HUS. Three patients underwent exploratory laparotomy. One patient had a hemoperitoneum from mesenteric and transmural bleeding of the entire intraabdominal colon. Another patient had undergone surgery elsewhere for presumed intussusception with pancolitis found at exploration. Fourteen days postoperatively, he had a spontaneous perforation of the transverse colon. The third patient presented with pancolitis and perforation of the transverse colon. Despite surgical intervention he died on the sixth postoperative day. One other patient was treated conservatively for pancreatitis, which developed 3 weeks after her presentation with HUS. Complications requiring surgical intervention in HUS are rare, potentially lethal, and usually involve the colon.

Mesenteric cysts in children.

Mesenteric cysts are rare intraabdominal lesions of childhood that may vary in presentation from an asymptomatic mass to an acute abdomen. From 1970 to 1990, 15 children were diagnosed and treated for mesenteric cysts at Ste Justine Hospital in Montreal. The ages ranged from birth to 18 years (average age, 6 years). There were 9 boys and 6 girls. Ten patients required emergency surgery and five underwent elective surgery. The main presenting symptom was abdominal pain. Ten patients had preoperative ultrasounds that were diagnostic for a cystic mass in all patients. The second most frequent preoperative diagnosis was appendicitis. The cysts were located in the small bowel mesentery in 5 cases, the base of the mesentery with retroperitoneal extension in 4 cases, the transverse mesocolon in 4 cases, and the gastrocolic ligament in 2 cases. Operative procedures performed included complete cyst excision (9 patients), complete excision with intestinal resection (5 patients), and drainage of the cyst (1 patient). The only recurrence in this series occurred after drainage. One other patient had recurrence of a mesenteric cyst following resection performed elsewhere. Mesenteric cysts are rare in children, are usually symptomatic, and are most commonly misdiagnosed as acute appendicitis. Accurate preoperative diagnosis is possible with current ultrasonographic imaging techniques. Complete cyst resection is the procedure of choice and results in an excellent outcome.

Diagnosis and treatment of gastroesophageal reflux in 500 children with respiratory symptoms: the value of pH monitoring.

Gastroesophageal reflux (GER) usually presents with digestive symptoms, failure to thrive, and/or respiratory symptoms. During the 8-year period from 1981 to 1989, 1,153 children underwent 20-hour pH monitoring to assess GER. All patients were graded using the scoring system of Euler and Byrne. Patients were classified as severe (score greater than 50), moderate (score 25 to 50), and normal (score less than 25). Five hundred (43.3%) of these patients presented with respiratory symptoms including apnea, cyanosis, or "near miss" sudden infant death syndrome (36%), poorly controlled asthma (28%), recurrent bronchopneumonia (13%), bronchiolitis (9%), and miscellaneous symptoms such as intermittent dyspnea, chronic cough, and stridor (12%). Eight patients (2%) had cystic fibrosis. The ages ranged from 1 month to 20 years (mean, 19.5 months). Twelve patients had technically inadequate studies and were excluded. Severe reflux was present in 156 patients (31%) and moderate reflux in 159 patients (31%). All patients were treated initially by medical therapy for a minimum of 8 weeks. The majority of patients (81%) had resolution of their symptoms with change in position, thickened feedings, and, when indicated, additional therapy with metoclopramide, cisapride, or domperidone. Most of these patients were found to have a specific position, usually prone, which decreased reflux. The remaining 57 patients had documentation of persistent reflux by pH monitoring and underwent an antireflux procedure. Of those patients undergoing surgery 51 had severe reflux and 6 had moderate reflux. Forty-four patients had a posterior 270 degrees wrap (Toupet), 10 had a 360 degrees wrap (Nissen), and 3 had an anterior 180 degrees wrap (Boix-Ochoa).(ABSTRACT TRUNCATED AT 250 WORDS)

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.