Management of complicated biliary disease in the pediatric population.

BACKGROUND: Cholesterol stones and biliary dyskinesia have replaced hemolytic disease as the primary indication for pediatric cholecystectomy. This study looks at the cohort of pediatric patients with complicated biliary disease, defined as choledocholithiasis and/or gallstone pancreatitis, to determine the incidence and best treatment options. METHODS: A retrospective review of all cholecystectomies performed over 15 years admitted to the surgical service at a single free-standing children's hospital was performed. Patient factors, indications for cholecystectomy, and final treatment were recorded. Complicated gallbladder disease was defined as having image-confirmed choledocholithiasis or gallstone pancreatitis. High-risk patients were those with imaging that demonstrated definitive choledocholithiasis or cholelithiasis with common bile duct enlargement. Low risk patients were those with cholelithiasis or gallbladder sludge on imaging combined with an elevated bilirubin and/or lipase. RESULTS: A total of 695 cholecystectomies were performed over the 15-year time period. Average patient age was 13.4 years. Of the 695 cholecystectomies, 457 were performed for stone disease (66%) (64 hemolytic) and 236 (34.0%) were performed for biliary dyskinesia. Hundred and three (14.8% of all cholecystectomies, 22.5% of those with stone disease) presented with choledocholithiasis and/or gallstone pancreatitis (complicated disease). In high-risk patients, 28/47 (59.6%) underwent endoscopic retrograde cholangiopancreatography/sphincterotomy. In low-risk patients (no choledocholithiasis or common duct enlargement), 13/56 (23.2%) required endoscopic retrograde cholangiopancreatography/sphincterotomy (P < .05). The indication for endoscopic retrograde cholangiopancreatography after cholecystectomy was choledocholithiasis and none of these patients had bile leak complications. CONCLUSION: The incidence of pediatric complicated biliary disease due to cholesterol stones is equal to that of adults. These data suggest that a patient with imaging evidence of choledocholithiasis or common bile duct enlargement may require endoscopic retrograde cholangiopancreatography, dependent on clinical course, and this should be strongly considered before cholecystectomy. Those without such radiographic findings can undergo laparoscopic cholecystectomy and have postoperative endoscopic retrograde cholangiopancreatography if needed.

Radiographic Determination of Skeletal Maturity Guides Optimal Timing of Bar Removal After Minimally Invasive Repair of Pectus Excavatum.

BACKGROUND: A small number of patients treated with minimally invasive correction of pectus excavatum recur after bar removal. This risk appears to be greater in younger children who continue to grow following bar removal. METHODS: We propose the use of wrist films to determine skeletal maturity and delay bar removal until it is completed. This is not possible in very young patients (less than 14 years of age) or necessary in patients older than 19. RESULTS: In the 14-year to 18-year age group, we have used wrist films to determine skeletal maturity in 25 patients. Ten patients (age 14-18) demonstrated full maturation, and their bars were removed at 2 years. Five patients had films that demonstrated nearly closed growth plates, and those bars were removed 6 months later (2.5 years post-insertion). Ten patients had 2 sets of films taken, initially at 2 years post-operation demonstrating open growth plates. Films 12 months later showed skeletal maturation. Their bars were removed at 3 years post-operation. There were no recurrences with an average follow-up of 3 years. DISCUSSION: Radiographic determination of skeletal maturity may be used as a guide to the timing of bar removal following the correction of pectus excavatum.

Results of pectus excavatum correction using a minimally invasive approach with subxyphoid incision and three-point fixation.

OBJECTIVES: This study reviews the results of our previously described modification of the minimally invasive (Nuss) procedure for correction of pectus excavatum. It utilizes a subxyphoid incision with central fixation to maximize safe bar passage and minimize bar displacement. METHODS: Consecutive patients corrected with the modified Nuss procedure between 2010 and 2015 form the basis of this study. RESULTS: During the study period, 73 patients had correction of their pectus excavatum by the modified Nuss procedure, utilizing subxyphoid incision and central fixation. Average age was 14.3 (range 8-19). 54 patients were male, 19 female. The average Haller index was 4.3 (range 3.2-7.2). No episodes of cardiac perforation, hemothorax or significant pneumothorax were recorded. Bar displacement occurred in two patients (2.7%) with one late recurrence after bar removal. CONCLUSIONS: Our modification of the Nuss procedure is effective at preventing intrathoracic complications and cardiac perforation. Central fixation had a lower rate of bar displacement compared to published reports. Additional efforts are needed to further reduce bar displacement.

Etiology and incidence of pediatric gallbladder disease.

PURPOSE: The spectrum of pediatric biliary tract disease is changing. The goal of this study was to examine the causes and comorbidities of pediatric gallbladder disease at our institution. METHODS: We performed a retrospective chart review on consecutive patient at Kosair Children's Hospital who underwent cholecystectomy over a 9-year time period ending in 2012. RESULTS: Among the 453 patients in the study group, the average age was 13.3 years and 67.2% were female. Indications for cholecystectomy were gallstones in 285 (63%) and biliary dyskinesia in 140 (33%). Of the patients with gallstones, 68 children (15%) had hemolytic disease. Although the number of cholecystectomies for hemolytic disease was relatively stable throughout our study, the number for biliary dyskinesia and non-hemolytic (cholesterol) cholelithiasis rose by 63% and 216%, respectively. Average body mass index (BMI) for patients with non-hemolytic (cholesterol) stones and biliary dyskinesia were significantly greater than the average BMI for patients with hemolytic stones (P < .0001). In addition, the average BMI for children with non-hemolytic (cholesterol) stones was greater than the average BMI with biliary dyskinesia (P < .0001). CONCLUSION: Symptomatic gallbladder disease increased over the study period. Biliary dyskinesia and children with non-hemolytic disease are responsible for this increase.

Lateral pediatric ectopic thyroid.

We present a case of a left-sided neck mass in an 8-year-old female that proved to be ectopic thyroid tissue. After excision, this patient continues to do well and is euthyroid without recurrence after 8-year follow-up. This is a very rare anomaly in a child, especially in conjunction with a normally located thyroid that is functional. We reviewed the literature of displaced ectopic thyroid tissue.

Recurrent neutropenic enterocolitis in a pediatric patient.

Management of an 8-year-old boy with Hodgkin lymphoma is presented. The patient had several recurrences of neutropenic enterocolitis and eventually required ileocecectomy. A review of the literature on this difficult problem affecting pediatric oncology patients is presented.

Diverticulitis in a child with Williams syndrome: a case report and review of the literature.

Diverticulitis is rare in pediatric patients and often associated with a more complicated course than that seen with adult patients. Certain syndromes, such as Williams syndrome, have been associated with an increase incidence of diverticular disease. We describe a 9-year-old boy with Williams syndrome who presented with rectal bleeding secondary to sigmoid diverticulitis. This case represents the youngest known patient with diverticulitis. Patients with this disorder who present with chronic or recurrent abdominal pain should be evaluated for diverticular disease and its potential complications.

Gastric trichobezoar: abdominal mass in a child with sickle cell disease.

Abdominal pain is a frequent occurrence among the pediatric population and can be a diagnostic challenge. Trichobezoar is a differential diagnosis that is often neglected. Different from previously reported cases, we present a 3-year-old girl with sickle cell disease with complaints of acute abdominal pain, suspecting sickle cell splenic sequestration. The child presented to the emergency department with sharp epigastric pain and an associated palpable upper abdominal mass. This case illustrates a large obstructing gastric trichobezoar and summarizes both the diagnostic modalities and treatment.

Laparoscopic Ladd procedure: a minimally invasive approach to malrotation without midgut volvulus.

The management of intestinal malrotation without midgut volvulus is controversial. Some advocate the Ladd procedure in all patients with malrotation, whereas others propose a more selective approach. We attempted the laparoscopic Ladd procedure on nine patients who were diagnosed with intestinal malrotation without volvulus. Patient records were retrospectively reviewed. Data were collected on patient presentation, operative procedure, hospital course, and outcome. The laparoscopic Ladd procedure was successfully completed in eight patients (aged 10 weeks to 25 years). One patient required conversion to an open procedure. Operative time averaged 111 minutes (range, 77-176 minutes). Hospital stay ranged from 3 to 5 days (average, 3.6 days). All patients were discharged home on a regular diet. There was one complication and no deaths. Eight patients had complete resolution of their symptoms. The laparoscopic Ladd procedure is a safe and effective procedure for infants, children, and adults who have intestinal malrotation without midgut volvulus. The operative times, hospital stay, and clinical outcomes were acceptable. We recommend that laparoscopic intervention be considered in patients with intestinal malrotation without volvulus. Intestinal malrotation occurs along a wide spectrum of anatomic variants and clinical presentations. The management of malrotation without midgut volvulus remains controversial. Most advocate performing the Ladd procedure on all patients found to have malrotation because there is no way to know which of these patients will develop catastrophic midgut volvulus. Some propose a more selective approach because of the morbidity associated with operative intervention. There have been a number of small series and case reports describing the use of laparoscopy to diagnose and correct malrotation. Proponents of this method point out its minimally invasiveness, patients' quick recoveries, and successful outcomes. We describe our experience with the laparoscopic Ladd procedure and its long-term results.

Undescended ovaries: a clinical review.

Undescended ovaries are frequently seen in conjunction with uterine malformations and are typically found during the course of an infertility evaluation. Other important clinical signs may prompt evaluation in an adolescent patient, though this may also be an incidental finding. An understanding of embryologic features is critical for management. It is additionally important to explain to patients and families that this is a normal developmental variant of the ovary that does not require removal in the absence of concerning pathology.

Hirschsprung's disease in an infant with colonic atresia and normal fixation of the distal colon.

The coexistence of colonic atresia and Hirschsprung's disease presents a diagnostic and therapeutic challenge. Colonic atresia is quickly recognized, and the majority of patients are diverted shortly after birth. The diagnosis of coincident Hirschsprung's disease usually is made after anastomotic failure after restoration of intestinal continuity. A recent compilation of these patients has suggested that Hirschsprung's disease may be predicted on the basis of nonfixation of the colon distal to the atresia. However, we recently cared for an infant with transverse colonic atresia and total colonic aganglionosis associated with normal orientation and fixation of the distal colon.

Primitive neuroectodermal tumor of the sternum in a child: resection and reconstruction.

Pediatric primary malignancies of the sternum are rare. They represent less than 1% of all bone tumors. Primitive neuroectodermal tumor of the chest wall or Askin's tumor is more often seen in the ribs than in the sternum. Surgical treatment involves resection of the tumor followed by primary reconstruction. We report the case of a 16-year-old adolescent boy who was diagnosed to have primitive neuroectodermal tumor of the sternum and underwent resection with primary reconstruction of the anterior chest wall. We describe for the first time the use of a 2-mm Gore-Tex Dualmesh plus biomaterial (W.L. Gore and Associates, Flagstaff, Ariz) and locking mandibular multiperforated titanium plates with screws (Stryker-Leibinger, Freiburg, Germany) for primary sternal reconstruction in a child. Despite having a wide resection, he did not require postoperative ventilation and had an uncomplicated recovery. The reconstructed chest wall has a normal contour with normal pulmonary physiology.

Müllerian agenesis and ovarian torsion. A case report and review of literature.

A case report of a premenarcheal patient with an ovarian torsion and müllerian agenesis is presented. An 11-year-old prepubertal girl presented with severe left lower quadrant abdominal pain and mild rebound. A computed tomography showed a normal appendix; an ultrasound showed a left ovary measuring 3 x 2 cm with multiple 0.5-mm simple cysts. A diagnostic laparoscopy showed the left ovary tube to be twisted, but the uterus was absent, and the right ovary and tube were not visualized in the appropriate location; instead, a right adnexal structure was buried in the right sidewall. The ovary was untwisted and fixed to the pelvic sidewall. Chromosomes were 46,XX, and her hormonal evaluation was normal. Ovarian torsion and müllerian agenesis are rarely reported. An association between the lax attachment of the adnexa and torsion may be a contributing factor in this condition.

University of Louisville experience and contributions to pediatric trauma care.

Care of the injured patient has been a focal point of surgical training at the University of Louisville. Under the direction of Dr. Hiram C. Polk, Jr, M.D., the Trauma Service has received national recognition for excellence in patient care and surgical research. This experience has provided a foundation for further endeavors in trauma care by program graduates. Summaries of contributions to the peer-reviewed literature or to hospital programs by former trainees who specialized in pediatric surgery are presented. Topics include the treatment of pediatric solid-organ injury, pelvic fracture, pellet-gun injury, and caustic cutaneous burns. The impact of the pediatric Trauma Service at Kosair Children's Hospital in Louisville, KY, with an emphasis on compassionate care and the development of a bereavement intervention program, also is presented.

Cathlink 20: a subcutaneous implanted central venous access device used in children with sickle cell disease on long-term erythrocytapheresis--a report of low complication rates.

BACKGROUND: Experience with the use of central venous access device (CVAD) in children with sickle cell disease (SCD) on hypertransfusion is limited and published studies report wide variability in the rates of CVAD-associated complications. PROCEDURE: In this study, a total of 18 Cathlink 20 ports (Bard Access systems, Salt Lake City, UT) were implanted in 15 patients aged 7-20 years with SCD for 19, 230 catheter patient days. RESULTS: No peri-operative complications were observed. Three episodes of catheter occlusion requiring replacement occurred in two patients for an observed rate of 0.16 per 1,000 catheter patient days for thrombotic occlusion. One patient required port replacement and another patient required replacement twice. In 13 out of 15 patients, the median duration of port use was 45 months. No episode of catheter-associated bacteremia or catheter tunnel infections was observed. CONCLUSIONS: Comparing our results with other reported studies of CVAD-associated complications in patients with SCD, we observed a lower rate of complications with the use of Cathlink 20 ports. These findings may be partly related to the design of the port and partly to a dedicated group of nurses accessing the ports in a controlled environment in an apheresis unit.

Ruptured hemorrhagic cyst in an undescended ovary.

The authors report on a patient with an uncommon congenital anomaly of bilateral undescended ovaries and tubes. She presented with an acute abdomen at 13 years of age. A computed tomography scan showed a cystic mass suspicious for congenital intestinal duplication. At the time of laparoscopy, she was found to have a bicornuate uterus and bilateral undescended ovaries with a ruptured right hemorrhagic ovarian cyst. This case shows that although a ruptured ovarian cyst can occur in such malpositioned organs, it can pose difficult diagnostic challenges. Suppression of the ovaries with the combined oral contraceptive pill may help to prevent further ovarian cyst development.