Indications and outcomes for tunneled central venous line placement via the axillary vein in children.

PURPOSE: To assess the indications, safety and outcomes of tunneled central venous catheters (CVCs) placed via a cutdown approach into the axillary vein in children, an approach not well described in this population. METHODS: A retrospective cohort study was performed on pediatric patients who received CVCs via open cannulation of the axillary vein or one of its tributaries between January 2006 and October 2016 at two hospitals. RESULTS: A total of 24 axillary CVCs were placed in 20 patients [10 male (42%); mean weight 7.0 kg (SD 2.9); mean age 10 months (SD 6)]. The most common indications for axillary vein access included neck or chest wall challenges (tracheostomies or chest wall wounds) (n = 18). The median duration of line placement was 140 days (IQR 146). The most common indications for removal were completion of therapy (n = 7, 39%) and infection (n = 5, 28%). There were no early complications. Long-term complications included infection (n = 5) or catheter malfunction (n = 3). CONCLUSIONS: Tunneled CVC placement via a cutdown approach into the axillary vein or its tributary can be an effective alternative approach to obtain long-term vascular access in children. Outcomes may be comparable to lines placed in traditional internal jugular and subclavian vein locations.

Pediatric short bowel syndrome and subsequent development of inflammatory bowel disease: an illustrative case and literature review.

Short bowel syndrome (SBS) in neonates is an uncommon but highly morbid condition. As SBS survival increases, physiologic complications become more apparent. Few reports in the literature elucidate outcomes for adults with a pediatric history of SBS. We present a case report of a patient, born with complicated gastroschisis resulting in SBS at birth, who subsequently developed symptoms and pathologic changes of inflammatory bowel disease (IBD) as an adult. The patient lived from age 7, after a Bianchi intestinal lengthening procedure, to age 34 independent of parenteral nutrition (PN), but requiring hydration fluid via G-tube. He was then diagnosed with IBD, after presenting with weight loss, diarrhea, and malabsorption, which required resumption of PN and infliximab treatment. This report adds to a small body of the literature which points to a connection between SBS in neonates and subsequent diagnosis of IBD. Recent evidence suggests that SBS and IBD have shared features of mucosal immune dysfunction and altered intestinal microbiota. We review current treatment options for pediatric SBS as well as multidisciplinary and coordinated transition strategies. We conclude that there may be an etiologic connection between SBS and IBD and that this knowledge may impact outcomes and approaches to care.

Malakoplakia and primary immunodeficiency.

Malakoplakia, a rare granulomatous disease caused by impaired macrophage response, has been reported only rarely in children. We report 3 unique cases, with lesions occurring in unusual locations in children with primary immune deficiencies.

Cystic biliary atresia: a wolf in sheep's clothing.

Neonatal obstructive jaundice is frequently explained by biliary atresia (BA) or the presence of a choledochal cyst (CC). Cystic biliary atresia (CBA) has been a proposed as a subtype of BA with projected improved outcomes. We aimed to characterize these lesions further. We conducted an Institutional Review Board-approved review of all patients treated for obstructive jaundice at our tertiary children's hospital over 10 years. Over the decade we evaluated 91 children with obstructive jaundice: 13 CBA, 52 BA, and 26 CC. Patients with isolated CBA and BA were diagnosed significantly earlier than those with CC (15.9, 54, and 281 days, respectively; P = 0.0001). There was a significant delay between diagnosis and surgical intervention for patients with CBA compared with BA: 17 days versus 5.7 days (P = 0.004). There was no difference in rate of transplant between CBA and BA (31 vs. 50%; P = 0.35). The time from surgery until transplant was 13.9 and 18.6 months for CBA and BA, respectively (P = 0.62). Although radiographically similar to CC, CBA behaves similarly to isolated BA. Delay in recognition and surgical treatment may affect outcomes and lead to an increased incidence of liver failure. The presence of a cystic biliary malformation in the setting of neonatal jaundice should be regarded as CBA until proven otherwise.

Noniatrogenic neonatal gastric perforation: the role of interstitial cells of Cajal.

Noniatrogenic neonatal gastric perforation is a rare and life-threatening condition whose etiology is often unclear. Interstitial cells of Cajal act as gastrointestinal pacemaker cells and express the proto-oncogene c-Kit. Six new cases were identified at our institution which presented with no mechanical, pharmacologic, or otherwise medical-related intervention prior to rupture. The number of interstitial cells of Cajal in nonnecrotic muscularis propria from five random high-power fields per specimen was compared using immunohistochemical stains for c-Kit. The authors show that a lack of interstitial cells of Cajal in the stomach musculature may be implicated in the development of noniatrogenic gastric perforation (p = 0.008). Further large-scale studies, including molecular and genetic analysis, may help to better understand this phenomenon.

Ciliated hepatic foregut cyst: four case reports with a review of the literature.

Ciliated hepatic foregut cysts (CHFCs) are rare congenital legions that arise from the embryonic foregut. The cysts are formed during fetal development by evagination from their respective portions of the foregut, and are characterized by a ciliated epithelial lining. Approximately 100 cases of CHFC have been reported, of which only 13 were in children. Although CHFC is typically benign, malignant transformation to squamous cell carcinoma (SCC) has been reported in 3 cases. Survival rate after progression to malignancy is poor, as SCC in this setting is biologically aggressive. We present 4 new cases of CHFC in children between 5 months and 17 years old. Our cases are unusual, as some of the cysts exhibit multilocularity and biliary communication, and 2 of our patients were diagnosed under the age of 1. Additionally, 1 of the cysts was 19.3 cm in diameter, making it the largest reported CHFC to our knowledge. Ciliated hepatic foregut cysts should be included in the differential diagnosis of hepatic lesions.

Button cholecystostomy for management of progressive familial intrahepatic cholestasis syndromes.

BACKGROUND/PURPOSE: Progressive familial intrahepatic cholestasis syndromes are characterized by impaired bile acid secretion resulting in pruritus, coagulopathy, diarrhea, and malnutrition leading to progressive liver failure and death in childhood. Partial internal or external biliary drainage can relieve symptoms and slow the progression of the disease. Objections to partial external biliary drainage include the need for a permanent biliary stoma with all the inherent complications of a stoma. We propose a novel approach to these diseases--placement of a "button" cholecystostomy tube. METHODS: Under general anesthesia and through a small right subcostal incision, a MIC-KEY button (Kimberly-Clark Worldwide, Inc, Draper, UT) is inserted into the mobilized fundus of the gallbladder and secured with 2 purse-string sutures. Time of drainage is adjusted to relieve pruritus. RESULTS: Three children with progressive familial intrahepatic cholestasis achieved adequate bile drainage via the cholecystostomy button to relieve pruritus for 1, 2, and 2 ½ years postoperatively, with drainage periods of 12 to 14 hours per day. There were no episodes of cholangitis. Dislodged tubes can be replaced, or stones can be retrieved via the tract that is formed. Patient (parent) acceptance has been excellent. CONCLUSION: Button cholecystostomy is simple to perform, relieves pruritus with intermittent (nighttime) drainage, avoids complications of a permanent stoma, avoids an enteric anastomosis, and is accepted by parents.

Use of tissue expanders in the repair of complex abdominal wall defects.

BACKGROUND/PURPOSE: Closure of abdominal wall defects in children poses a challenge for pediatric surgeons. We describe a technique using tissue expanders placed either intraperitoneally or in the abdominal wall to aid in the reconstruction of a variety of complex abdominal wall defects. METHODS: The tissue expanders are inserted under general anesthesia. Initial expansion is done in the operating room with attention to peak airway pressure, urine output, and end-tidal carbon dioxide. The expanders are inflated in the outpatient setting via percutaneous access until the calculated inflation volume is achieved. They are then removed; and definitive closure is accomplished using a combination of native tissue flaps, abdominal component separation techniques, biomaterials, and synthetic material. RESULTS: Six children underwent tissue expansion for treatment of abdominal wall defects (omphalocele, n = 3), trauma (n = 1), and thoracopagus twins (n = 1 pair). One to 4 expanders were used per patient, with all having a successful reconstruction of their abdominal walls. Two to 3 operations were required to restore abdominal domain and consisted of expander insertion, removal with reconstruction, and possible revision of the reconstruction. CONCLUSIONS: Tissue expanders possess a broad range of applications for abdominal wall reconstruction and can be used in patients of all ages.

Role of hepatic portocholecystostomy ('gallbladder Kasai') in treating infants with biliary atresia.

The aim of this study is to compare liver function and cholangitis episodes during the first year postoperatively between patients who undergo hepatic portocholecystostomy (HPC) and patients who undergo hepatic portoenterostomy (HPE). Records of six patients who underwent HPC for biliary atresia (BA) and 27 patients who underwent HPE for BA were reviewed retrospectively. Comparison was done of the patient's total bilirubin, albumin, and international normalized ratio values preoperatively and at 3 months, 6 months, and 1 year postoperatively. Comparison was also done of the occurrence of ascending cholangitis during the first year postoperatively and in rates of transplant and mortality during long-term follow-up. Preoperative laboratory values between the two groups were not significantly different. At 6 months, the patients who underwent HPC had significantly lower total bilirubin levels compared with those who underwent HPE (HPC 0.8 +/- 0.96, n = 4; HPE 4.93 +/- 7.73, n = 21; P < 0.05). No other laboratory values or rates of ascending cholangitis, transplant, or mortality showed a significant difference. Those patients who underwent HPC had significantly lower total bilirubin levels at 6 months postoperatively. This may suggest that HPC may be a superior operative technique for patients who are candidates for the operation.

Bedside placement of an aortic occlusion balloon to control a ruptured aorto-esophageal fistula in a small child.

A child had a percutaneous endoscopic gastrostomy tube removed by transecting it at skin level. The internal portion ("the bolster") was allowed to pass in the GI tract. She subsequently had odynophagia. Magnetic resonance imaging identified a foreign body within the lower thoracic esophagus. During esophagoscopy, the bolster was removed. The following morning, she had massive hematemesis with cardiovascular collapse. As an emergent maneuver, an intra-aortic balloon was percutaneously deployed at the bedside without fluoroscopic guidance. This temporized the exsanguination. Subsequent intraoperative aortography confirmed an aortoesophageal fistula. A stent-graft was deployed with immediate hemodynamic stabilization. She later underwent esophageal resection and recovered well.

NUT midline carcinoma in a newborn with multiorgan disseminated tumor and a 2-year-old with a pancreatic/hepatic primary.

NUT midline carcinoma (NMC) is a rare and aggressive malignant epithelial tumor defined by rearrangement of the NUT gene on chromosome 15. In two thirds of cases, NUT is involved in a balanced translocation with BDR4 on chromosome 19, while in the remaining cases, NUT is rearranged with variant fusion partners such as BRD3. These undifferentiated tumors primarily affect midline structures, usually in the upper aerodigestive tract and mediastinum. Most reported cases have followed a rapidly lethal clinical course. We report the clinical and pathological findings of NMC in the youngest patients identified so far. The 1st case involves a newborn who presented with a supraorbital mass and extensive multiorgan involvement, including the spine, lungs, liver, pancreas, adrenal glands, and subcutaneous tissue. The 2nd patient was a 2-year-old male with an abdominal mass involving the liver and pancreas with pulmonary metastasis. Histopathological analysis of both tumors showed undifferentiated malignant neoplasms, and immunohistochemistry showed positivity for epithelial markers. Both tumors demonstrated t(15;19), and immunohistochemistry with NUT monoclonal antibodies and fluorescent in situ hybridization confirmed NUT rearrangement. The patients died from disease at 1 and 2 months postpresentation. Thus far, 25 cases have been reported, including our 2 current cases. Presentation ages range from 0 to 78 years (mean, 23 years). Herein, we report the 2 youngest reported cases of NMC, including the 1st congenital case and the 1st case arising within the liver/pancreas. Increased awareness and further molecular studies are required for a better understanding of NMC pathobiology and improved therapeutic outcomes.

Pseudoangiomatous stromal hyperplasia of the breast in pediatric patients: an underrecognized entity.

Pseudoangiomatous stromal hyperplasia (PASH) is a benign lesion consisting of mammary stromal proliferation with anastomosing slits mimicking vascular spaces. Grossly, it most often resembles fibroadenoma, but may commonly be confused with angiosarcoma and other types of benign vascular proliferations. While PASH has been described in female and male adults since the mid-1980s, there have been only a few accounts in the pediatric population. We present a series of 12 pediatric patients with PASH, including a 3-year-old male, who we believe to be the youngest patient to present with this entity. In our study, PASH was found in 12% of tumors diagnosed preoperatively as fibroadenomas and in 12% of cases diagnosed preoperatively as gynecomastia. Our series documents that PASH is not uncommon in pediatric breast pathology and delineates important differences between adult and pediatric presentations of this entity.

Unusual presentation of ovarian cellular fibroma coexisting with nodular sclerosing Hodgkin's disease: a case report and review of the literature.

A case of a nonfunctioning, noncalcified ovarian cellular fibroma in a 15-year-old adolescent girl is described. The fibroma was found incidentally in a patient who was later found to have nodular sclerosing Hodgkin's disease. The patient presented with symptoms suggestive of lymphoma but on exam was found to have a mobile and nontender abdominal mass. Laparotomy exposed a smooth, well-circumscribed left ovarian mass. A left salpingoophorectomy was performed. The mass weighed 2390 g and measured 19 x 15 x 8.5 cm. Approximately 15% of the tumor was composed of large cellular nodules identified as spindle cells having a storiform pattern. Mild nuclear atypia with 2 mitosis per 10 high-power fields was noted. An ovarian fibroma in a 15-year-old is an unexpected diagnosis. Less than 1% of ovarian stromal tumors are found in patients 19 years and younger. The uncommon histology and additional classification as a cellular fibroma as well as the simultaneous diagnosis of 2 neoplasms in this case are remarkable.

Primary ovarian rhabdomyosarcoma in children.

Although rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, primary rhabdomyosarcomas of the ovary are extremely rare, with only eight well-documented pediatric cases previously reported in the literature. We present two additional cases: an alveolar RMS originating in the right ovary with metastatic spread to the splenic flexure of the colon and to both lungs in a 13-year-old African American girl, and an embryonal RMS arising in the right ovary of a 6-year-old Caucasian girl with pre-operative intra-abdominal rupture and a malignant right pleural effusion. Both patients had complete resection of their primary tumors and received chemotherapy including vincristine, doxorubicin and cyclophosphamide with good response to therapy. Both are alive 8 and 9 months post-operatively.

Congenital spigelian hernias and cryptorchidism.

PURPOSE: Spigelian hernias in childhood are rare. Only 24 infants in the English literature have been identified to have spigelian hernias, and 12 of these have been associated with cryptorchidism. Spigelian hernias are more commonly seen in the adult population and are considered to be acquired because they are typically associated with trauma or other etiologies of increased intraabdominal pressure. In the infant however, the etiology remains unclear, but a congenital defect in abdominal wall development is suspected. METHODS: We discuss the presentation and treatment of 4 additional patients with spigelian hernias (2 siblings included) associated with cryptorchidism. RESULTS: The hernias occurred within the well-described spigelian hernia belt in the semilunar line at the level of the semicircular fold of Douglas. Of the 6 repaired spigelian hernias, 5 were closed primarily with absorbable suture similar to previously reported cases; the sixth hernia required a patch closure because of its large size. All cryptorchid testes (7) were repaired in single-stage orchiopexies. CONCLUSIONS: Spigelian hernias are rare entities in infants. We present 4 new cases of spigelian hernias associated with cryptorchidism and, with previously reported cases, discuss the probability of a congenital origin of these hernias in infants.

Antireflux surgery outcomes in pediatric gastroesophageal reflux disease.

OBJECTIVES: Antireflux surgery is performed frequently in children with gastroesophageal reflux disease (GERD). Few comparative studies exist which assess the indications for and short- or long-term outcome of open Nissen fundoplication (ONF) and laparoscopic Nissen fundoplication (LNF) for pediatric GERD. We investigated the frequency of reoperation and factors that might influence its occurrence. METHODS: We performed a retrospective, follow up cohort study of all children

Postoperative outcomes of extremely low birth-weight infants with necrotizing enterocolitis or isolated intestinal perforation: a prospective cohort study by the NICHD Neonatal Research Network.

OBJECTIVE: Purposes of this study were: 1) to compare mortality and postoperative morbidities (intra-abdominal abscess, wound dehiscence, and intestinal stricture) in extremely low birth weight (ELBW) infants who underwent initial laparotomy or drainage for necrotizing enterocolitis (NEC) or isolated intestinal perforation (IP); 2) to determine the ability to distinguish NEC from IP preoperatively and the importance of this distinction on outcome measures; and 3) to evaluate the association between extent of intestinal disease determined at operation and outcome measures. BACKGROUND: ELBW infants who undergo operation for NEC or IP have a postoperative, in-hospital mortality rate of approximately 50%. Whether to perform laparotomy or drainage initially is controversial. Also unknown is the importance of distinguishing NEC from IP and the current ability to make this distinction based on objective data available prior to operation. METHODS: A prospective, multicenter cohort study of 156 ELBW infants at 16 neonatal intensive care units (NICU) within the NICHD Neonatal Research Network. RESULTS: Among the 156 enrolled infants, 80 underwent initial peritoneal drainage and 76 initial laparotomy. Mortality rate was 49% (76 of 156). Ninety-six patients had a preoperative diagnosis of NEC and 60 had presumed IP. There was a high level of agreement between the presumed preoperative diagnosis and intraoperative diagnosis in patients undergoing initial laparotomy (kappa = 0.85). The relative risk for death with a preoperative diagnosis of NEC (versus IP) was 1.4 (95% confidence interval, 0.99-2.1, P = 0.052). The overall incidence of postoperative intestinal stricture was 10.3%, wound dehiscence 4.4%, and intra-abdominal abscess 5.8%, and did not significantly differ between groups undergoing initial laparotomy versus initial drainage. CONCLUSIONS: Survival to hospital discharge after operation for NEC or IP in ELBW neonates remains poor (51%). Patients with a preoperative diagnosis of NEC have a relative risk for death of 1.4 compared with those with a preoperative diagnosis of IP. A distinction can be made preoperatively between NEC and IP based on abdominal radiographic findings and the patient's age at operation. Future randomized trials that compare laparotomy versus drainage would likely benefit from stratification of treatment assignment based on preoperative diagnosis.

Gastrointestinal stromal tumors arising from the stomach: a report of three children.

BACKGROUND: Gastrointestinal stromal tumors (GIST) are a unique subset of intestinal mesenchymal tumors that behave in an aggressive fashion. They have been commonly described in adults but have been rarely observed in children. METHODS: The authors review the presentation, diagnostic workup, operative records, pathologic specimens, and outcomes of 3 children with GISTs that originated from the stomach. RESULTS: All 3 children presented after upper gastrointestinal bleeding from the gastric tumor. The first was a 10-year-old girl who underwent partial gastrectomy but had recurrence 8 years later requiring a second resection. She subsequently had a hepatic metastasis 8 years later requiring a third resection. The second patient was a 9-year-old girl who had an antrectomy with a Bilroth I reconstruction and was noted to have a synchronous liver metastasis that was also resected. Despite Imatinib Mesylate, she had further hepatic metastases. The third child was a 4-year-old boy who recently underwent a partial gastrectomy and has no signs of metastatic disease at this time. CONCLUSIONS: GISTs are unusual tumors that have been rarely described in children. When they arise in the stomach, they often present after upper gastrointestinal bleeding. Diagnosis can be made by endoscopy and biopsy. GISTs require resection and close observation for hepatic metastases. Current studies are ongoing for the potential role of Imatinib Mesylate for GISTs in children.

Lymphangioma of the falciform ligament--a case report.

A rare case of a lymphangioma of the falciform ligament in a child is described. He presented with abdominal pain and was found to have an unusual intraabdominal mass. Resection was curative.