Outcomes of a pediatric surgical oncology fellowship in a pediatric cancer institution.

Surgery plays an important role as part of the treatment plan in most children with malignant solid tumors in regards to initial biopsy, upfront resection, and delayed resection. Surgeons also play a critical role in the treatment of surgical complications that may arise during medical treatment. The pediatric surgical oncologist should be familiar with the current treatment guidelines, histology implications, chemotherapy and radiation side effects, tumor staging, and overall care of the child with cancer. Specific training in pediatric surgical oncology is not widespread internationally and it represents a potential undervalued intervention for improving global pediatric cancer care.

Guidewire Catheter Exchange in Pediatric Oncology: Indications, Postoperative Complications, and Outcomes.

BACKGROUND: Maintaining long-term central venous catheters (CVCs) in children undergoing chemotherapy can be challenging. Guidewire catheter exchange (GCE) replaces a CVC without repeat venipuncture. This study evaluated the indications, success rate, and complications of GCE in a large cohort of pediatric cancer patients. PROCEDURE: Medical records of pediatric cancer patients who underwent GCE at our institution between 2003 and 2013 were retrospectively reviewed. Variables analyzed included gender, age at GCE, primary cancer diagnosis, indication for GCE, absolute neutrophil count (ANC) at GCE, vein used, success rate, and postoperative complications (<30 days after exchange). RESULTS: A total of 435 GCEs performed in 407 patients (230 males and 177 females) were reviewed. Median age at GCE was 8 years (range, 0.2-24). Acute lymphoblastic leukemia was the most common diagnosis (50.6%). The primary indication for GCE was the desire to have an alternative type of CVC (71%). Other indications included catheter displacement (17%), catheter malfunction (11%), and catheter infection (1%). Median ANC at GCE was 2,581/mm(3) (range, 0-43,400). Left subclavian vein was more commonly used (57.7%). The success rate of GCE was 93.4% (406 of 435 procedures, 95% confidence interval: 91.0-97.5%). A total of 33 (7.5%) postoperative complications occurred including central line associated bloodstream infection (CLABSI) (n = 20, 4.5%), catheter dislodgement (n = 6, 1.4%), and catheter malfunction (n = 7, 1.6%). CONCLUSIONS: We conclude that GCE in pediatric cancer patients is associated with a high success rate and a low risk of complications. The most common postoperative complication, CLABSI, occurred at a rate significantly lower than following de novo CVC placement.

Safety of central venous catheter placement at diagnosis of acute lymphoblastic leukemia in children.

BACKGROUND: Central venous catheters (CVC) facilitate the management of patients with cancer. Optimal timing for placement of a CVC is controversial. We sought to determine whether early placement in children with acute lymphoblastic leukemia (ALL), a group at high risk for infection and thrombosis, was associated with an increased rate of surgical complications. PROCEDURE: We evaluated the incidence and risk factors for early surgical complications in children with ALL diagnosed between 2004 and 2009 at a single pediatric cancer center. RESULTS: One hundred seventy-two patients were studied. There were 17 episodes of bloodstream infection, for a 30-day incidence of 9.8% (95% CI, 5.9-15%). There were no surgical site infections and no CVC was removed due to infection. Early thrombosis occurred in only one patient, 3 days after CVC placement. Infection was not influenced by catheter type, patient age, body mass index, or fever at the time of placement. The infection rate was not statistically higher when the ANC was <500/mm(3) at the time of CVC placement (14.2% vs. 6.8%; P = 0.12). CONCLUSION: Early CVC placement at the time of diagnosis of ALL was associated with a low surgical complication rate with no catheters requiring removal due to infection. Utilizing our current methods of preoperative preparation, surgical management and postoperative CVC care, early placement of a CVC is safe in children with ALL even when their ANC is <500/mm(3) , but larger cohort studies would be helpful to further clarify this issue.

Is reexcision in pediatric nonrhabdomyosarcoma soft tissue sarcoma necessary after an initial unplanned resection?

PURPOSE: The aim of this study was to determine the importance of pretreatment reexcision (PRE) of pediatric nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) after initial unplanned resection. METHODS: The authors retrospectively reviewed the records of 116 consecutive patients with surgically resected NRSTS treated at their institution between February 1978 and September 1999. Ninety-four (81.0%) patients had undergone unplanned resections before referral to their institution for further therapy. Demographic data, tumor characteristics, treatment administered, and outcomes were recorded. RESULTS: Sixty-nine patients (73.4%) underwent PRE at a median interval after the initial unplanned resection of 29 days. Twenty-five patients were thought unsuitable for PRE because of the proximity to vital neurovascular bundles. Tumors deemed not feasible for PRE were more likely to be greater than 5 cm (P =.0094) and high grade (P =.0200). Tumor was found in 33 (47.8%) of the PRE specimens, and 24 of these patients (72.7%) were deemed disease free after achieving negative surgical margins. Residual tumor was more likely to be found after PRE in head and neck primary tumors than in trunk wall or extremity primary tumors (P =.0128). There were no local failures in the 60 PRE patients who achieved clear margins. The estimated 5-year event-free and 5-year overall survival rates in these 60 patients were 98.3% +/- 2.0% and 98.2% +/- 2.1%, respectively. CONCLUSIONS: Pretreatment reexcision should be performed whenever feasible in pediatric patients with NRSTS who had an initial unplanned resection. The proportion of patients with residual tumor in the PRE specimen is high, and negative microscopic margins can be achieved after PRE in most patients with residual tumor. Despite delay in obtaining a complete surgical resection, no local recurrences were seen. The possibility of NRSTS should be considered when resecting a soft tissue mass in children, and diagnostic incisional biopsy followed by wide local excision with negative microscopic margins should be the surgical goal.

Childhood cancer: patterns of protocol participation in a national survey.

Cancer is still the chief cause of death by disease in children, ages one to 14. As improved survival rates have been reported for pediatric cancer patients who are treated on controlled clinical trials, it is important to understand the national utilization of such protocols. In 1993, a survey of childhood cancer was conducted by the Commission on Cancer of the American College of Surgeons. Data regarding type of disease, protocol participation, age, sex, race, insurance, and geographical region were voluntarily submitted by more than 200 hospital cancer registries. Included in this study were 2,208 children and adolescents 21 years of age or younger who were diagnosed in 1987, and 2,293 who were diagnosed in 1992. Pediatric centers (i.e., members of the Pediatric Oncology Group or Children's Cancer Group) submitted 55.1% of the cases and other institutions, 44.9%. It was found that more patients treated at pediatric centers were on protocols (53.8%) than were those treated at other institutions (25.1%). In general, the younger the patient (five years of age or younger), the greater the chance of being on protocol (pediatric centers, 63.7%; others, 42.0%), with very poor adolescent protocol participation (pediatric centers, 34.8%; others, 12.1%). Nevertheless, overall protocol participation was still lower than expected, even in children younger than five years of age, and adolescent participation in controlled clinical trials was low and similar to adult figures. The percentage of childhood cancer cases seen at pediatric centers was smaller than in other series. It was concluded that pediatric cancer centers need to continue to encourage patient participation in controlled clinical trials, with special emphasis on adolescents.

Presurgical window of carboplatin and surgery and multidrug chemotherapy for the treatment of newly diagnosed metastatic or unresectable osteosarcoma: Pediatric Oncology Group Trial.

PURPOSE: Relapse remains a significant problem in patients with metastatic osteosarcoma. The response to carboplatin of patients with newly diagnosed metastatic or unresectable osteosarcoma was assessed in an upfront phase II window, which was followed-up by surgery and intensive multiagent chemotherapy. PATIENTS AND METHODS: Thirty-seven patients, ages 3 to 23 years with histologically confirmed diagnoses of osteosarcoma, were treated between January 1992 and November 1994 with carboplatin 1,000 mg/m2 per dose administered as a 48-hour continuous infusion. Two courses were administered in 3-week intervals, depending on marrow recovery. After radiographic reevaluation, patients underwent surgical removal of tumor (if feasible) and then 40 weeks of chemotherapy with high-dose methotrexate, ifosfamide, doxorubicin, and cisplatin. RESULTS: One of the 37 evaluable patients demonstrated a partial response to carboplatin; there were no complete responses. Patients were additionally analyzed by the response of pulmonary metastases to therapy and the extent of tumor necrosis of the primary lesion. By these criteria, 8 of 37 (22%) of patients showed a response at one or more sites, whereas 20 of 37 (54%) had unequivocal disease progression. Severe myelosuppression was the major toxicity. The projected 3-year event-free and overall survival rates were 23.9% and 31.9%, respectively. Only 1 of 17 patients with unresectable disease or distant bone metastases remains alive, in contrast to 6 of 17 patients with the lung as their only metastatic site and two of three patients with resected regional bone metastases. CONCLUSIONS: Continuous-infusion carboplatin demonstrated limited activity as an upfront agent in patients with metastatic osteosarcoma at diagnosis, even at doses that result in severe and prolonged myelosuppression. Patients with isolated pulmonary metastases or resectable bone metastases have a longer median survival time and greater chance of long-term survival than do patients with unresectable bone disease, for whom the prognosis remains dismal.

Nonrhabdomyosarcoma soft tissue sarcomas in children: is age at diagnosis an important variable?

PURPOSE: The associations between age at diagnosis, tumor characteristics, and outcome in children diagnosed with nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) were studied. METHODS: Retrospective review was conducted of 192 children from 1962 through 1996. Patients were divided into groups: birth to 1 year (n = 13), 1 to 5 years (n = 26), 5 to 10 years (n = 49), 10 to 15 years (n = 55), and older than 15 years (n = 49) of age at diagnosis. Characteristics including IRS group, histological grade and pattern, tumor size, and invasiveness were investigated. Survival rate was estimated by age group. The median follow-up was 8.8 years (range, 2 to 28 years). RESULTS: There were 81 group I patients, 40 group II, 41 group III, and 30 group IV. A significant difference of IRS groups among the age groups was seen (P = .034). There were no IRS group IV patients less than 1 year of age; 50% of IRS group IV patients were older than 15 years. A significant difference in the distribution of histological grade among the age groups (P = .032) was seen. Ten of 13 (77%) children less than 1 year of age had low-grade tumors, whereas 42%, 45%, 60%, and 37% of patients aged 1 to 5, 5 to 10, 10 to 15, and older than 15 years, respectively, had low-grade tumors. Patients older than 15 years had the highest incidence of invasive tumors (59%). Histological pattern also varied with age. The most prevalent histology in the less-than-1-year age group was infantile fibrosarcoma. No predominant histology was seen in the 1- to 5-year age group. Malignant fibrous histiocytoma was the most frequent histological subtype in children between 5 and 10 years of age. In the 10- to 15-year age group and children older than 15 years the malignant peripheral nerve sheath tumor and synovial sarcoma were the most prevalent subtypes. Without adjusting for any other factors, age group was prognostic of survival (P = .007). Patients less than 1 year at diagnosis had the best outcome, with a 5-year survival rate of 92% +/- 9%. Five-year estimates were lowest for patients older than 15 years (49% +/- 7%). CONCLUSIONS: Significant differences in IRS group, histological grade, and histological subtype were observed in different age groups. Infants with NRSTS were more likely to have low grade, less invasive, and lower stage tumors. These characteristics may account for their improved prognosis.

3D gadolinium-enhanced MRA: evaluation of hepatic vasculature in children with hepatoblastoma.

We used contrast-enhanced three-dimensional magnetic resonance angiography (3D MRA) modified for pediatric use to evaluate the hepatic vasculature prior to partial hepatectomy in five consecutive children with hepatoblastoma. Modifications included non-breath-hold technique in four of the five children who were sedated. The single breath-hold technique was performed in only one awake child. Scan delay times were based on contrast infusion time rather than total infusion time. The hepatic artery, portal vein, and inferior vena cava were identified in all patients. MRA findings were confirmed by conventional angiography in one patient and by surgery in all. Contrast-enhanced 3D MRA is a useful and rapid technique prior to partial hepatectomy in patients with hepatoblastoma.

Neuroblastoma regression and immunity induced by transgenic expression of interleukin-12.

PURPOSE: Interleukin-12 (IL-12) is a cytokine with potent antitumor effects. The authors sought to assess its capacity to increase tumor immunogenicity when expressed by tumor cells in a murine model of neuroblastoma. METHODS: Syngeneic A/J mice were inoculated subcutaneously with 2 x 10(6) cells from a murine neuroblastoma-derived cell line (neuro-2a). In situ transduction of the neuroblastoma cells was achieved by intratumoral injection of an adenoviral vector encoding both subunits of the murine IL-12 heterodimer. Growth of the IL-12 gene-modified tumor cells was compared with untreated neuro-2a cells. Tumor immunity was assessed by rechallenging mice that had rejected their tumor with unmodified neuroblastoma cells. The contribution of cytotoxic T lymphocytes (CTLs) was evaluated through cytotoxicity assays. RESULTS: Eighteen (72%) of 25 tumor-bearing mice treated with the mlL-12 adenoviral vector exhibited tumor regression, with 12 mice (48%) completely rejecting their tumors over 2 to 3 weeks. None of the mice that had rejected their tumor and were rechallenged with unmodified neuro-2a cells subsequently developed new tumors. Pooled splenocytes from mice rejecting their tumors showed significant tumor killing (>20% cytolysis) in vitro in 51Cr release assays. CONCLUSIONS: Adenoviral-mediated IL-12 expression by tumor cells in a murine neuroblastoma model produced a significant antitumor response. Most treated tumors demonstrated at least transient regression, whereas many completely regressed. Cured mice exhibited protective immunity and CTL activity against the tumor. These data confirm the immunomodulatory efficacy of IL-12 as part of a vaccine-based antineuroblastoma strategy.

Enhanced neuroblastoma transduction for an improved antitumor vaccine.

BACKGROUND: A recent clinical trial of an antineuroblastoma vaccine used adenovirus serotype 5 (Ad5) vectors to transduce autologous tumor cells with the gene encoding IL-2. A method to improve transduction efficiency was sought to enable the use of lower viral titers, especially when in situ adenoviral-mediated tumor cell transduction is considered. MATERIALS AND METHODS: A chimeric adenoviral delivery vector was utilized in which the fiber head from adenovirus serotype 3 was incorporated into the backbone of Ad5. Since the fiber head protein is responsible for viral attachment to target cells, a different spectrum and range of infectivity might result. Both the chimeric (Av9LacZ4) and Ad5 (Av1LacZ4) vectors were constructed to carry a beta-galactosidase transgene. The relative transduction efficiency of these two vectors was then evaluated in five tumor-derived short-term neuroblastoma cultures and four established neuroblastoma cell lines. Enzyme activity was assessed using three different methods: in situ staining, flow cytometric analysis, and a quantitative assay. RESULTS: A significant improvement in transduction efficiency of the short-term neuroblastoma cultures with the new chimeric adenovector was demonstrated. A similar improvement in transduction efficiency was not observed in the established cell lines, suggesting that the cell surface receptor for the Ad 3 serotype had been lost in vitro. Increased transduction of tumor cells with N-myc amplification was also observed. CONCLUSIONS: The newly constructed chimeric adenoviral vector transduces short-term neuroblastoma cultures more efficiently than the standard Ad5 vector. This vector will permit the use of lower viral titers and may be useful in other adenoviral-based gene-therapy protocols. Increased transgene expression in N-myc-amplified cells offers possible selectivity for in situ gene delivery.

Treatment of metastatic osteosarcoma at diagnosis: a Pediatric Oncology Group Study.

PURPOSE: To estimate the duration of survival (S) of patients with metastatic osteosarcoma (MOS) at diagnosis treated with a multiagent, ifosfamide-containing chemotherapeutic and surgical regimen and to evaluate the toxicity of this regimen. PATIENTS AND METHODS: Thirty patients aged younger than 30 years received two courses of ifosfamide followed by surgery on the primary tumor and metastatic sites. Patients then received a postsurgical multiagent chemotherapeutic regimen that consisted of high-dose methotrexate (HDMTX), ifosfamide, doxorubicin, and cisplatin. RESULTS: The 5-year event-free survival (EFS) rate was 46.7% (95% confidence interval [CI]; 28.5 to 64.9) and 5-year S rate was 53.3% (95% CI; 35.1 to 71.5). Three patients with bone metastases and one patient with lymph node metastases died. Twenty-six patients presented with pulmonary metastatic nodules only. Eight of these patients had at least eight nodules at diagnosis and had an estimated 5-year EFS rate of 25.0% compared with 66.7% for the 18 patients with less than eight nodules (P=.06). Fourteen patients presented with bilateral lung metastases and had a 5-year EFS rate of 35.7% compared with the 12 patients who presented with unilateral involvement and had a 5-year EFS rate of 75.0% (P=.03). The hematopoietic toxicity experienced by the patients during the entire regimen was relatively mild. Seven patients had renal toxicity characterized by hypophosphatemia and/or hypokalemia. CONCLUSION: This ifosfamide-containing regimen is tolerable and effective in the treatment of patients with osteosarcoma (OS) who present with lung metastases. However, better regimens are required for this group of patients.

Wilms tumor presenting with abdominal pain: a special subgroup of patients.

BACKGROUND: Although significant progress has been made in the management of children with Wilms tumor, two major controversies still exist: the extent of radiographic evaluation necessary before surgery and the role of preoperative chemotherapy. This study sought to determine whether patients with Wilms tumor who presented with abdominal pain defined a special subset of patients who might require a more extensive preoperative work-up and neoadjuvant chemotherapy. METHODS: From 1970 to 1995, 250 children were treated for Wilms tumor at a single pediatric institution. A retrospective chart review determined presenting signs and symptoms for each patient. RESULTS: Thirty-four (14%) patients (mean age 5.5 years) sought medical attention with a chief complaint of abdominal pain. The stage distribution for these patients tended to be higher and was significantly different (P <.001, chi(2)-analysis) from those presenting without pain. Four (14%) of 29 patients with tumor available for analysis were found to have anaplastic histology. CONCLUSION: These data suggest that patients with Wilms tumor who present with abdominal pain represent a special subgroup that tends to be older and has an increased incidence of tumor rupture, anaplasia, and higher stage. These patients may benefit from a more extensive preoperative evaluation and consideration of neoadjuvant chemotherapy.

Comparison between single-dose and divided-dose administration of dactinomycin and doxorubicin for patients with Wilms' tumor: a report from the National Wilms' Tumor Study Group.

PURPOSE: The National Wilms' Tumor Study (NWTS)-4 was designed to evaluate the efficacy, toxicity, and cost of administration of different regimens for the treatment of Wilms' tumor (WT). PATIENTS AND METHODS: Between August 6, 1986 and September 1, 1994, 1,687 previously untreated children less than 16 years of age with stages I to II/favorable histology (FH) or stage I/anaplastic histology WT (low-risk [LR] group) or stages III to IV/FH WT or stages I to IV/clear cell sarcoma of the kidney (high-risk [HR] group) were randomized to treatment that included vincristine and either divided-dose (standard [STD]) courses (5 days) or single-dose (pulse-intensive [PI]) treatment with dactinomycin. HR patients also received either STD courses (3 days) or PI treatment with doxorubicin. RESULTS: The 2-year relapse-free survival (RFS) rates for LR patients were 91.3% for 544 randomized to treatment with PI and 91.4% for 556 randomized to treatment with STD chemotherapy (P = .988). The 2-year RFS rates for HR patients were 87.3% for 299 randomized to treatment with PI and 90.0% for 288 randomized to treatment with STD chemotherapy (P = .865). CONCLUSION: We conclude that patients treated with PI combination chemotherapy for LR or HR WT or clear cell sarcoma of the kidney have equivalent 2-year RFS to those treated with STD regimens. PI drug administration is recommended as the new standard based on demonstrated efficacy, greater administered dose-intensity, less severe hematologic toxicity, and the requirement for fewer physician and hospital encounters.

Hemopericardium from coronary artery laceration complicating extracorporeal membrane oxygenation.

OBJECTIVE: We report the clinical course and successful surgical treatment of hemopericardium resulting from coronary artery (CA) laceration in two patients with congenital diaphragmatic hernia (CDH) undergoing extracorporeal membrane oxygenation (ECMO) bypass. STUDY DESIGN: Retrospective case review. RESULTS: Two neonates with CDH had needle aspiration for either pneumothorax or pericardial effusion before initiation of ECMO. While on bypass, progressive hemopericardium led to narrow pulse pressure and decreased venous return that limited bypass flow. Widened cardiac silhouette on chest radiographs suggested hemopericardium; echocardiography was confirmatory in one case. The underlying diagnosis of CA laceration was made during pericardiotomy and treated with surgical patching. CONCLUSIONS: Pre-ECMO history of cardiothoracic needle aspiration is important because complications such as hemothorax or hemopericardium may arise once ECMO bypass is initiated. Inadvertent CA laceration may lead to acute hemopericardium, compromising venous drainage. However, CA laceration can be successfully repaired while the patient is on bypass.

Severe head injury caused by airbag deployment.

Passenger-side airbags are present in over 21 million automobiles and will be required on all passenger vehicles by the model year 1999. Although airbags are effective supplemental restraint systems and have saved over 1,500 lives, airbag-related injuries have been reported. The National Highway Traffic Safety Administration recently reported 15 child deaths caused by airbag deployment. All of these children were believed to be improperly restrained in the front passenger position of a car equipped with a passenger-side airbag. This case represents the first serious injury caused by airbag deployment in a child that was properly positioned in an approved child restraint.

Endodermal sinus tumor in children.

Malignant germ cell tumors account for about 3% of neoplasms in children, and endodermal sinus tumor (EST) is the most common histological subtype. The authors reviewed 22 years' experience (at their institution) in the management of 37 patients with this tumor. Fifteen of them (41%) had a sacrococcygeal primary, 10 had a testicular tumor (27%), 6 had an ovarian tumor (16%), 3 had a vaginal tumor (8%) and 3 had tumors at other sites (8%). Seven (19%) patients presented with metastatic disease, primarily pulmonary. The serum alpha-fetoprotein (AFP) level was elevated in all cases tested. The initial chemotherapy regimen included vincristine, actinomycin, cyclophosphamide (VAC), and Adriamycin (6 patients), but since 1985 the regimen has been changed to include cisplatin or Carboplatin, etoposide, and bleomycin (21 patients). Eight patients with testicular tumors initially were treated with surgical excision alone. Computed tomography results were a poor predictor of recurrence, but AFP surveillance was extremely sensitive. No second-look operation detected residual tumor in the absence of AFP elevation. Initial relapse for all patients tended to occur early (within 2 years), locally, and often with pulmonary metastases. Although historically the prognosis for patients with EST has been poor, the overall 2-year survival rate in this series was 70%. The best prognosis was among the children who had a testicular primary tumor (survival rate, 100%). The 2-year survival rate for patients with ovarian tumors was 67%; for those with sacrococcygeal primaries it was 60%. These results suggest that the prognosis for children with EST has improved significantly over the past decade. Contributing factors include therapy based on cisplatin, etoposide, and bleomycin, and relapse surveillance with serial AFP determinations. Second-look procedures should be reserved for patients who have an increasing level of serum AFP, suspicious computed tomography findings, and no obvious evidence of metastatic disease.

Treatment of osteosarcoma with ifosfamide: comparison of response in pediatric patients with recurrent disease versus patients previously untreated: a Pediatric Oncology Group study.

This study was designed to test if the activity of a phase II agent, ifosfamide, would have been underestimated if it was tested exclusively in a population of children and young adults with recurrent osteosarcoma. The response rate to ifosfamide was compared in patients younger than 30 years of age with previously untreated osteosarcoma with metastases at diagnosis and/or unresectable primary tumors (stratum 1) with that of patients with recurrent osteosarcoma following adjuvant chemotherapy who were not previously exposed to ifosfamide (stratum 2). Evaluation of response was conducted 3 weeks after two courses of ifosfamide (2400 mg/m2 x 5 days) were administered 3 weeks apart. Nine of 33 (27%) evaluable patients in stratum 1 responded (1 complete and 8 partial responses) to ifosfamide. Among 30 evaluable patients in stratum 2, only 3 (10%) responded (1 complete and 2 partial responses; P = .04) Both groups of patients received equal doses of ifosfamide and experienced comparable toxicities. Results from this study suggest that the activity of new agents will be underestimated if tested in a population of heavily pretreated patients with recurrent disease. When possible, new chemotherapeutic agents should be tested in patients with a poor prognosis who have not been exposed to chemotherapy.

Lobar lung transplantation as a treatment for congenital diaphragmatic hernia.

The mortality rate for infants severely affected with congenital diaphragmatic hernia (CDH) remains high despite significant advances in surgical and neonatal intensive care including delayed repair and extracorporeal membrane oxygenation (ECMO). Because of the increasingly successful experience with single-lung transplantation in adults; this approach has been suggested as a potential treatment for CDH infants with unsalvageable pulmonary hypoplasia. The authors report on a newborn female infant who was the product of a pregnancy complicated by polyhydramnios. At birth, she was found to have a right-sided CDH and initially was treated with preoperative ECMO, followed by delayed surgical repair. Despite the CDH repair and apparent resolution of pulmonary hypertension, the infant's condition deteriorated gradually after decannulation, and escalating ventilator settings were required as well as neuromuscular paralysis and pressor support because of progressive hypoxemia and hypercarbia. A lung transplant was performed 8 days after decannulation, using the right lung obtained from a 6-week-old donor. The right middle lobe was excised because of the size discrepancy between the donor and recipient. After transplantation, the patient was found to have duodenal stenosis and gastroesophageal reflux, which required duodenoduodenostomy and fundoplication. The patient was discharged from the hospital 90 days posttransplantation, at 3 1/2 months of age. Currently she is 24 months old and doing well except for poor growth. This case shows the feasibility of single-lung transplantation for infants with CDH, and the potential use of ECMO as a temporary bridge to transplantation. Lobar lung transplantation allowed for less stringent size constraints for the donor lung.

Early hernia repair in the premature infant: long-term follow-up.

The incidence of inguinal hernia and incarceration is high among premature infants. Optimal timing, anesthetic technique, and long-term results of hernia repair in hospitalized premature infants remain undefined. The authors reviewed the records of 52 consecutively treated premature infants who underwent bilateral inguinal herniorrhaphy under general anesthesia before discharge from the intensive care nursery. There were no significant differences in gestational age, birth weight, age and weight at time of surgery, or presence of preoperative apnea or bradycardia in between infants extubated within 24 hours and those intubated for more than 24 hours. Twenty-four infants (46%) were available for follow-up of 24 months or more (mean follow-up period, 57 months). One recurrence was identified, representing 4% of the long-term follow-up group and 2% of the initial population. Two patients had asymmetric testicular volumes suggestive of unilateral atrophy. The short- and long-term results suggest that repair under general anesthesia can be safely performed before discharge from the intensive care nursery.