Multi-disciplinary care in patients with complex pediatric general and thoracic surgical pathology: lessons learned from a 20-year experience.

PURPOSE: Complex pediatric surgery patients with thoracic tumors invading the mediastinum and infradiaphragmatic tumors extending into the chest are at risk for surgical morbidity and mortality if the patient's care is not coordinated. We sought to identify areas of focus when managing these patients to improve care. METHODS: A 20-year, retrospective study of pediatric patients with complex surgical pathology was performed. Demographic data, pre-operative characteristics, intraoperative data, complications, and outcomes data were collected. Three index cases were highlighted to provide granularity in patient management. RESULTS: Twenty-six patients were identified. Common pathology included mediastinal teratomas, foregut duplications, advanced Wilms tumors, hepatoblastoma, and lung masses. All cases were performed in a multidisciplinary fashion. All cases were done with pediatric cardiothoracic surgery and three cases (11.5%) required pediatric otolaryngology. Eight patients (30.7%) required cardiopulmonary bypass. Operative and 30-day mortality was zero. CONCLUSIONS: Management of complex pediatric surgical patients requires a multidisciplinary approach throughout the patient's hospital course. This multidisciplinary team should meet in advance of a patient's procedure to create a customized care plan that may include pre-operative optimization. At the time of their procedure, all necessary and emergency equipment should available. This approach improves patient safety and has resulted in excellent outcomes. LEVEL OF EVIDENCE: IV.

Complete Absence of the Extrahepatic Biliary Tree in a Newborn With Pigmented Stools.

"Yellow stools in neonatal cholestasis exclude biliary atresia." This conventional wisdom led to the development of the infant stool color card, which alerts parents to seek medical referral when pale stools are observed, a strategy that has been shown to improve survival in infants with biliary atresia (BA). Here, we present a case of a newborn with significant direct hyperbilirubinemia (direct bilirubin level of up to 9.2 mg/dL on day of life 10) who continued to produce colored stools. Whole-genome sequencing results were negative for genetic causes of cholestasis. Hepatobiliary scintigraphy findings were nonexcretory. A liver biopsy specimen revealed cholestasis, ductular hyperplasia, giant cell formation, minimal inflammation, minimal portal or periportal fibrosis, and no evidence of viral changes. On day of life 38, during the exploratory laparotomy, the patient was found to have complete absence of the extrahepatic biliary tree, or biliary aplasia, possibly a rare, severe form of BA. This report aims to increase our vigilance and help prevent diagnostic error in patients with signs and symptoms of BA who may produce pigmented stools. Primary care physicians should hence refer an infant (early and urgently) to a pediatric gastroenterologist for further workup for a direct bilirubin level >1.0 mg/dL with any total bilirubin level, irrespective of the color of the infant's stools.

Patience is a virtue: Multiple preoperative visits are associated with decreased recurrence in pediatric pilonidal disease.

BACKGROUND/PURPOSE: This study aimed to compare preoperative management strategies for patients undergoing trephination for pilonidal disease and evaluate risk factors for recurrence. METHODS: A retrospective review was performed of children undergoing index surgical treatment with trephination for pilonidal disease between September 2017 and April 2019. Intraoperative and postoperative management were standardized. Demographic and perioperative data were collected and analyzed. RESULTS: One-hundred twenty patients were identified with a median follow-up time of 7.5 months (interquartile range 4.1-13.2 months). Overall, 24 (20%) patients had a postoperative recurrence of pilonidal disease. Patients with multiple preoperative surgery clinic visits were less likely to have recurrent disease compared to those seen only once preoperatively (11% vs 26%, p = 0.040). Compared to patients without recurrence, those who recurred went to the operating room sooner from the time of initial surgical consultation (32 days vs 54 days, p < 0.001). Perioperative antibiotics, history of acute infection, and prior drainage procedures were not risk factors for recurrence. CONCLUSIONS: Multiple preoperative clinic visits are associated with a lower recurrence rate in children undergoing trephination for pilonidal disease. An increased duration of preoperative medical management may be responsible for this finding. Prospective study is needed to confirm these findings and identify additional factors that influence recurrence. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: III (Retrospective Comparative).

Ovarian volume ratio is a reliable predictor of ovarian torsion in girls without an adnexal mass.

PURPOSE: The aims of this study were to identify ultrasound-based predictors of ovarian torsion in girls without an adnexal mass and establish a set of normal values for ovarian volume ratio (OVR). METHODS: A retrospective review was performed of all premenarchal patients ≥3 years of age with a normal pelvic ultrasound between January 2016 and January 2019. A comparison group of premenarchal girls presenting between 2011 and 2019 with torsion in the absence of an adnexal mass was utilized. RESULTS: Five-hundred and four premenarchal girls underwent pelvic ultrasound evaluation with a normal examination. The mean OVR was 1.6 ±â€¯0.7 (range 1.0-6.5). OVR did not vary with age (r = -0.06) as compared to ovarian width which increased steadily with age (r = 0.53, p < 0.001). OVR was increased in girls with torsion (7.6 vs 1.4, p < 0.0001), and by receiver operating characteristic (ROC) analysis a cutoff value of >2.5 demonstrated the best diagnostic accuracy of any predictive variable (sensitivity 100%, specificity 94%, AUC 0.991, p < 0.001). CONCLUSIONS: OVR is an excellent predictor of ovarian torsion in premenarchal girls without an adnexal mass. Unlike ovarian width, OVR does not increase with age, and a cutoff OVR > 2.5 demonstrates high sensitivity and specificity for identifying ovarian torsion in this population. TYPE OF STUDY: Study of diagnostic test. LEVEL OF EVIDENCE: Level III.

Trephination versus wide excision for the treatment of pediatric pilonidal disease.

BACKGROUND/PURPOSE: To evaluate outcomes of trephination compared to wide excision in children undergoing initial surgical treatment of pilonidal disease. METHODS: A retrospective review was conducted of patients undergoing initial pilonidal excision between September 2017 and September 2018. Operations were categorized as either trephination or wide excision via an open or closed-wound technique. Outcomes were evaluated and data analyzed by chi-squared and one-way ANOVA tests. RESULTS: One-hundred and five patients were identified, with a mean follow-up of 4.6 months. Trephination was performed in 57% of patients, and of the remaining patients undergoing wide excision, 62% of wounds were left open. There were no demographic differences among the three groups. Compared to both the open and closed techniques, trephination was associated with fewer wound complications (17% vs 29% vs 3%, respectively, p = 0.006), and postoperative visits (4.4 vs 2.4 vs 1.4, respectively, p < 0.001). There was no difference in recurrence rates among groups. CONCLUSION: Trephination for pilonidal disease in pediatric patients is associated with a lower wound complication rate and fewer postoperative visits compared to wide excision. Recurrence rates are similar among the various surgical methods. Further prospective study would be useful to describe long-term outcomes of patients undergoing trephination for pilonidal disease. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: III (retrospective comparative).

Carinal Resection and Reconstruction for an Obstructing Inflammatory Myofibroblastic Tumor in a Child.

Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue tumor characterized by proliferation of fibroblastic cells associated with an inflammatory infiltrate. Inflammatory myofibroblastic tumors have a predilection for the pediatric population and are usually found in the lung parenchyma but rarely at the carina. They rarely metastasize but can be locally destructive. Surgical resection is the cornerstone of therapy, which results in excellent survival despite risk of local recurrence. We present the case of a nine-year-old girl with an IMT mass at the carina and obstructing the left main stem bronchus, requiring extensive resection and reconstruction.

Premenarchal patients present differently: A twist on the typical patient presenting with ovarian torsion.

BACKGROUND/PURPOSE: The aim of this study was to characterize differences between pre- and postmenarchal females with ovarian torsion. METHODS: A single-center review was conducted of all nonneonatal pediatric patients with ovarian torsion from 2011 to 2018. Clinical data were compared between pre- and postmenarchal patients. RESULTS: One hundred and twenty-seven patients were identified, and 25% were premenarchal. Premenarchal patients were more likely to have a delay in diagnosis (38% vs 20%, p = 0.042), develop ovarian necrosis (34% vs 17%, p = 0.036), and present without an associated adnexal mass (44% vs 0%, p < 0.001). All patients without a mass (n = 14) were premenarchal and presented with ovarian asymmetry. Patients without an associated mass underwent oophoropexy in the majority of cases. There were no differences in postoperative complication or recurrence rates between groups. CONCLUSION: Premenarchal females with ovarian torsion can present differently than females postmenarche and often have a delay in diagnosis. Premenarchal females are more likely to undergo torsion without an associated adnexal mass and are at higher risk for ovarian necrosis. Oophoropexy is an appropriate treatment in the absence of an adnexal mass. A high-index of suspicion for ovarian torsion should be maintained for premenarchal females presenting with abdominal pain and an otherwise negative workup. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: Level III.

Giant Bullous Emphysema With Placental Transmogrification: A Case Report of a 14-Year-Old With Right Middle- and Lower-Lobe Involvement.

Giant bullous emphysema with placental transmogrification is an extremely rare entity, with 30 previously reported cases. Of these reported cases, it is typically identified with varied clinical and radiological impressions, presents in young adulthood to elderly, is always unilateral, and usually involves just one lobe. Despite the unknown pathogenesis, this diagnosis carries an excellent prognosis and is curative with complete resection. The pulmonary placental transmogrification is histologically indistinguishable from placental origin. Although not necessary to utilize because of the male predominance and no reported association, immunohistochemical stains can be used to prove lung origin. We report an extremely rare case of 2-lobe involvement of giant bullous emphysema with placental transmogrification in a boy 14 years of age, who is the youngest diagnosed patient with this lung abnormality.

Esophageal Bronchus.

Communicating bronchopulmonary foregut malformations are uncommon anomalies. This report describes a case of "esophageal bronchus" managed successfully in a newborn with a nearly total pneumonectomy.

Fetal omphalocele in a common bottlenose dolphin (Tursiops truncatus).

A routine pregnancy ultrasound examination of a 30-yr-old, multiparous, common bottlenose dolphin, Tursiops truncatus, detected an approximately 16-wk (gestational age) fetus with an omphalocele, an abdominal wall defect at the base of the umbilical cord. Throughout the pregnancy, ultrasound allowed for identification of the omphalocele contents, which included a portion of the liver and intestinal loops. The maximum diameter of the omphalocele was 11.4 cm at an estimated 51-wk gestation. Color Doppler was utilized to study the blood flow within the omphalocele as well as diagnose an associated anomaly of the umbilical cord, which contained three vessels instead of four. Gross necropsy and histopathology confirmed the ultrasound diagnoses. This is the first report of an omphalocele in a T. truncatus fetus, and the first report of a fetal and umbilical cord anomaly diagnosed with ultrasound in a cetacean.

Genitourinary rhabdomyosarcoma: unusual diagnosis presenting within hours of delivery.

Genitourinary rhabdomyosarcoma of the newborn is extremely rare. We present a case report of a newborn delivered with a palpable abdominal mass revealing rhabdomyosarcoma on biopsy. Prenatal care was normal. The child was treated with multimodal therapy including extensive chemotherapy, surgical excision, and radiation therapy. Surgical excision included cystoprostatectomy, ureterostomy, abdominoperineal resection and colostomy placement. He continued to progress and eventually succumbed to his disease.

Magnet-associated intestinal perforation results in a new institutional policy of ferromagnetic screening prior to MRI.

Foreign body ingestions are common and the vast majority pass through the gastrointestinal tract without complication. Some ingestions, however, result in serious morbidity and mortality. We present a case in which the patient's chief complaint of severe posterior neck pain was unrelated to his foreign body ingestion (multiple magnets). The ingestion of magnets was not disclosed by the child to either the providing medical team or to the patient's family. In order to evaluate the patient's complaint of severe focal neck pain, MRI of the neck was performed. The authors believe it to be feasible that the MRI scan resulted in intestinal perforations that might not have occurred during the natural course of the ingestion. This complication might have been prevented if the patient had undergone screening with a ferromagnetic detector prior to entering the MRI suite. Because of the serious complications related to this case, all pediatric patients at our institution are now screened with ferromagnetic detectors prior to entering the MRI suite. We encourage nationwide policy revision to prevent further incidents similar to the one described in this case.

Acute appendicitis presenting with a painful inguinal mass: complication related to patent processus vaginalis and testicular maldescent.

Appendicitis with or without perforation is a common pediatric emergency department diagnosis, as are the findings of patent processus vaginalis and cryptorchidism. We describe the unique presentation of perforated appendicitis complicated by a tender inguinal mass resulting from the presence of purulence as well as a cryptorchid but viable testis within a patent processus vaginalis. We describe our diagnostic approach and review the literature relevant to inguinoscrotal presentations of appendicitis.

Renal tumors in the second decade of life: results from the California Cancer Registry.

BACKGROUND: Renal tumors are rare in adolescents and young adults. The aim of this study was to characterize the histologic condition, epidemiology, and survival of renal tumors in patients aged 11 to 20 years old using a large, population-based database. METHODS: The California Cancer Registry was reviewed from the years 1988 to 2004. All renal tumors in patients aged 11 to 20 years old were identified. The data were analyzed with relation to patient age, sex and ethnicity, tumor histologic examination, and actuarial mortality rates. RESULTS: Seventy-seven primary renal malignancies were identified. Thirty-nine (51%) were renal cell carcinoma, 23 (30%) were Wilms' tumor, and 15 (20%) were other tumor types. The mean age of the patients with renal cell carcinoma was 16.7 years old, which was significantly older than the Wilms' tumor patients (13.9 years; P < .01). The 5-year cumulative survival rate of patients with renal cell carcinoma was 54%, which was worse than that of Wilms' tumor patients (77%). CONCLUSION: Primary renal malignancies are uncommon in the second decade of life. The most common tumor type in this age-group is renal cell carcinoma followed by Wilms' tumor. Patients with renal cell carcinoma tend to be older and have a lower survival than patients with other kidney tumors.