Epidemiology of gastroschisis: A population-based study in California from 1995 to 2012.

BackgroundAlthough the incidence of gastroschisis is increasing, risk factors are not clearly identified.MethodsUsing the Linked Birth Database from the California Office of Statewide Health Planning and Development from 1995 to 2012, patients with gastroschisis were identified by ICD-9 diagnosis/procedure code or birth certificate designation. Logistic regressions examined demographics, birth factors, and maternal exposures on risk of gastroschisis.ResultsThe prevalence of gastroschisis was 2.7 cases per 10,000 live births. Patients with gastroschisis had no difference in fetal exposure to alcohol (p = 0.609), narcotics (p = 0.072), hallucinogenics (p = 0.239), or cocaine (p = 0.777), but had higher exposure to unspecified/other noxious substances (OR 3.27, p = 0.040; OR 2.02, p = 0.002). Gastroschisis was associated with low/very low birthweight (OR 5.08-16.21, p < 0.001) and preterm birth (OR 3.26-10.0, p < 0.001). Multivariable analysis showed lower risk in black (OR 0.44, p < 0.001), Asian/Pacific Islander (OR 0.76, p = 0.003), and Hispanic patients (OR 0.72, p < 0.001) compared to white patients. Risk was higher in rural areas (OR 1.24-1.76, p = 0.001). Compared to women age < 20, risk decreased with advancing maternal age (OR 0.49-OR 0.03, p < 0.001). Patients with gastroschisis had increased total charges ($336,270 vs.$9012, p < 0.001) and length of stay (38.1 vs. 2.9 days, p < 0.001). Mortality was 4.6%.ConclusionsThis is the largest population-based study summarizing current epidemiology of gastroschisis in California.Type of studyRetrospective comparative cohort study.Level of evidenceIII

Thoracoamniotic shunt placement for a right-sided congenital diaphragmatic hernia complicated by hydrops

AbstractMortality associated with congenital diaphragmatic hernia (CDH) is high, and the role of prenatal management continues to evolve. We report a case of a right-sided CDH complicated by fetal hydrops successfully managed with thoracoamniotic shunt placement. Subsequent ultrasounds indicated resolution of hydrops. Despite preterm premature rupture of membrane and preterm delivery at 32 3/7 weeks gestation, the infant survived to hospital discharge at 2.5 months of life. This is the first case of a hydropic right-sided CDH successfully treated with a thoracoamniotic shunt

Necrotizing enterocolitis following diazoxide therapy for persistent neonatal hypoglycemia.

Recalcitrant neonatal hypoglycemia poses a treatment challenge for clinicians. When a patient's hypoglycemia does not respond to dextrose infusion, several medication options are available, including diazoxide(1,2). Several side effects of diazoxide are described in the literature, including fluid retention with the risk of development of congestive heart failure(3,4). We describe a case of necrotizing enterocolitis in a patient with Beckwith-Wiedemann Syndrome with persistent neonatal hypoglycemia who was treated with increasing doses of diazoxide

Resection of a thoracoabdominal ganglioneuroma via a retroperitoneal minimally invasive approach.

A 10-year-old girl presented to her pediatrician with a history of cough and fever. A chest radiograph revealed a paraspinal mass. On cross-sectional imaging, the mass traversed the diaphragm, extending from T9 to L1 spinal levels with involvement of the T10-12 neural foramen. Vanillylmandelic and homovanillic acid levels were normal. On review of historical radiographs, the mass had increased in size. Thus, surgical resection was recommended for diagnosis and treatment. The patient was placed in left lateral decubitus position. The retroperitoneal space was accessed inferior to the twelfth rib. One 12 mm and two 5 mm ports were used. Development of the retroperitoneal space was achieved with both blunt dissection and a vessel-sealing device. The diaphragm was incised to resect the thoracic component of the mass. The tumor was adherent at the neural foramen and was resected flush with the spine. The diaphragm repaired primarily. She was discharged home on post-operative day four without complication. Pathology demonstrated a ganglioneuroma. The patient was well at her follow-up, and imaging one year postoperatively was without recurrence. No additional treatment was required. A laparoscopic retroperitoneal approach allows for a safe, minimally invasive resection of a thoracoabdominal mass without violation of the abdominal cavity

Resection of a thoracoabdominal ganglioneuroma via a retroperitoneal minimally invasive approach

A 10-year-old girl presented to her pediatrician with a history of cough and fever. A chest radiograph revealed a paraspinal mass. On cross-sectional imaging, the mass traversed the diaphragm, extending from T9 to L1 spinal levels with involvement of the T10-12 neural foramen. Vanillylmandelic and homovanillic acid levels were normal. On review of historical radiographs, the mass had increased in size. Thus, surgical resection was recommended for diagnosis and treatment.The patient was placed in left lateral decubitus position. The retroperitoneal space was accessed inferior to the twelfth rib. One 12 mm and two 5 mm ports were used. Development of the retroperitoneal space was achieved with both blunt dissection and a vessel-sealing device. The diaphragm was incised to resect the thoracic component of the mass. The tumor was adherent at the neural foramen and was resected flush with the spine. The diaphragm repaired primarily. She was discharged home on post-operative day four without complication. Pathology demonstrated a ganglioneuroma. The patient was well at her follow-up, and imaging one year post-operatively was without recurrence. No additional treatment was required.A laparoscopic retroperitoneal approach allows for a safe, minimally invasive resection of a thoracoabdominal mass without violation of the abdominal cavity. Keywords: Pediatric laparoscopic, Retroperitoneoscopic, Ganglioneuroma, Ganglioneuroblastoma, Neuroblastoma, Thoracoabdominal resectio

Midgut volvulus and complex meconium peritonitis in a fetus with undiagnosed cystic fibrosis

In utero small bowel volvulus with meconium peritonitis is a rare complication of cystic fibrosis.We report the case of fetal small bowel volvulus with necrosis, perforation and meconium peritonitis in a fetus with undiagnosed cystic fibrosis. The mother presented with four days of decreased fetal movement and ultrasound findings of fetal small bowel dilation with wall thinning and ascites. The fetus' status declined three days thereafter, prompting an emergent delivery. The infant was born with peritonitis and underwent an exploratory laparotomy with a small bowel resection and interval anastomosis. Following restoration of continuity, the patient was able to tolerate oral and enteral nutrition with appropriate growth. This report provides an example of the signs, symptoms and sonographic findings associated with this rare fetal complication and explores the intricacies of prenatal genetic testing. Keywords: Cystic fibrosis, Fetal cystic fibrosis complication, Fetal small bowel volvulus, Fetal small bowel perforation, Complicated meconium peritoniti

Novel Use of a Bronchial Blocker in a Challenging Case of Congenital Diaphragmatic Hernia-A Case Report.

The diagnosis of congenital diaphragmatic hernia (CDH) is associated with significant morbidity and mortality. Survival of neonates with CDH has improved recently, although the clinical course is complicated by sequelae of hypoplastic pulmonary parenchyma and vasculature, pulmonary hypertension, ventilation/perfusion (V/Q) mismatch, reduced pulmonary function and poor somatic growth. In this case report, we describe an infant with an antenatal diagnosis of CDH with a poor prognosis who underwent initial surgery followed by a tracheostomy but had a worsening clinical course due to a large area of ventilated but poorly perfused lung based on a V/Q nuclear scintigraphy scan. The emphysematous left lung was causing mediastinal shift and compression of the right lung, further compromising gas exchange. The infant had clinical improvement following bronchial blockade of the under-perfused left lung. This paved the way for further management with resection of the under-perfused lung lobe and continued clinical improvement. We present the novel use of selective bronchial blockade in a challenging case of CDH to determine if surgical lung resection may benefit the infant. We also review the physiology of gas exchange during the use of a bronchial occluder and the relevant literature

The Utility of Discharge Antibiotics in Pediatric Perforated Appendicitis Without Leukocytosis.

INTRODUCTION: Optimal management of pediatric perforated appendicitis remains a topic of active investigation. Our institutional clinical practice guidelines (CPGs) were modified to discontinue antibiotics on discharge for patients with normal white blood cell count (WBC) without left shift. We hypothesized that patients would receive fewer antibiotics without increased complications. METHODS: Patients <18 y old with perforated appendicitis who underwent laparoscopic appendectomy between November 1, 2016 and May 31, 2021 at a tertiary care childrens hospital were included. Primary outcome was adverse events: postdischarge surgical site infection (SSI), 30-day emergency department (ED) visits, or readmissions. Outcomes were compared before and after CPG modification. Multivariable regression was performed to identify factors associated with SSI. RESULTS: There were 113 patients pre- and 97 patients post-CPG modification. 23.1% of patients in the pre-cohort had an elevated discharge WBC or left shift compared to 18.9% of patients in the post-cohort (P = 0.48). Significantly fewer patients were prescribed antibiotics on discharge in the post-cohort (70.8% pre versus 14.4% post, P < 0.0001) and for fewer days (2 pre versus 0 post, P < 0.0001). Total antibiotic days decreased significantly (6.1 pre versus 4.6 post, P < 0.0001). There was an increase in postdischarge SSIs on univariate analysis (1.8% pre versus 9.3% post, P = 0.03), ED visits (9.7% pre versus 19.6% post, P = 0.04), and readmissions (5.3% pre versus 11.3% post, P = 0.13). On multivariable analysis, being in the post-cohort was not significantly associated with post-discharge SSIs after adjusting for sex, symptom duration, initial WBC, and discharge antibiotic duration (OR 0.25, 95% CI 0.04-1.4, P = 0.11). CONCLUSIONS: Modification of a pediatric perforated appendicitis clinical practice guideline to discontinue antibiotics on discharge with a normal WBC without left shift was effective in decreasing antibiotic duration. This was associated with an increase in SSIs on univariate analysis, which did not persist on multivariable analysis and requires further investigation

Malignant primitive epithelioid sarcoma with features of rhabdoid tumor presenting in utero with diffusely metastatic disease.

Diagnosis of a tumor in utero is a rare occurrence and poses diagnostic and therapeutic challenges. In cases of tumor-associated hydrops, there is significant risk of fetal demise, and prenatal intervention may be considered to avoid this outcome when possible. When fetal intervention is unlikely to improve survival, information can be useful for counseling families. We present a rare case of fetal diagnosis of a primary renal malignancy with widespread metastases and hydrops, with unique immunohistochemical findings consistent with malignant primitive epithelioid sarcoma with features of rhabdoid tumor