Intestinal Atresias.

Intestinal atresia is a form of congenital bowel obstruction that requires operative repair in the early neonatal period. Duodenal atresia and jejunoileal (JI) atresia are appropriately seen as distinct entities. Both can be suspected with fetal imaging, which can assist with prenatal counseling of families. Duodenal atresia is more commonly associated with comorbidities, whereas JI atresia is more often an isolated finding. Surgical repair is essential and is typically well tolerated. Although it may take time to achieve intestinal function postoperatively, these infants are usually able to tolerate full feeds after resolution of the ileus. Excellent short- and long-term outcomes for isolated duodenal atresia and JI atresia are expected.

Hypertrophic Pyloric Stenosis.

Hypertrophic pyloric stenosis is a common condition seen in the first 1 to 3 months after birth. Patients typically present with nonbilious projectile emesis after feeds that may result in hypokalemic, hypochloremic metabolic alkalosis. Although inability to tolerate feeds is frequently seen with self-limited conditions such as reflux, a low threshold to obtain an ultrasonographic image is important to prevent a delay in diagnosis. Although operative intervention is the treatment, it is imperative that patients are hydrated and serum electrolyte concentrations normalized before the induction of anesthesia. Laparoscopic pyloromyotomy is safe and effective. Postoperative emesis is normal, and reassurance to parents is appropriate. There is no significant long-term physiologic impairment from pyloric stenosis after successful surgical intervention.

Limiting hospital resources for acute appendicitis in children: Lessons learned from the U.S. epicenter of the COVID-19 pandemic.

INTRODUCTION: The COVID-19 pandemic resulted in the suspension of nonemergent surgeries throughout New York. Our tertiary care children's hospital pivoted towards a brief trial of intravenous (IV) antibiotic therapy in all patients in order to limit operating room (OR) utilization and avoid prolonged hospital stays. We describe our pandemic-based strategy for non-operative management (NOM) of appendicitis but with a limited duration of IV antibiotics. METHODS: We performed a retrospective study of children treated for acute appendicitis at our center from 3/31/2020 to 5/3/2020 during the peak of the New York pandemic. We compared appendicitis volume to similar months in prior years. We evaluated failure of NOM, length of stay, and compared characteristics of children we successfully treated with our expanded NOM protocol to previously published inclusion criteria for NOM. RESULTS: 45.5% of children (25/55) with acute appendicitis underwent NOM. Of the 30 who underwent surgery, 13 had complicated appendicitis while 17 had simple appendicitis. Three patients were COVID-positive, although none had respiratory symptoms. The majority of patients presenting with acute appendicitis (78.2%) did not meet previously published criteria for NOM. CONCLUSIONS: We treated a similar volume of children with acute appendicitis during the pandemic compared to prior years. We applied non-operative management to nearly half our patients, even as we expanded inclusion criteria for NOM to reduce OR utilization, but limited the duration of the antibiotic trial to avoid prolonged hospital stays. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: IV.

Clarifying Misleading Lumps and Sinuses in the Newborn.

Neonates often have congenital lumps or sinuses. It is expected that pediatricians will distinguish those with important physiologic implications from those without. Accurate understanding of these lesions is important for the practitioner to avoid unnecessary tests and anxiety and to ensure that seemingly benign lesions with important implications are addressed in a timely manner. This review aims to clarify the consequences of some lesions that can easily be misinterpreted, offering guidance in the initial management of patients with congenital lumps or sinuses. We address several lesions that can easily be misconstrued, including wattles, preauricular lesions, sacral sinuses, second branchial cleft anomalies, torticollis, and dermoid cysts.

Is fecal diversion necessary during ileal pouch creation after initial subtotal colectomy in pediatric ulcerative colitis?

BACKGROUND: Pediatric patients with medically refractory ulcerative colitis (UC) often undergo an initial subtotal colectomy end ileostomy (STC-I). The role of fecal diversion in the subsequent completion proctectomy/ileal-pouch anal anastomosis (CP-IPAA) remains controversial. METHODS: A multi-institutional retrospective review was performed of pediatric UC patients who underwent an STC-I followed by CP-IPAA from 2008 to 2016. 37 patients were included [diverted (n = 20), undiverted (n = 17)]. RESULTS: Children who underwent undiverted CP-IPAA had a longer length of stay (days) compared to the diverted group (9, 6.5-13 vs. 6, 5-6, p = 0.002). The 30-day complication rate was significantly higher in the undiverted group (p = 0.003) although the difference in anastomotic leak, readmission rate, unplanned computer tomography use, and reoperation was not statistically significant. Three patients with undiverted CP-IPAA required additional surgery in the perioperative period for fecal diversion. The mean long-term follow-up was 25.68 ± 21.56 months. There were no significant differences in functional pouch outcomes. CONCLUSIONS: Patients who underwent an undiverted CP-IPAA after initial STC-I had significantly more complications in the immediate postoperative period compared to diverted patients, although this did not translate into long-term differences in functional outcomes. Questions remain regarding careful patient selection and counseling for undiverted pouches in the pediatric UC population.

Perinatal hemorrhage from ulceration of the umbilical cord: A potentially catastrophic association with duodenal and jejunal obstruction.

PURPOSE: The purpose of this study is to review published reports and contribute new cases of umbilical cord ulceration (UCU) with perinatal hemorrhage into the amniotic cavity in the setting of duodenal or jejunal obstruction because knowledge of this sequence is poorly disseminated and could be lifesaving. METHODS: Published reports of UCU with hemorrhage associated with congenital duodenal or jejunal obstruction were reviewed. Chart review was conducted for the cases encountered at our institutions between January 2008 and March 2017. We noted perinatal complications, method of delivery, gestational age, birth weight, gender, number, location, and pathologic description of umbilical cord ulcers, and outcome. RESULTS: Thirty-one reports and 7 new cases were studied. Perinatal complications included: preterm labor or preterm premature rupture of membranes: 63%; fetal distress: 95%; mean gestational age: 33weeks; premature gestation: 95%; bloody amniotic fluid: 90%. Pathological analysis of UCUs revealed solitary, multifocal, helical and punched-out lesions. There were 12 neonatal deaths (32%), and 12 intrauterine deaths (32%). Survival rate was 37%. CONCLUSIONS: UCU with perinatal hemorrhage is associated with duodenal and jejunal obstruction. Knowing the typical clinical signs of this potential catastrophic complication could prompt lifesaving delivery. TYPE OF STUDY: Prognostic LEVEL OF EVIDENCE: IV.

Necrotizing Enterocolitis.

Necrotizing enterocolitis (NEC) is a frequently encountered condition in the premature neonate, which can have devastating effects. The signs and symptoms of NEC are variable and can be confused with those of sepsis. An abdominal radiograph is often obtained for diagnosis, and findings that indicate NEC include pneumatosis and portal venous gas. The treatment of NEC includes gastrointestinal rest, gastric decompression, broad-spectrum intravenous antibiotics, and systemic support. A finding of pneumoperitoneum signifies intestinal perforation, which requires surgical intervention. Long-term sequelae of NEC include short-gut syndrome, intestinal stricture, and neurodevelopmental delays. The presentation of intestinal stricture can be puzzling. It can appear at presentation as a bowel obstruction or, conversely, as increased stool output or diarrhea. The clinician should have a high level of suspicion for intestinal stricture in a patient with a history of NEC.

Trends in the Diagnosis and Management of Pediatric Appendicitis.

• On the basis of class B evidence and consensus, acute appendicitis in children can often be diagnosed clinically with only selective use of imaging. (13)(14)(15)(16) • On the basis of class B evidence and consensus, ultrasonography is the test of choice when acute appendicitis is suspected but is unclear based on history, physical examination, and laboratory results. (17)(18)(19) • On the basis of class B evidence and consensus, the use of computed tomography scan should be limited to cases of suspected complex appendicitis with abscess or when there is clinical suspicion for acute appendicitis but ultrasonography results are not helpful. (16) • On the basis of class C evidence and consensus, children with possible appendicitis ideally should be treated in medical centers that have skilled sonographic personnel. (21) • On the basis of class B evidence and consensus, simple appendicitis should be treated by appendectomy during normal operating hours. Preoperative treatment with intravenous antibiotics and fluids during the overnight hours halts disease progression and allows for the safest surgery with the benefit of a full and rested staff. (24)(25)(26) • On the basis of class B evidence and consensus, complex appendicitis with a well-defined abscess can be treated nonoperatively initially, with the option of an interval appendectomy after recovery from the acute infection. (29)(30) (31)(32)(33)

Recent trends in the operative experience of junior pediatric surgical attendings: a study of APSA applicant case logs.

PURPOSE: Pediatric surgical education and workforce have changed significantly in the past decade. To document trends in the operative experiences of junior pediatric surgeons, we examined case logs submitted by applicants for membership to APSA. METHODS: Case logs for 164 APSA membership applicants from 2006 to 2013 were reviewed. Total case volume, categories, and specific operations were analyzed. Negative binomial regression assessed for significant associations between the number of cases and the application year, presence of a pediatric surgery training program, region of the country, and years since fellowship completion. RESULTS: Overall case numbers decreased initially after 2006/2007, but have remained stable since. Decreasing trends were seen in a number of specific cases/categories. The number of newborn cases did not change. Significant variations in operative experience were identified depending upon region, presence of a pediatric surgery training program, and years since fellowship completion. Median reported value for several important cases was ≤4 per year, and for some was zero. CONCLUSION: These data describing the experience of young pediatric surgeons supplement recent observations regarding pediatric surgery fellows and general surgery residents. The limited exposure of surgeons to particularly rare conditions appears to be an unresolved problem. This information will be useful in developing future workforce proposals.

Placental mesenchymal dysplasia with hepatic mesenchymal hamartoma: a case report and literature review.

Placental mesenchymal dysplasia (PMD) is characterized by placentomegaly and grapelike vesicles resembling a partial molar pregnancy and in most cases, a phenotypically normal fetus. Hepatic mesenchymal hamartoma (HMH) is a benign hamartomatous proliferation of mesenchymal liver tissue. PMD has been associated with HMH. Although rare, in combination, it is known to carry a poorer prognosis than in fetuses without structural abnormalities. There are only a few reported cases of PMD and associated HMH with varying management strategies and outcomes, precluding ascertainment of the most appropriate treatment plan. We present a case of PMD with associated cystic HMH resulting in fetal death. We also reviewed the published literature on this issue and explored possible management strategies to prevent adverse fetal and neonatal outcomes.

Life-threatening postoperative hemorrhage from hepatic artery pseudoaneurysm successfully treated by transcatheter embolization in a 5-year-old.

Upper gastrointestinal bleeding caused by a pseudoaneurysm after hepatobiliary operation is well described in adults. This can be successfully treated with transcatheter embolization under angiographic guidance. We report a case of massive upper gastrointestinal bleeding in a 5-year-old boy secondary to a pseudoaneurysm of the right hepatic artery after choledochal cyst excision. A coil embolization successfully treated this life-threatening event and spared the child, the morbidity of a challenging gastrointestinal and vascular reconstruction. Use of percutaneous interventional technique to treat this rare complication of choledochal cyst excision has not been previously described in the pediatric surgical literature. Transcatheter embolization of a pseudoaneurysm may be a safe and less morbid treatment alternative for this surgical complication even in the pediatric population.

A comparison of the cleft lift procedure vs wide excision and packing for the treatment of pilonidal disease in adolescents.

BACKGROUND/PURPOSE: The cleft lift for pilonidal disease is a flap procedure designed to counteract suspected causes of closed-technique failure. This study compares cleft lift with wide excision and packing in adolescents with respect to complications, healing, and recurrence. METHODS: Charts of all patients surgically treated for pilonidal disease at our institution from August 2000 to August 2009 were reviewed retrospectively. Wide excision was routinely performed until May 2007 when the cleft lift as described by Bascom was instituted here. Factors examined were postoperative complications, wound healing, and disease recurrence. RESULTS: Seventy patients (49 males, 21 females; mean age, 16 years; mean weight, 170.5 lb) with pilonidal disease underwent a total of 39 cleft lift procedures and 34 wide excision procedures. All but 1 cleft lift patient (97.4%) healed completely, whereas 25 (73.5%) of 34 patients in the excision group healed (P < .001). The remaining 9 excision patients had chronic wounds, 3 of whom have undergone cleft lift with full healing. One cleft lift patient had recurrent disease (2.5%) compared with 7 (20.6%) of 34 excision patients (P < .02). CONCLUSIONS: The cleft lift procedure is a superior treatment method of pilonidal disease in adolescents, resulting in primary healing, lower likelihood of recurrent disease, and simplified wound care.

Nonoperative management of symptomatic urachal anomalies.

INTRODUCTION: Symptomatic urachal anomalies are rare disorders that consist of urachal remnants or fistulas with or without an associated cyst. Traditionally, when a urachal anomaly was recognized, operative excision was performed. There has been a shift toward the nonoperative management of urachal anomalies at many centers, although there is little in the literature to support this practice. METHODS: A retrospective chart review of patients with urachal anomalies was performed from January 2002 to March 2008. Children with a draining umbilicus and no radiographic or surgical confirmation of a urachal anomaly were excluded. RESULTS: Fifteen patients with symptomatic urachal anomalies were identified. The average age was 3.5 years (4 weeks to 14 years). Symptoms included umbilical drainage (n = 10), abdominal pain (n = 6), omphalitis (n = 4), intraabdominal mass (n = 3), dysuria (n = 1), recurrent urinary tract infections (n = 1), and fever (n = 4). The diagnosis was confirmed by ultrasound (n = 13) and/or computed tomographic scan (n = 4). The surgically treated cases included 7 urachal cysts (5 uninfected, 2 infected) and 1 patent urachal fistula. Mean follow-up is 37 months, and there have been no reported recurrences. Those treated without surgical excision included 4 patent urachal fistulas (mean follow-up, 20 months-no recurrences) and 3 infected urachal cysts (percutaneous drainage [n = 2] and laparoscopic drainage [n = 1]-no recurrences on ultrasound at 26 months). CONCLUSION: Nonoperative management of urachal anomalies is a reasonable approach and may be extended to infected urachal cysts after initial drainage. Infected cysts that are adequately drained seem to obliterate with time. Modern ultrasonography facilitates thorough follow-up. We propose a treatment algorithm for the management of suspected urachal anomalies.

Failure of appendectomy to resolve appendiceal intussusception.

Appendiceal intussusception is a rare entity that primarily affects children. The optimal surgical management is not known. We describe a child with appendiceal intussusception treated by simple appendectomy who suffered recurrent intussusception of the cecum at the base of the appendiceal stump. He was cured by resection of a rim of cecum around the appendiceal staple line. Partial cecectomy with appendectomy may be the most effective operation for appendiceal intussusception.

Pitfalls in prenatal diagnosis of unusual congenital abdominal wall defects.

We report three cases of unusual skin covered abdominal wall defects not accurately diagnosed by prenatal sonography. An associated omphalocele was recognized in two but misinterpreted as a giant omphalocele in one. Suspicious sonographic features--an enlarged abdominal circumference, irregular laxity of the abdominal--may be clarified by MRI.