Cloacal Dysgenesis Sequence in a Preterm Neonate.

BACKGROUND Cloaca malformations result from a disproportion of apoptosis, cell growth, and maturation. The range of cloacal malformations is extensive and diverse, with a lack of a straightforward classification system. Cloacal dysgenesis sequence (CDS), also known as urorectal septum malformation sequence, is a rare cloaca variant described as the absence of a perineal orifice. Prenatal magnetic resonance imaging and antenatal ultrasounds may reveal a cloacal malformation; however, many patients are not diagnosed with cloacal malformation until birth. CASE REPORT We present a case of a female neonate delivered by a 23-year-old G2P1T1A0L0 mother who had received comprehensive prenatal care. During pregnancy, bilateral multicystic dysplastic kidneys were identified prenatally, leading to the in utero placement of a vesicoamniotic shunt. The physical exam revealed a distended abdomen with reduced abdominal musculature and laxity, ascites, a vesicoamniotic shunt in place, absent urethra, ambiguous genitalia with no vaginal opening, no perineal opening, and clubfoot. Abdominal radiograph showed findings consistent with significant abdominal ascites. An exploratory laparotomy was performed that included diverting colostomy, mucous fistula creation, tube vaginostomy, removal of the vesicoamniotic shunt, and suprapubic tube placement. The patient recovered well from this operation with no complications. CONCLUSIONS CDS is an uncommon condition in pediatric patients, and although sonographic findings can reveal urinary tract abnormalities, prenatal imaging might not always identify CDS. Our case underscores the uniqueness of the case and the significance of early detection and immediate medical and surgical intervention.

Are pediatric surgery fellowship websites ready for the changing paradigms in the virtual interview era?

Purpose: With the COVID-19 pandemic, in-person fellowship interviews were curtailed, leading candidates to seek information from other resources. Our main purposes were (1) to determine what information recent participants in the match needed to evaluate programs and (2) to assess which of these were available online. Methods: A focus group of ten recent graduates/applicants identified information that was important in choosing a fellowship program. In August 2020 and December 2021, websites belonging to the American Pediatric Surgical Association (APSA) and individual programs were assessed. Results: Recent applicants identified 55 pieces of information considered important to their decision making. Of 57 pediatric surgery fellowships, 98% were listed on APSA's website. Program descriptions on APSA's website listed on average 60% of program information desired by applicants. All listed fellowship director, accreditation status, faculty list, and current fellow(s). Other descriptors frequently noted were alumni (95%), graduate's board performance (83%), ECMO exposure (77%), and curriculum (70%). Information desired but less frequently available were fellow case logs (63%), trauma center designation (53%), burn center designation (40%), research opportunities (30%), candidate interview assistance (25%), and supplemental fellowships (12%). There were 7% of program descriptions that were not updated for at least a year. Conclusions: APSA and individual program websites were complimentary. Websites often lacked data that applicants sought to inform their rank list. To best adapt to the evolving virtual interview paradigm, we suggest reporting key information on a central APSA website with more nuanced information available via links to program specific websites. Supplementary Information: The online version contains supplementary material available at 10.1007/s44186-023-00104-w.

Establishment of a successful robotic pediatric general surgery practice.

Robotic-assisted surgery (RAS) has a variety of theoretical advantages, including tremor filtration, optimal visualization, and improvement of surgeon ergonomics. Though it has achieved wide application in pediatric urology, the majority of pediatric general surgeons do not employ RAS. This study reports our institution's experience with RAS on a pediatric general surgery team. Following IRB approval, a retrospective review of all pediatric patients at our academic children's hospital who underwent RAS between 2017 and 2022 for pediatric general surgical conditions was performed. Patient demographics, operation performed, operating time, complications, and recovery were evaluated. A total of 159 children underwent RAS, increasing from 10 patients in 2017 to 59 patients in 2022. The median age and weight were 15.3 years and 76.4 kg, and 121 (76.1%) were female. The application of RAS was successful in all cases. There were no intraoperative complications and no conversions to an open approach. Eleven patients (6.9%) had unplanned presentations to the emergency department within 30 days. Five of these patients (3.1%) required admission to the hospital. This study demonstrates that the application of RAS in an academic pediatric general surgery practice is feasible and safe. The application of RAS to pediatric general surgery should continue to increase as operative teams increase their experience and comfort.Level of evidence Level IV.

Case report: Duplicated appendicitis with history of cloacal exstrophy causing bowel obstruction.

INTRODUCTION: Cloacal and bladder exstrophy are rare embryological defects that can cause developmental disruption of surrounding organ structures, the pelvis, spinal cord, and small intestines being the most commonly affected. Duplicated appendix is another rare embryological defect that has historically caused confusing clinical presentations. Our case highlights a rare instance of a patient with cloacal exstrophy who presented with a bowel obstruction and an associated inflamed duplicated appendix. CASE PRESENTATION: A newborn male is born with omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex. As primary surgical reconstruction was pursued, the patient was found to have a non-inflamed duplicated appendix, which was left unremoved. In the following months, the patient experienced episodes of small bowel obstruction, eventually requiring surgical intervention. During this operation, the duplicated appendix was noted to be inflamed, prompting removal of both appendices. DISCUSSION: This case highlights the increased prevalence of duplicated appendix in a patient with cloacal exstrophy, as well as the utility of prophylactic appendectomy for patients incidentally found to have a duplicated appendix intraoperatively. The duplicated appendix may lead to increased rates of complications and atypical presentation of appendicitis, supporting the practice of prophylactic appendectomy in patients with an incidentally found duplicated appendix. CONCLUSION: We suggest clinicians be aware of the association and potentially atypical presentation of appendicitis in patients with a duplicated appendix, particularly in the setting of cloacal exstrophy. The decision to prophylactically remove an incidentally found, non-inflamed duplicated appendix may be beneficial in preventing confusing clinical presentations and future complications.

What animal model should I use to study necrotizing enterocolitis?

Unfortunately, we are all too familiar with the statement: "Necrotizing enterocolitis remains the leading cause of gastrointestinal surgical emergency in preterm neonates". It's been five decades since the first animal models of necrotizing enterocolitis (NEC) were described. There remains much investigative work to be done on identifying various aspects of NEC, ranging from the underlying mechanisms to treatment modalities. Experimental NEC is mainly focused on a rat, mouse, and piglet models. Our aim is to not only highlight the pros and cons of these three main models, but to also present some of the less-used animal models that have contributed to the body of knowledge about NEC. Choosing an appropriate model is essential to conducting effective research and answering the questions asked. As such, this paper reviews some of the variations that come with each model.

Milky pericardial effusion causing tamponade in a neonate after extracorporeal membrane oxygenation cannulation.

Cardiac tamponade is a known life-threatening complication of extracorporeal membrane oxygenation (ECMO), often secondary to hemopericardium from major vascular or cardiac perforation. We present the unique case of a neonate with a milky pericardial effusion causing tamponade after ECMO cannulation, managed successfully with pericardial window. Understanding ECMO physiology and its effect on the classic presentation of tamponade is critical and can prevent delays in diagnosis. While hemopericardium is most commonly seen in these cases, findings of a non-bloody, milky effusion should prompt further workup for infection, chylopericardium or total parenteral nutrition-associated pericardial effusion, as the appropriate management can mitigate immediate and potential long-term sequelae.

Short-term outcomes of thoracoscopic versus open lobectomy for congenital lung malformations.

PURPOSE: Thoracoscopic and open approaches for the management of congenital lung malformations (CLM) has been debated. The aim of this study is to compare 30-day outcomes for non-emergent lobectomies in children. METHODS: The National Surgical Quality Improvement Program-Pediatric database was queried for patients undergoing CLM resection from 2013 to 2020. Outcomes were compared by operative technique in an intention-to-treat model and then propensity matched. RESULTS: 2157 patients met inclusion criteria and underwent non-emergent pulmonary lobectomy for CLM. The intended operative approach was thoracoscopic in 57.7% of patients. Patients in the open group compared to the thoracoscopic were more likely to be born premature, have chronic lung disease, require preoperative oxygen support, and be ventilator dependent. After propensity matching, there was no statistically significant difference in 30-day mortality, unplanned readmission, and other complications between the thoracoscopic and open groups. Thoracoscopic approach was associated with a shorter length of stay. The proportion of cases approached via thoracoscopy increased over time from 48.8% in 2013 to 69.9% in 2020. CONCLUSIONS: This large multicenter retrospective matched analysis demonstrates thoracoscopic lobectomy in children has similar favorable 30-day outcomes and shorter length of stay for the non-emergent management of CLM, compared to open thoracotomy. LEVEL OF EVIDENCE: Level III.

The Current Pediatric Surgery Job Market: A Perspective of Recent Fellowship Graduates.

PURPOSE: This study describes the job market from the perspective of recent pediatric surgery graduates. METHODS: An anonymous survey was circulated to the 137 pediatric surgeons who graduated from fellowships 2019-2021. RESULTS: The survey response rate was 49%. The majority of respondents were women (52%), Caucasian (72%), and had a median student debt burden of $225,000. Considering job opportunities, respondents strongly emphasized camaraderie (93%), mentorship (93%), case mix (85%), geography (67%), faculty reputation (62%), spousal employment (57%), compensation (51%), and call frequency (45%). 30% were satisfied with the employment opportunities available, and 21% felt strongly prepared to negotiate for their first job. All respondents were able to secure a job. Most jobs were university-based (70%) or hospital employed (18%) positions where surgeons covered median of two hospitals. 49% wanted protected research time, and 12% of respondents were able to secure substantial, protected research time. The median compensation for university-based jobs was $12,583 below the median AAMC benchmark for assistant professors for the corresponding year of graduation. CONCLUSION: These data highlight the ongoing need for assessment of the pediatric surgery workforce and for professional societies and training programs to further assist graduating fellows in preparing to negotiate their first job. TYPE OF STUDY: Survey LEVEL OF EVIDENCE: Level V.

Multiple Perianal Epidermal Cysts Found in a Case of Lowe Syndrome: A Case Report and Review of the Literature.

BACKGROUND Lowe syndrome, also known as oculocerebrorenal syndrome, is a rare genetic condition caused by an X-linked mutation of the OCRL1 gene, with an estimated prevalence in the general population of approximately 1 in 500 000. It is a multisystem disorder most commonly affecting the eyes, central nervous system, and kidneys. These commonly manifest as congenital cataracts, intellectual disability, and proximal renal dysfunction (Fanconi-type). Epidermal lesions are an uncommon manifestation of this condition, and the association is not completely understood. CASE REPORT Here we present a case of a 9-year-old boy with Lowe syndrome who presented with multiple cystic masses found in the perianal region. An excision was then performed to remove the masses and found that the lesions were epidermal cysts, which are infrequently found in Lowe syndrome. After excision, the patient recovered uneventfully without complications. CONCLUSIONS While epidermal cysts are an uncommon manifestation that have been documented, our case remains unique given the location and associated symptoms of the lesions. At presentation, the constellation of pain and perianal masses was concerning for a malignant etiology. However, after diagnostic imaging was performed, these lesions were found to be epidermal cysts, an infrequent manifestation of Lowe syndrome. Few previous case reports described cystic lesions in association with Lowe syndrome, and none, to our knowledge, have described multiple symptomatic perianal lesions. This case is important to consider because epidermal cystic lesions can be found with this presentation and should be considered on differential diagnoses for dermatologic findings in Lowe syndrome patients.

A 12-Year-Old Girl with Juvenile Granulosa Cell Tumor of the Ovary, Presenting with Adolescent Hyperprolactinemia, Galactorrhea, and Amenorrhea.

BACKGROUND Juvenile-type granulosa cell tumors (JGCTs) are a rare subtype of sex cord stromal tumor with a characteristic histology that is commonly found in the first 3 decades of life. It most commonly presents with symptoms of hyperestrogenism, which may present as precocious pseudopuberty or as menstruation-related symptoms, allowing for early detection of the tumor. CASE REPORT We present the case of a 12-year-old girl who presented to her primary care provider (PCP) with secondary amenorrhea with intermittent abdominal pain, who underwent an ultrasound for further evaluation, which revealed a large incidental pelvic mass. She was admitted to the Emergency Department (ED) and had findings of galactorrhea and hyperprolactinemia on examination. Imaging studies demonstrated a large ovarian mass measuring 15.0×9.0×18.8 cm that was resected, and subsequent pathology results showed JGCT stage 1A. CONCLUSIONS Prognosis of granulosa cell tumors (GCT) largely depends on its initial size, stage at diagnosis, residual tumors after surgery, and the subtype of GCT. If the patient is of reproductive age, fertility-sparing surgical options must be considered and patients must be regularly monitored for recurrence. JGCTs can present with minimal to no symptoms of precocious puberty in young girls but may present with amenorrhea, which may be considered normal for their developmental age. Although JGCTs are rare, they are important to include in differential diagnoses of younger female patients with abdominal pain, especially if accompanied by hormonal irregularities.

Prenatal administration of heparin-binding epidermal growth factor-like growth factor in an experimental model of necrotizing enterocolitis decreased both incidence and severity of the disease.

Background: Necrotizing enterocolitis (NEC) is the leading gastrointestinal cause of death in premature infants and causes long-term disabilities. Previously, enteral heparin-binding epidermal growth factor-like growth factor (HB-EGF) administered after birth demonstrated decreased incidence and severity of NEC in a neonatal animal model of NEC. We investigated the potential prophylactic strategy of preventing NEC using prenatally administered HB-EGF. Methods: An HB-EGF (800 µg/kg/dose) dose was injected into pregnant rats via tail vein or intraperitoneal route 2 hours prior to delivery. After cesarean section (C-section) at 21 days' gestation, the rat pups were subjected to the NEC protocol by inducing stressors: hypoxia, hypothermia, hypertonic feeds, and orogastric gavage of lipopolysaccharide (2 mg/kg). Postnatally, pups were monitored for 96 hours and assessed for the development of clinical and postmortem histological NEC. Results: The experimental NEC incidence in untreated, stressed rat pups was 66%. Compared with untreated pups, the maternal administration of HB-EGF correlated with a significant NEC incidence and severity decrease in rat pups. The strongest decrease was seen when HB-EGF was administered via the intraperitoneal route 2 hours prior to C-section (66% vs 31%, *p<0.05). Prenatal HB-EGF administration significantly increased pups' survival after NEC protocol exposure, with the greatest benefit observed in the group that received HB-EGF intraperitoneally 2 hours before delivery. Conclusions: Prenatal administration of HB-EGF decreases the incidence and severity of NEC, preserves gut barrier function and increases survival. This may represent a novel prophylactic clinical strategy for NEC offered to mothers at risk of delivering a premature infant.

An Unusual Case of Primary Ileal Trichobezoar Causing Intussusception.

BACKGROUND Intussusception, which is the prolapse of one section of intestine into another, is a common cause of small-bowel obstruction in pediatric patients. Bezoars are concretions of ingested foreign material. Trichobezoars, which are bezoars made of hair, occur in the context of trichotillomania, the compulsive pulling of hair, and trichophagia, the eating of hair. If gastric trichobezoars grow to sufficient size, an intestinal extension can serve as a lead point for intussusception to occur. Rarely, hair passes completely through the stomach and forms a trichobezoar within the small bowel. This obstruction can also create lead points and cause intussusception. This is one of the few reported cases of intussusception due to a primary intestinal bezoar. CASE REPORT We present the case of an 8-year-old boy with an unknown history of trichophagia and a preliminary diagnosis of appendicitis. Upon imaging, a bowel obstruction related to a small-bowel intussusception was discovered to be the probable cause. A diagnostic laparoscopy revealed an ileo-ileal intussusception caused by an ileal bezoar. Conversion to exploratory laparotomy assisted in removing the causative bezoar. The patient recovered without postoperative complications. CONCLUSIONS We report a case of an isolated intestinal trichobezoar causing intussusception in a boy. While intussusception secondary to a trichobezoar most commonly occurs due to 'Rapunzel syndrome,' this case shows that it is possible for an intestinal trichobezoar to form without the presence of 'Rapunzel syndrome.' This unique cause of intussusception presented as a small-bowel obstruction, requiring evacuation of the bezoar.

Objective Personality Determination: A Useful Addition to the Traditional Process of Pediatric Surgical Fellow Selection.

BACKGROUND: Selection of pediatric surgical trainees is a multifaceted process heavily influenced by in-person interviews to determine personality makeup and institutional compatibility. We present our experience in utilizing a validated personality inventory in the selection of our candidates. METHODS: All applicants selected for an in-person interview for the 2020 Pediatric Surgery Match were offered the Big Five Inventory (BFI) questionnaire. An initial rank order list (ROL) was formulated employing application information and in-person interview score. A reformulated ROL was created after incorporating BFI data. Correlation of specific personality characteristics vs position on the ROL was assessed for both the initial ROL and the reformulated ROL. RESULTS: Thirty-four applicants were interviewed and 24 (70.6%) were ranked. Linear regression analysis identified a lower neuroticism score directly related to a higher position on the initial ROL {R = .4626, P = .023}. The reformulated ROL identified a higher conscientiousness score {R = .5331; P = .007} and a lower neuroticism score {R = -.4383; P = .032} correlated with ascending the final ROL. CONCLUSIONS: The self-administered BFI adds objectivity to personality determination gleamed from the standard face-to-face interview. Conscientiousness and neuroticism had a significant impact on the final ROL position. Adding objective personality data appears to provide additional aid in this difficult process.

Large mesenteric cyst mimicking an ovarian cyst in an 8-year-old: A case report.

INTRODUCTION AND IMPORTANCE: Pediatric mesenteric cysts, rare and usually benign intra-abdominal tumors, are a difficult preoperative diagnosis due to ambiguous clinical characteristics. The final diagnosis is typically established only during surgery or histological analysis. CASE PRESENTATION: An 8-year-old female presented with five days of worsening abdominal pain, associated with nausea, vomiting, and fever, as well as vague tenderness in the right quadrants on examination. Computed tomography imaging showed a 10.5 × 8.7 × 7 cm abdominal mass, most suspicious for a cystic mass of ovarian origin. Upon diagnostic laparoscopy, a mesenteric cyst extending to the root of the mesentery was visualized and entirely resected after conversion to an exploratory laparotomy. Histopathological examination of both the cystic fluid and specimen suggest a benign mesenteric cyst. CLINICAL DISCUSSION: Although mesenteric cysts are noticeably rare, it is important differential to consider in pediatric patients with non-specific symptoms like abdominal pain and distention, intestinal obstruction, or a palpable abdominal mass. Notably, these cysts can be managed successfully by complete surgical resection with an excellent outcome. CONCLUSION: This report recounts an interesting case of a large mesenteric cyst that mimicked an ovarian cyst in a pre-pubertal girl.

Thoracoscopic segmentectomy for a large previously undiagnosed CPAM presenting as a spontaneous pneumothorax: A case report.

INTRODUCTION: Incidentally found congenital pulmonary airway malformations (CPAM) in older children are extremely rare and have traditionally been managed with minimally invasive versus open lobectomy of the affected lobe. PRESENTATION OF CASE: In this report, we present a 11-year-old male who presented with a recurrent spontaneous pneumothorax and was found to have a large symptomatic CPAM confined to a single segment of the right lower lobe. The patient was successfully treated with thoracoscopic segmentectomy without any residual disease seen on follow up imaging. DISCUSSION: Minimally invasive thoracoscopic approach has many advantages over open approach including better pain control, reduced hospital length of stay, and decreased intraoperative blood loss. With increasing use of minimally invasive approaches, lung-sparing surgery has demonstrated to be a viable and an attractive option for definitive resection of CPAM, without compromising resection margins and/or future lung function. CONCLUSION: This report demonstrates that minimally invasive lung-sparing surgical treatment of a large CPAM is feasible in older children.

Rare case report of anorectal malformation and intestinal atresia.

INTRODUCTION: Intestinal malformations, such as intestinal atresia, malrotation, and anorectal malformation, are rare events, but concurrent intestinal atresia and anorectal malformation are rare in combination. These anomalies have similar in utero and perinatal symptoms, which can make the diagnosis of both defects prenatally challenging. PRESENTATION OF CASE: We present a case of a male infant with a prenatal diagnosis of truncus arteriosus who on a 32-week routine prenatal ultrasound was suspected to have an intestinal malformation. On day of life one, the patient was taken to the operating room and found to have both type IIIa ileal atresia and anorectal malformation with normal bowel in between. A complete work up for vertebral defects, anorectal malformation, cardiac septal defects, esophageal atresia, renal anomalies, and radial limb defects (VACTERL) anomalies did not reveal additional anomalies. DISCUSSION: In this case, prenatal information supported intestinal obstruction, and the rare combination of both intestinal atresia and anorectal malformation proved surprising and interesting. CONCLUSION: We suggest providers be aware of the potential of multiple alimentary tract malformations to improve operative preparation and reduce the morbidity or mortality risk from repeat procedures when possible.

The diminishing experience in pediatric surgery for general surgery residents in the United States.

PURPOSE: The Accreditation Council for Graduate Medical Education (ACGME) regulates the general surgery residency curriculum. Case volume remains a priority as recent concerns surrounding a lack of proficiency for certain surgical cases have circulated. We hypothesize that there is a significant decrease in pediatric surgery case numbers during general surgery residency despite residents meeting the minimum case requirements. METHODS: We reviewed publicly available ACGME case reports for general surgery residency from 1999 to 2018. Cases are classified as Surgeon Chief or Surgeon Junior. Analyzed data included case classifications, number of residents, and number of residency programs. Simple linear regression analysis was performed. RESULTS: We identified a significant decrease in total number of logged pediatric surgery cases over the past 20 years (p<0.001). Nearly 60% of cases were logged under a single category - inguinal/umbilical hernia. From the past five years, pyloric stenosis was the only other category with an average of greater than two cases logged (range 2.1-2.8). CONCLUSION: We identified a significant decrease in total pediatric surgery case numbers during general surgery residency from 1999 to 2018. Though meeting set requirements, overall case variety was limited. With minimal number of cases required by the ACGME, graduating general surgery residents may lack proficiency in simple pediatric surgery cases.

Single site versus conventional laparoscopic appendectomy: some pain for no gain?

INTRODUCTION: The optimal laparoscopic appendectomy approach is not clear, comparing single site laparoscopic appendectomy (SILA) to conventional 3-port appendectomy (CLA). We investigated outcomes in pediatric patients comparing SILA to CLA: length of operation, length of stay, time to resumption of regular diet, follow up, rehospitalization, and cost. METHODS: Data was collected from children 1 to 18 years with appendectomy at Loma Linda University from 2018 to 2020, operated by two surgeons. Analysis utilized two-sample T, chi-squared, and Fisher's exact tests. RESULTS: Of 173 patients, 77 underwent SILA and 96 had CLA. There was no gender, age, or race difference between groups. Mean WBC was 17.5 × 103/mL in SILA group, compared to 15.3 × 103/mL in CLA group (P = 0.004). Operative time was 47.0 SILA compared to 49.5 minutes CLA (P = 0.269). Of SILA cases, 55.8% were simple appendicitis, while 53.3% of the CLA cases were simple (P = 0.857). Regular diet was resumed after 1.7 days in the SILA group, 1.1 days in CLA (P = 0.018). Length of stay was 2.9 days for SILA, 2.4 days for CLA (P = 0.144). Seven children required hospital readmission, 5 SILA and 2 CLA (P = 0.244). Five of the children who returned had intra-abdominal abscesses, of whom 4 had SILA. There was no difference in cost. CONCLUSIONS: The operative techniques had similar outcomes and operative times. There was a trend toward more intra-abdominal abscesses in the SILA group. Further study and longer follow up is needed to determine if there is an advantage to one laparoscopic approach over another.

Lipoblastomas and liposarcomas in paediatric patients: A case series.

Lipoblastomas and liposarcomas are rare causes of soft tissue masses in paediatric patients. In this retrospective clinical case series we identified 11 patients from our paediatric database (10 with a lipoblastoma and one with a liposarcoma) who had attended our hospital between 1998 and 2019. The median age of patients with lipoblastoma was 29 months. All lipoblastoma cases were managed with surgical excision and histological examination. The 18-year old patient with liposarcoma presented with a metastatic and unresectable tumour that was unresponsive to chemotherapy and radiation. Our experience demonstrates the importance of differentiating the type of soft tissue mass in children.