Open abdominal wall defects and open spina bifida at a regional hospital in northern KwaZulu-Natal - bellwether conditions for neonatal surgery capacity.

BACKGROUND: Abdominal wall defects (AWDs), such as gastroschisis and omphalocele, and neural tube defects (NTDs) such as open spina bifida (SB) are common congenital anomalies. These anomalies are considered a leading cause of neonatal mortality and have been advocated as bellwether conditions to measure access to surgical care. METHODS: Newborns with open SB or AWD presenting to the nursery at Queen Nandi Regional Hospital over four years (2018-2021) were retrospectively identified. Clinical and electronic database records were reviewed to determine if transfers to definitive tertiary care occurred timeously. Reasons for delays and associated morbidity and/or mortality were investigated. RESULTS: Sixty-five patients were identified and two were excluded due to unavailable or incomplete records. It took a median of 8 days (IQR 2-18 days) to reach tertiary care, with SB cases waiting significantly longer (median 16 days,IQR 8-25 days) (p = 0.000). Lack of tertiary service capacity was the main reason for delays. The COVID-19 pandemic did not affect time intervals (p = 0.676). Complications were common and overall mortality at our facility was high (n = 11/63, 17.46%). CONCLUSION: Newborns with open SB or AWDs experience marked delays in reaching definitive care. This is more pronounced for cases of SB and was not influenced by the pandemic. Lack of tertiary service capacity (including bed availability, limited staff, and theatre time) is the most important limiting factor.

Persistent and newly developed gastrointestinal symptoms after surgery for intestinal malrotation in children: Dysmotility or disorders of gut and brain interaction?

OBJECTIVES: Surgery for intestinal malrotation (IM) aims to correct the defect and improve symptoms; however, many have persistent gastrointestinal (GI) symptoms postoperatively. We evaluated the incidence, clinical presentation, and long-term outcomes of children with surgically repaired IM and its possible association with disorders of gut and brain interaction (DGBI). METHODS: Multicenter retrospective study was conducted in patients from 0 to 21 years old, who had surgery for IM from 2000 to 2021 across three pediatric tertiary care centers. Data analyzed included demographics, time to diagnosis, idiopathic diagnosis, incidental diagnosis, postoperative follow-up, surgical time, and the need for surgery including bowel detorsion. Outcome variables were the presence of postoperative GI symptoms and DGBIs, and overall resolution of symptoms. We also evaluated the potential association of demographics and other included variables with our outcome variables. RESULTS: Ninety-two patients with surgically corrected IM were included, 54% were male, and median age of diagnosis and surgical correction was 4.9 and 7.8 months, respectively. Median follow-up after surgery was 64 months. A total of 77% had postoperative GI symptoms, and notably, 78% of patients without symptoms before surgery (incidental diagnosis) developed GI symptoms postoperatively and 27% of patients met Rome IV criteria for a one or more DGBI. No factors were associated to the presence of postoperative symptoms or DGBIs in multivariate analysis. Female gender was the only factor associated with lack of resolution of symptoms at follow-up. CONCLUSION: Pediatric IM is commonly associated with postoperative GI symptoms and DGBI well beyond surgery. An increased awareness about the prevalence of DGBI in these patients may help reach a prompt and accurate diagnosis, and improve their quality of life.

Persistent disparities between trauma center types in the management of children with high-grade blunt splenic injuries.

BACKGROUND: In the early 2000s, substantial variations were reported in the management of pediatric patients with blunt splenic injury (BSI). The purpose of this study was to assess the recent trends and disparities between different types of trauma centers. We hypothesized that there would be persistent disparities despite decreased trends in the rate of splenectomy. METHODS: This is a retrospective cohort study using the American College of Surgeons Trauma Quality Improvement Program database. We included patients (age ≤18 years) with high-grade BSI (Abbreviated Injury Scale 3-5) between 2014 and 2021. The patients were divided into three groups based on trauma center types (adult trauma centers [ATCs], mixed trauma centers [MTCs], and pediatric trauma centers [PTCs]). The primary outcome was the splenectomy rate. Logistic regression was performed to evaluate the association between trauma center types and clinical outcomes. Additionally, the trends in the rate of splenectomy at ATCs, MTCs, and PTCs were evaluated. RESULTS: A total of 6601 patients with high-grade BSI were included in the analysis. Overall splenectomy rates were 524 (17.5%), 448 (16.3%), and 32 (3.7%) in the ATC, MTC, and PTC groups, respectively. ATCs and MTCs had significantly higher splenectomy rates compared to PTCs (ATCs: OR = 5.72, 95%CI = 3.78-8.67, and p < 0.001 and MTCs: OR = 4.50, 95%CI = 2.97-6.81, and p < 0.001), while decreased trends in the splenectomy rates were observed in ATCs and MTCs (ATCs: OR = 0.92, 95%CI = 0.87-0.97, and p = 0.003 and MTCs: OR = 0.92, 95%CI = 0.87-0.98, and p = 0.013). CONCLUSIONS: This study suggested persistent disparities between different trauma center types in the management of children with high-grade BSI.

Exploring the clinical characteristics and prevalence of the annular pancreas: a meta-analysis.

BACKGROUND: The annular pancreas (AP) is a rare gastrointestinal congenital malformation, in which malrotation of the pancreatic ventral bud in the seventh week of embryonic development manifests in a partial or complete ring of tissue around the second part of the duodenum. METHODS: The main online medical databases such as PubMed, ScienceDirect, Wiley online library, Web of Science, and EBSCO discovery service were used to gather all relevant studies on the AP. RESULTS: A total of 12,729,118 patients were analyzed in relation to the prevalence of AP. The pooled prevalence of AP was 0.0045% (95% CI: 0.0021%-0.0077%). The most frequent comorbidity in adults and children was duodenal obstruction, with a pooled prevalence of 24.04% and 52.58%, respectively (95% CI: 6.86%-46.48% and 35.56%-69.31%, respectively). The most frequent operation in adult patients with AP was duodenojejunostomy, with pooled prevalence established at 3.62% (95% CI: 0.00%-10.74%). CONCLUSION: The diagnostic complexity of AP is accentuated by its nonspecific clinical symptoms, making accurate identification reliant on imaging studies. Therefore, having a thorough knowledge of the clinical characteristics of the AP and its associated anomalies becomes paramount when faced with this rare congenital condition.

Ileal prolapse through patent omphalomesenteric duct in a two year-old boy: a case report.

BACKGROUND: Patent omphalomesenteric duct is one of the birth defects included in the spectrum of vitelline duct abnormalities. It is a rare anomaly with estimated prevalence of 0.13-0.2% in the general population. The most common presentation of patent vitelline duct is yellowish or mucoid type umbilical discharge which is usually noted in neonatal age or infancy. The main stay of diagnosis is clinical and outcome is favorable as long as timely surgical correction is offered. Here we present a 2 years old male child who presented with ileal prolapse through patent vitelline duct which is an exceptional mode of presentation of this pathology. CASE PRESENTATION: 2 years old Ethiopian male child who was noticed to have umbilical discharge since early infancy presented with protrusion of pinkish mass per the umbilicus of 4 h duration. He had no signs and symptoms of bowel obstruction. Abdominal examination revealed a prolapsed bowel which was viable via the umbilicus which was about 6 cm long. Otherwise, he had no abdominal tenderness or rigidity. He was explored with a smiley incision just above the umbilicus. The prolapsed bowel was reduced gently to the abdominal cavity. The tract of the Patent vitelline duct was identified and completely resected along with a wedge of ileum at its base. Primary repair of the ileal end where the tract was inserted was done in two layers and abdomen was closed in layers. The child had smooth post op course and was discharged on the 4th post-operative day. CONCLUSION: Prolapse of a bowel through the umbilicus is unusual presentation of a rare anomaly namely patent vitelline duct. This presentation warrants early surgical intervention before bowel ischemia issues. Hence, all clinicians dealing with children should be aware of this rare pathology so that urgent surgical management can be offered.

Investigation of a transient increase in omphalocele prevalence in a birth cohort of TRICARE beneficiaries.

BACKGROUND: The Department of Defense Birth and Infant Health Research (BIHR) program leverages medical encounter data to conduct birth defect surveillance among infants born to military families. Omphalocele is a major abdominal wall defect with an annual prevalence of ~2 per 10,000 births in BIHR data, but an unexpected increase was observed during 2017-2019, reaching 6.4 per 10,000 births in 2018. To investigate this transient increase in prevalence, this study aimed to validate the omphalocele case algorithm among infants born 2016-2021. METHODS: Omphalocele cases were identified by ICD-10 code Q79.2 (exomphalos) on one inpatient or two outpatient infant encounter records and validated using parental and infant electronic health records. Characteristics of true and false positive cases were assessed using bivariate analyses and compared over time. RESULTS: Of 638,905 live births from 2016 to 2021, 230 met the ICD-10 case definition for omphalocele; 138 (60.0%) cases were eligible for validation, of which 68 (49.3%) were true positives. The geometric mean time from birth to first ICD-10 omphalocele diagnosis was 1.1 (standard error [SE] 0.1) days for true positives and 11.9 (SE 3.1) days for false positives. Among the 70 false positives, 36 (51.4%) were cases of confirmed umbilical hernia; rates of umbilical hernia and delayed omphalocele diagnoses (>30 days after birth) were elevated among false positives during 2017-2019. CONCLUSIONS: Higher misuse of ICD-10 code Q79.2 during 2017-2019 likely influenced the associated increase in omphalocele prevalence. Timing of diagnosis should be considered for omphalocele case definitions using medical encounter data.

Factors associated with D-lactic acidosis in pediatric intestinal failure: A case-control study.

BACKGROUND: D-lactic acidosis (DLA) is a serious complication of short bowel syndrome (SBS) in children with intestinal failure (IF). Malabsorbed carbohydrates are metabolized by bacteria in the intestine to D-lactate which can lead to metabolic acidosis and neurologic symptoms. METHODS: A retrospective chart review was performed in children ≤18 years old with SBS who had one of the following criteria: unexplained metabolic acidosis, neurologic signs or symptoms, history of antibiotic therapy for small bowel bacterial overgrowth, or high clinical suspicion of DLA. Cases had serum D-lactate concentration >0.25 mmol/L; controls with concentrations ≤0.25 mmol/L. RESULTS: Of forty-six children, median age was 3.16 (interquartile range (IQR): 1.98, 5.82) years, and median residual bowel length was 40 (IQR: 25, 59) cm. There were 23 cases and 23 controls. Univariate analysis showed that cases had significantly lower median bicarbonate (19 vs. 24 mEq/L, p = 0.001), higher anion gap (17 vs. 14 mEq/L, p < 0.001) and were less likely to be receiving parenteral nutrition, compared with children without DLA. Multivariable analysis identified midgut volvulus, history of intestinal lengthening procedure, and anion gap as significant independent risk factors. Midgut volvulus was the strongest independent factor associated with DLA (adjusted odds ratio = 17.1, 95% CI: 2.21, 133, p = 0.007). CONCLUSION: DLA is an important complication of pediatric IF due to SBS. Patients with IF, particularly those with history of midgut volvulus, having undergone intestinal lengthening, or with anion gap acidosis, should be closely monitored for DLA.

Adult-onset congenital intestinal malrotation: A case report and literature review.

BACKGROUND: Intestinal malrotation is an infrequent congenital anomaly primarily observed in neonates, and adult-onset cases are exceedingly rare. Studies on adult congenital intestinal malrotation are limited. METHODS: A case with congenital intestinal malrotation is reported in our study. The clinical data were collected and the treatment process and effect were evaluated. RESULTS: A 45-year-old female who had been experiencing vomiting for over 40 years was admitted to our hospital. According to the result of CT scan, intestinal volvulus accompanied by bowel obstruction was suspected. Then laparoscopic examination was applied to the patient and was ultimately diagnosed with adult congenital intestinal malrotation. We performed Ladd's procedure combined with gastrojejunostomy and Braun anastomosis. The patient recovered well and was successfully discharged from the hospital on the 13th day after surgery. After a 6-month follow-up, the symptom of vomiting was significantly alleviated and body weight was gained for 10 kg. She was very satisfied with the treatment. CONCLUSION: Adult congenital intestinal malrotation is a rare disease that is often misdiagnosed owing to nonspecific clinical manifestations. Therefore, awareness about this condition should be enhanced. Surgery remains the cornerstone of treatment for this disease. Combining gastrojejunostomy and Braun anastomosis with the traditional Ladd procedure can optimize surgical outcomes.

The people behind the papers - Julia Grzymkowski and Nanette Nascone-Yoder.

As the digestive system develops, the gut tube lengthens and convolutes to correctly package the intestine. Intestinal malrotation is a prevalent birth anomaly, but its underlying causes are not well understood. In this new study, Nanette Nascone-Yoder and colleagues show that exposure of Xenopus embryos to atrazine, a widely-used herbicide, can disrupt cellular metabolism in the developing gut tube and lead to intestinal malrotation. We caught up with first author Julia Grzymkowski and corresponding author Nanette Nascone-Yoder, Professor at North Carolina State University, to hear more about the story.

Left-sided appendicitis managed laparoscopically: a case report.

BACKGROUND: Appendicitis is one of the most common causes of acute abdominal pain and remains the most common abdominal-related emergency seen in emergency room that needs urgent surgery (Yang et al. in J Emerg Med 43:980-2, 2012. 10.1016/j.jemermed.2010.11.056, Wickramasinghe et al. in World J Surg 45:1999-2008, 2021. 10.1007/s00268-021-06077-5). The characteristic presentation is a vague epigastric or periumbilical discomfort or pain that migrates to the lower right quadrant in 50% of cases. Other related symptoms, such as nausea, anorexia, vomiting, and change in bowel habits, occur in varying percentages. The diagnosis is usually reached through comprehensive history, physical examination, laboratory tests, and radiological investigations as needed. Nowadays, computed tomography of the abdomen and pelvis is considered the modality of choice for definitive assessment of patients being evaluated for possible appendicitis. Anatomical variations or an ectopic appendix are rarely reported or highlighted in literature. CASE PRESENTATION: Left-sided appendicitis is a rare (Hu et al. in Front Surg 2022. 10.3389/fsurg.2022.896116) and atypical presentation and has rarely been reported. The majority of these cases are associated with congenital midgut malrotation, situs inversus, or an extremely long appendix (Akbulut et al. in World J Gastroenterol 16:5598-5602, 2010. 10.3748/wjg.v16.i44.5598). This case is of significance to raise awareness regarding an anatomical variation of the appendix that might delay or mislead diagnosis of appendicitis and to confirm safety of a laparoscopic approach in dealing with a left-sided appendicitis case (Yang et al. in J Emerg Med 43:980-2, 2012. 10.1016/j.jemermed.2010.11.056). We report a case of left-sided appendicitis in a 12-year-old child managed successfully via a laparoscopic approach. CONCLUSION: Appendicitis remains the most common abdominal-related emergency that needs urgent surgery (Akbulut et al. in World J Gastroenterol 16:5598-5602, 2010. 10.3748/wjg.v16.i44.5598). Left-sided appendicitis is a rare (Hu et al. in Front Surg 2022. 10.3389/fsurg.2022.896116, Hu et al. in Front Surg 9:896116, 2022. 10.3389/fsurg.2022.896116) and atypical presentation and has rarely been reported. Awareness regarding an anatomical variation of the appendix and diagnostic modalities on a computed tomography scan help avoid delay in diagnosis and management of such a rare entity (Vieira et al. in J Coloproctol 39(03):279-287, 2019. 10.1016/j.jcol.2019.04.003). A laparoscopic approach is a safe approach for management of left-sided appendicitis (Yang et al. in J Emerg Med 43:980-2, 2012. 10.1016/j.jemermed.2010.11.056, Hu et al. in Front Surg 9:896116, 2022. 10.3389/fsurg.2022.896116).

[Intestinal malrotation accompanied by a right paraduodenal hernia in an adult: a case report].

A 72-year-old woman was admitted to our department in March 2020 for an evaluation of nausea, vomiting, diarrhea, liver dysfunction, and hypokalemia, which had persisted intermittently since 2013. Thickening of the descending duodenal wall and a sac-like appearance the intestinal tract in the vicinity of the duodenal papilla were observed in abdominal computed tomography. No duodenojejunal curvature, with two intestinal loops identified in the descending region, was detected in contrast-enhanced upper gastrointestinal imaging. Based on these imaging findings, the patient was diagnosed with intestinal malrotation (incomplete rotation and fixation) accompanied by a right paraduodenal hernia based on the Nishijima classification. Thus, surgery was performed at our hospital. Gastrointestinal symptoms did not recur, and liver dysfunction and hypokalemia improved postoperatively.

Patent vitellointestinal duct with ileal prolapse in a newborn: A case report and literature review.

RATIONALE: Patent vitellointestinal duct is the most common omphalomesenteric duct anomaly to present with symptoms. PATIENT CONCERNS: A 10-day-old child presented with increase in the size of a polypoidal lesion into a large, "Y"-shaped reddish, prolapsing lesion, discharging gaseous, and fecal matter at her umbilicus. A laparoscopic exploration was performed, followed by wedge resection and anastomosis. No complications occurred during postoperative follow-up. DIAGNOSES: A patent vitellointestinal duct with ileal prolapse. INTERVENTIONS: The resection of extended intraperitoneal intestinal tube was performed. OUTCOMES: During the follow-up 3 months after surgery, the umbilical cord of the child healed well after surgery. LESSONS: Timely surgical treatment can minimize the occurrence of complications, and the overall prognosis is good after surgery.

Liver abnormalities following SARS-CoV-2 infection in children 1 to 10 years of age.

OBJECTIVE: Beginning in October 2021 in the USA and elsewhere, cases of severe paediatric hepatitis of unknown aetiology were identified in young children. While the adenovirus and adenovirus-associated virus have emerged as leading aetiological suspects, we attempted to investigate a potential role for SARS-CoV-2 in the development of subsequent liver abnormalities. DESIGN: We conducted a study using retrospective cohorts of deidentified, aggregated data from the electronic health records of over 100 million patients contributed by US healthcare organisations. RESULTS: Compared with propensity score matched children with other respiratory infections, children aged 1-10 years with COVID-19 had a higher risk of elevated transaminases (HR (95% CI) 2.16 (1.74 to 2.69)) or total bilirubin (HR (95% CI) 3.02 (1.91 to 4.78)), or new diagnoses of liver diseases (HR (95% CI) 1.67 (1.21 to 2.30)) from 1 to 6 months after infection. Patients with pre-existing liver abnormalities, liver abnormalities surrounding acute infection, younger age (1-4 years) or illness requiring hospitalisation all had similarly elevated risk. Children who developed liver abnormalities following COVID-19 had more pre-existing conditions than those who developed abnormalities following other infections. CONCLUSION: These results indicate that SARS-CoV-2 may prime the patient for subsequent development of liver infections or non-infectious liver diseases. While rare (~1 in 1000), SARS-CoV-2 is a risk for subsequent abnormalities in liver function or the diagnosis of diseases of the liver.

Diagnostic accuracy of upper gastrointestinal series in children with suspected intestinal malrotation.

Intestinal malrotation (IM) results from an altered or incomplete rotation of the fetal midgut around the superior mesenteric artery axis. The abnormal anatomy of IM is associated with risk of acute midgut volvulus which can lead to catastrophic clinical consequences. The upper gastro-intestinal series (UGI) is addressed as the gold standard diagnosis procedure, but a variable failure degree has been described in literature. The aim of the study was to analyze the UGI exam and describe which features are the most reproducible and reliable in diagnosing IM. Medical records of patients surgically treated for suspected IM between 2007 and 2020 at a single pediatric tertiary care center were retrospectively reviewed. UGI inter-observer agreement and diagnostic accuracy were statistically calculated. Images obtained with antero-posterior (AP) projections were the most significant in terms of IM diagnosis. Duodenal-Jejunal Junction (DJJ) abnormal position resulted to be the most reliable parameter (Se = 0.88; Sp = 0.54) as well as the most readable, with an inter-reader agreement of 83% (k = 0.70, CI 0.49-0.90). The First Jejunal Loops (FJL), caecum altered position and duodenal dilatation could be considered additional data. Lateral projections demonstrated an overall low sensitivity (Se = 0.80) and specificity (Sp = 0.33) with a PPV of 0.85 and a NPV of 0.25. UGI on the sole AP projections ensures a good diagnostic accuracy. The position of the third portion of the duodenum on lateral views showed an overall low reliability, therefore it was not helpful but rather deceiving in diagnosing IM.

Surgical Strategies for Neonates with Prenatally Diagnosed Congenital Biliary Dilatation.

BACKGROUND: This study aimed to develop a postnatal treatment strategy for infants with prenatally diagnosed congenital biliary dilatation. METHODS: We performed a retrospective study of patients with prenatal diagnosed congenital biliary dilatation (CBD), aged <1 year who underwent surgery at our hospital between 2013 and 2023. We classified the patients into two groups, the "early group," consisting of patients who could not wait for growth, and required early surgery, and the "scheduled group," consisting of patients who were asymptomatic and could undergo scheduled surgery, and compared them. The parameters for early surgical prediction were AST, ALT, TB, DB, and CRP levels at birth, 1 week, 2 weeks, 1 month, 2 months, and 3 months after birth, and immediately before surgery, as well as the cyst diameter, presence of intrahepatic bile duct dilation, and presence of debris in the common bile duct. RESULTS: During the study period, 15 patients were diagnosed prenatally. The cyst diameter was significantly larger at all points in the early group. Patients with a cyst diameter of >30 mm at birth, intrahepatic bile duct dilatation at birth, and postnatal enlargement of the common bile duct to >30 mm are more likely to develop symptoms early. Blood biochemistry tests showed no significant differences between the two groups. CONCLUSIONS: Patients with a cyst diameter >30 mm in the early postnatal period require careful postnatal management and parents should be counseled regarding the high likelihood of their child needing surgery within the first 3 months of life. LEVEL OF EVIDENCE: Level IV.

[Ovarian masses in infants and children]. / Raumforderungen des Ovars im Kindesalter.

BACKGROUND: Abnormalities of the ovary are frequently seen on ultrasound examination, sometimes symptomatic, but are more commonly asymptomatic. PURPOSE: Presentation of the most important entities of ovarian masses and their imaging features in infants and children. Discussion of criteria for differentiation between benign and potentially malignant masses. MATERIALS AND METHODS: Review of current literature and presentation of image examples. RESULTS: The most common lesions are ovarian cysts in infants, which usually do not require therapy. Because of the risk of torsion, surgery should be discussed for lesions with a size of 5 cm or more. Benign teratomas represent three-quarters of all solid tumors of the infantile ovary. Malignant masses are rare. The task of imaging is to assess the potential risk of malignancy, also using imaging scores. CONCLUSIONS: Imaging plays a crucial role for therapeutic considerations. Depending on the potential risk, ovarian-sparing surgery is preferred to preserve fertility, as long as the oncologic risk is reasonable.

'It's just a mucocele': a case report of a massive appendiceal mucocele presenting as a left upper quadrant mass.

Tumours of the appendix are very rare tumours that can and often present with a mucocele. This is a case report highlighting the associated pathology of appendix tumours and the management of a large mucocele. Specifically, how a right hemicolectomy is very rarely needed in these cases regardless of size and local anatomical relationships and some important considerations for the practicing surgeon in the non-tertiary centre that encounters a case like this.