Patent processus vaginalis: role of ultrasound in pediatric population with unilateral inguinal hernia and other predictors.

INTRODUCTION: Inguinal hernia (IH) repair is a common procedure in the daily practice of pediatric surgeons. In a developing country with limited facilities, it is important to be able to predict and diagnose contralateral patent processus vaginalis (CPPV) to avoid the risk and cost of further surgery. OBJECTIVES: To assess the accuracy of ultrasound for the detection of CPPV, using laparoscopic evaluation as a confirmatory test. We also looked for various predictors of CPPV in our study population. METHODS: 141 patients were included in this 2-year, cross-sectional prospective study. Inclusion criteria were unilateral inguinal hernia elective patients with no major comorbidities and aged between 2 months and 8 years. Each patient was assessed in outpatient clinics and then a pre-operative ultrasound was conducted. This was followed by laparoscopic evaluation during repair of the hernia. RESULTS: Of the 141 patients included, 110 (78%) were males, 121 (85.9%) were born at full term, and 96 (68.1) had right-sided hernia. Mean age was 2.64 ± 1.9 years. Ultrasound was 85.7% sensitive in the detection of CPPV, 90.8% specific, and 90.1% accurate. In our analysis of patients younger than 1 year, right-sided hernia and defect size more than 10 mm were statistically significant predictors for a CPPV. CONCLUSIONS: Ultrasound has a high accuracy profile and is a useful alternative in limited resource settings with restricted access to minimally invasive surgery for the prediction of CPPV. Patients younger than 1 year with a right-sided hernia or a manifested hernia defect larger than 10 mm are at a higher risk of having a CPPV.

Transhepatic ultrasound guided embolization as a successful novel technique in treatment of pediatric complex intrahepatic arterioportal fistula: a case report and review of the literature.

INTRODUCTION: Intrahepatic vascular shunts "IHVS" are abnormal communications between intra-hepatic vasculature involving the arterial, portal, or hepatic venous system. Arterio-portal fistula "APF" is an intrahepatic communication between the hepatic arterial system and the portal venous system without any communication with the systemic venous circulation. APF is considered a rare cause of portal hypertension and gastrointestinal bleeding in infancy. CASE PRESENTATION: A 3-month-old Mediterranean female with known cardiac congenital anomalies presented to us with abdominal distension and diarrhea. Ultrasonography revealed massive ascites and computerized tomography (CT) abdomen with intravenous (IV) contrast revealed a left hepatic lesion. On further evaluation, an intrahepatic arterio-portal vascular malformation was detected. Attempted trans arterial embolization failed and radiology team successfully carried out direct trans hepatic ultrasound guided coiling of the aneurysmal venous sac followed by successful resection of segment 4 of the liver with the vascular malformation avoiding life threatening intra operative bleeding. CONCLUSION: Any child with recurrent gastrointestinal bleeding, failure to thrive, vomiting, diarrhea, steatorrhea, splenomegaly, or ascites should be investigated for intrahepatic arterio-portal fistula "IAPF". Our novel technique of direct trans hepatic ultrasound guided coiling is an alternative method if trans arterial embolization "TAE" failed.

Complicated urachal cyst in two pediatric patients: a case report.

BACKGROUND: A urachal cyst has a rare incidence that has been reported as 1/5,000 live birth. CASE PRESENTATION: We report two patients with a complicated urachal cyst, a 5-year-old female who presented to the emergency department with severe abdominal pain and a 3-year-old female presenting with abdominal pain and constipation. Upon laparoscopic exploration both patients had complicated urachal cysts which were adherent to the urinary bladder. CONCLUSION: Complicated urachal cysts can present with acute abdominal pain.

Laparoscopic inguinal hernia repair in bladder exstrophy, a new modified solution to an old problem: A cohort study.

PURPOSE: Open inguinal hernia repair in children with bladder exstrophy is challenging and associated with a high recurrence rate (15%-22%). We report our initial experience with laparoscopic repair of inguinal hernias in five children with repaired bladder exstrophy. This study is the third describing inguinal hernia repair in bladder exstrophy patients using the laparoscopic approach. In this study, we report a different laparoscopic technique. METHODS: This retrospective study was performed on data regarding laparoscopic inguinal hernia repair collected over one year (July 2019-2020). We carried out the laparoscopic inguinal hernia repair, closing the internal inguinal ring using a non-resorbable sliding knot suture and incorporating the transversalis fascia for reinforcement, followed by purse-string closure of the peritoneum. Peri- and postoperative outcome parameters, including recurrence rate, were evaluated over a follow-up period of 14 months. RESULTS: A total of seven male patients from July 2019 to 2020 were admitted to our center with a history of repaired bladder exstrophy and reducible inguinal hernias. Two patients had open inguinal hernia repair due to parental preference and five patients had laparoscopic repair. In the laparoscopic group three patients had bilateral inguinal hernias in one of them a metachronous hernia was discovered intraoperstively, and the remaining two patients had unilateral hernias, one on the right side and the other on the left side. All patients in the laparoscopic group had an uneventful recovery and were discharged within 24 h and there were no complications or recurrences during follow-up. CONCLUSION: Laparoscopic inguinal hernia repair is a better alternative to managing inguinal hernias in children with bladder exstrophy.

Umbilical hernia repair post umbilical cord graft closure of gastroschisis: A cohort study.

INTRODUCTION: Gastroschisis a common congenital anomaly in the anterior abdominal wall, the bowel is present outside the abdominal cavity, completely devoid of any coverings, management of gastroschisis involves umbilical cord graft coverage of the defect after bowel reduction when there are concerns about compartmental syndrome, this is a widely used technique but there are few reports about the incidence umbilical hernia development after this technique and need for future repair of the defect. PRESENTATION OF CASES: We had 8 patients with simple gastroschisis who had umbilical cord graft coverage of the defect at birth between 2017 and 2020, we present 4 patients who had the cord graft without cutting of rectus fascia, 2 patients resolved spontaneously and 2 developed an umbilical hernia requiring repair. DISCUSSION: Umbilical cord graft has been reported in several studies, in those studies the authors reported the spontaneous closure of the defect and some reported that incising the rectus fascia will contribute to development of the umbilical hernia, in our series the rectus fascia was preserved yet 2 patients developed umbilical hernia. CONCLUSION: Pediatric surgeons should look out for umbilical hernia in patients who had umbilical cord graft repair of gastroschisis defect and closure should be carried out by an experienced surgeon.

Early onset left ventricular remodeling in juvenile systemic lupus erythematosus; Insight from 3-dimensional speckle tracking echocardiography.

BACKGROUND: Early diagnosis and treatment of myocardial affection in patients with systemic lupus erythematosus (SLE) are crucial. OBJECTIVES: To evaluate the ventricular systolic function in juvenile-onset systemic lupus erythematosus (j-SLE) patients by 3-D speckle tracking echocardiography (3D-STE) and to determine the predictors of left ventricular (LV) dysfunction if present. METHODS: Twenty-six SLE patients without heart failure and 21 healthy controls were studied by standard echocardiogram and 3D-STE. Conventional parameters included LV ejection fraction (EF), fractional shortening (FS), and mitral annular plane systolic excursion (MAPSE). Global LV strain (GLS) and global area strain (GAS) were obtained by 3D-STE. Medical records, including diagnosis criteria, duration of disease, and SLE disease activity index (SLEDAI) were evaluated. RESULTS: The mean age was similar in patients and controls 11.42 vs 11.48 years p = 0.93. The mean duration of the disease was 1.87 ± 1.02 years and SLEDAI ranged from 0 to 9. By conventional and tissue Doppler imaging echocardiography, only MAPSE was significantly lower in SLE patients compared to controls (14.56 vs 18.46 mm, p < 0.001). By 3D speckle tracking echocardiography, GLS and GAS were significantly reduced in SLE patients compared to controls (-15.07 vs -19.9.4%, -34.6% vs -39.7%, respectively, p < 0.001). Multiple linear regression and ROC analyses indicated that the SLEDAI score was the only predictive factor for the left ventricular remodeling. CONCLUSIONS: These results indicate that early subclinical LV dysfunction occur in jSLE patients even with normal EF and SLE disease activity might be a potential driver for LV deformation.

Laparoscopic-assisted transanal pull-through for hirschsprung's children older than 3 years: A case series.

CONTEXT: Hirschsprung's disease (HD) is a congenital anomaly affecting the enteric nervous system commonly the rectosigmoid region. Treatment is surgical where the aganglionic segment is resected, and bowel continuity is restored by a coloanal anastomosis. In 1999, Georgeson et al. proposed a new technique of primary laparoscopic-assisted pull-through for HD as a new gold standard. AIM: The aim of the study was to evaluate the outcome of the laparoscopic-aided transanal pull-through procedure for the management of HD in children older than 3 years of age. METHODS: This study was conducted on 15 consecutive patients presented to a university hospital, diagnosed as having HD relying on their clinical features, barium enema study and rectal biopsy. In all cases, laparoscopic-assisted transanal pull-through was attempted. The laparoscopic part included transition zone identification, seromuscular biopsy for fresh frozen histopathology and sigmoid and rectal mobilisation as much as possible down the peritoneal reflection. The transanal part included mobilisation of the rectal lower segment by 2-3 cm, resection till the ganglionic segment, and full-thickness two-layer coloanal anastomosis was done. RESULTS: Early complications occurred in eight cases: enterocolitis in four cases and perianal excoriation occurred in four cases. There were no cases of anastomotic leak. Late complications occurred in seven patients: four cases developed stricture at the site of coloanal anastomosis and three cases experienced enterocolitis at 6 and 9 months postoperatively. CONCLUSION: Laparoscopic-aided transanal pull-through procedure is a safe, feasible modality for the management of HD in children more than 3 years.

Hernia uteri inguinale in an 18 months old female infant: A case report.

INTRODUCTION: Inguinal hernia is the most frequent hernia of childhood. About 15-20% of females with inguinal hernias, the ovaries and/or the fallopian tubes may be contents of the herniation sac. Meanwhile uterus is very rare to be encountered. Our aim is to alert Pediatric surgeons to such condition and remind them of the important technical steps to treat and manage this condition. CASE REPORT: Elective surgery on an 18 months old female child with right inguinal hernia was performed electively through an open inguinal approach; the uterus, fallopian tubes and ovary were found in the hernia sac. DISCUSSION: The etiology of the hernia uterine inguinale in general is controversial, however there may be some degree of weakness of the uterine and ovarian suspensory ligament. Thompson noted that if there is failure of fusion of the Mullerian ducts, it leads to increase in the mobility of ovary and uterus, increasing the possibility of herniation of ovary, tubes and uterus. It was reported to be associated with disorders of sexual development. Surgical procedure for inguinal hernias containing uterus is often different from the ones containing only the ovary, as these organs are strongly attached to the hernia sac and it has to be freed from the wall of the hernia sac. CONCLUSION: We want to stress that in hernia uterii ovarii, dissection of the sac is essential for successful hernia repair unlike routine hernioraphy and to consider associated disorder of sexual differentiation.

A report of two infant cases operated for jejunal duplication cyst associated with malrotation and volvulus.

INTRODUCTION: Both Enteric duplication and intestinal malrotation are concerning causes for intestinal obstruction in the pediatric age group and they very rarely coexist in the same patient. PRESENTATION OF CASES: We present 2 cases of previously healthy children, the first is a 4-month-old infant and the second is a 1.5-year-old boy, both presented with recurrent attacks of bilious vomiting that proved to be due to acute midgut volvulus caused by an enteric duplication cyst associated with intestinal malrotation. DISCUSSION: Enteric duplication and intestinal malrotation are two of the concerning causes of billious vomiting in the pediatric age group. They could be encountered at any level of the alimentary tract from the tongue to the anus. The term malrotation refers to all abnormalities of intestinal position. The coincidence of intestinal malrotation and an enteric duplication cyst (EDC) is very rare and has been described only in a few case reports. CONCLUSION: The concomitance of EDC and intestinal malrotation is extremely rare and should be kept in mind in a child presenting with bilious vomiting especially in a child preoperatively diagnosed with a duplication cyst.

Fetus in Fetu: Lessons Learned from a Large Multicenter Cohort Study.

INTRODUCTION: Fetus in fetu (FIF) is an extremely rare condition of abnormal twinning during embryogenesis. Most publications are single case reports. We describe the combined experience of four large tertiary referral centers with FIF which were not previously reported or published, and thereby draw conclusions to establish criteria for the workup, diagnosis, and management including intraoperative risk. MATERIALS AND METHODS: A survey was forwarded to a national pediatric surgery group which includes members from all pediatric surgery centers in the country enquiring about unpublished cases of FIF encountered over a 20-year interval. The cohort was analyzed for age of presentation, type of presentation, diagnostic workup, surgical management, and outcome. RESULTS: From 1998 to 2018, a total of 10 FIF cases were included in the study. Mean age of presentation was 4 months. Computed tomography and ultrasound were the main preoperative diagnostic modality in our cohort. Resection of the mass was curative in nine cases. Two cases in which the FIF was in direct topographic proximity to the biliary tree suffered severe intraoperative or lethal postoperative complications. CONCLUSION: Complete excision of FIF is the treatment of choice and generally results in excellent long-term quality of life. Mortality is rare and may be associated with biliary involvement and retroperitoneal right upper quadrant location of the FIF tends to be associated with increased risk in excision, and there is also a possible association with the presence of immature elements in the pathology report.

Cecal Volvulus in a child with congenital dilated cardiomyopathy: A case report.

INTRODUCTION: Cecal volvulus is an extremely rare cause of intestinal obstruction in the pediatric age group and its incidence is unknown. PRESENTATION OF CASE: We present a unique case of cecal volvulus in a 3 year old female with congenital dilated cardiomyopathy. DISCUSSION: Cecal mobility due to malfixation and malrotation is the main cause. Cecal Volvulus usually presents with constipation, abdominal pain and distention. Common complications entail strangulation, ischemia and gangrene. Cecal volvulus has been reported in association with various abnormalities. However, Incidence of cecal volvulus in a child with congenital dilated cardiomyopathy has not been reported. Surgery is the mainstay of management. CONCLUSION: Cecal volvulus should be kept in mind in a child presenting with signs suggestive of intestinal obstruction.

A case report of an asymptomatic male child with multiple entero-enteric fistulae post multiple magnet ingestion.

INTRODUCTION: Foreign body ingestion cases are very common in children. They usually present with obvious symptoms allowing proper diagnosis and management; however, magnet ingestion can be asymptomatic. This disorder is considered uncommon as well as challenging to diagnose before complications of intestinal obstruction or peritonitis occur due to entrapment of intestinal walls in the magnetic field. CASE PRESENTATION: An asymptomatic four-year-old male child was admitted to our centre after passing two bullet-shaped magnets in stool that were noticed by his mother. The child was asymptomatic. An abdominal X-ray revealed five bullet-shaped magnets in the lower abdomen. Laboratory investigations were normal except for a mild leukocytosis. Surgery was performed with excision of seven entero-enteric fistulae and two intestinal anastomoses. DISCUSSION: Between 2003 and 2006, the Centers for Disease Control and Prevention reported 20 complicated cases of magnet ingestion in children aged 10 months to 11 years among whom 75% had bowel perforations, and 20% suffered from generalized peritonitis. Half that number of patients required emergency laparotomy. As a result, the Consumer Product Safety Commission (CPSC) issued the first warning announcing the hazards of high-powered magnets used in children's toys, which had been increasing exponentially. It is clear that the diagnosis of magnet ingestion is made commonly due to complications, such as peritonitis or death. However, the patient in this case was completely asymptomatic and had no complications. CONCLUSION: A high index of suspicion regarding complications is recommended in asymptomatic cases of suspected magnet ingestion. Avoid delaying intervention once a diagnosis has been made.

Multi-parametric MR imaging using apparent diffusion coefficient and fat fraction in quantification of bone marrow in pediatrics with Gaucher disease.

PURPOSE: To assess multi-parametric MR imaging with apparent diffusion coefficient (ADC) and fat fraction (FF) in detection and quantification of bone marrow involvement in pediatric patients with Gaucher disease. MATERIAL AND METHODS: This study was conducted upon 29 pediatric patients with Gaucher disease and 13 age and sex matched controls that underwent diffusion-weighted MR imaging and dual-echo gradient recalled echo imaging. The ADC and FF of the bone marrow were calculated. RESULTS: There was significant difference in ADC (P = 0.001) and FF (P = 0.001) of bone marrow between patients and controls. The cutoff ADC and FF differentiate patients from controls were 0.47 × 10-3 mm2/s and 0.36 with area under the curve of 0.947 and 0.885 and accuracy of 86.5% and 83.8% respectively. There was significant difference in ADC (P = 0.001) and FF (P = 0.001) between untreated (n = 17) and treated (n = 12) patients. The cutoff ADC and FF differentiate untreated from treated patients were 0.39 × 10-3 mm2/s and 0.27 with area under curve of 0.886 and 0.851 and accuracy of 88% and 84% respectively. CONCLUSION: Multi-parametric MR imaging using ADC and FF are quantitative imaging parameters that can be used for detection and quantification of vertebral bone marrow involvement in pediatric patients with Gaucher disease.

Assessment of the liver and spleen in children with Gaucher disease type I with diffusion-weighted MR imaging.

PURPOSE: To assess hepatic and splenic apparent diffusion coefficient (ADC) in children with Gaucher disease type I with diffusion-weighted MR imaging and to correlate hepatic and splenic ADC with parameters of disease severity. SUBJECTS AND METHODS: Prospective study was conducted upon 25 children (11 treated and 14 untreated) with Gaucher disease and 12 age and sex matched control children. They underwent diffusion-weighted MR imaging of abdomen. Hepatic and splenic ADC and volume were calculated. RESULTS: There was statistically difference in hepatic and splenic apparent diffusion coefficient (P=0.001) between patients and controls. The cutoff ADC of liver and spleen used to differentiate patients from controls were 0.47 and 0.39×10-3mm2 with area and curve of 0.947 and 0.886 respectively. There was significant difference in hepatic and splenic ADC between untreated and treated patients (P=0.003 and 0.001). Hepatic ADC correlated with splenic volume (r=-0.721), hepatic volume (r=-0.555) and chitotriosidase (r=-0.413). Splenic ADC correlated with splenic volume (r=-0.652), hepatic volume (r=-0.544) and chitotriosidase (r=-0.355). CONCLUSION: Hepatic and splenic ADC can detect hepatic and splenic infiltration in Gaucher disease and correlated with some parameters of disease severity.

Serum vitamin D level in Egyptian children with Familial Mediterranean fever.

BACKGROUND: The aim of the study is to measure plasma vitamin D levels in a group of Egyptian children with familial Mediterranean fever (FMF) compared to healthy children. METHODS: The study enrolled 52 children with FMF and 40 apparently healthy controls. Serum vitamin D level was measured by enzyme-linked immunosorbent assay. RESULTS: The mean serum vitamin D level was significantly lower in children with FMF than control group (12.3±3.4 and 21.2±3.5ng/mL, respectively, p<0.001). Vitamin D level was significantly lower in female patients than males (11.3±2.9, 13.2±3.6, respectively p=0.04). No statistically significant relations were detected between vitamin D level and different clinical, laboratory and genetic variables. CONCLUSION: Vitamin D levels were lower in Egyptian FMF children than healthy controls. There is a speculation that vitamin D deficiency in FMF patients may be related to inflammation. Further studies with larger number of patients before and after Vitamin D, therapy may be needed. Supplementation with high doses of vitamin D seems appropriate for children with FMF.

Effect of the number of applications of acetone-based adhesives on microtensile bond strength and the hybrid layer.

PURPOSE: The current study was carried out to evaluate the effect of doubling the adhesive layers of three acetone-based adhesives on the microtensile bond strength and ultra morphological characterization of the resin dentin interface using SEM. MATERIALS AND METHODS: A total of 27 caries-free human molars were used. Superficial flat dentin surfaces were obtained by wet grinding the buccal surfaces. Three adhesive systems Prime & Bond NT (G1), XENO IV (GII) and G BOND (GIII) were used according to three different protocols: (A) according to the manufacturer's instructions, (B) doubling the adhesive layers and light curing and (C): doubling the adhesive layers with intermediate curing between each layer. Resin composite buildups were made using TPH Spectrum resin composite on the bonded surfaces in 1 mm light cured increments for 40 seconds each. The bonded teeth were sectioned to obtain sticks for microtensile testing. The testing was conducted using a universal testing machine at a crosshead speed of 1 mm/minute. The microtensile bond strength means and standard deviations were calculated and the data were statistically analyzed using Two-way Analysis of Variance (ANOVA) and Tukey's post-hoc tests (p < or = 0.05). Ultra morphological characterization of the resin-dentin interface and representative fractured dentin specimens were examined using SEM. RESULTS: When applied according to the manufacturer's instructions, Prime & Bond NT (total etch adhesive) showed significantly high microtensile bond strength with a mean value +/- SD of 35 +/- 12.7 MPa followed by XENO IV (21.2 +/- 9.4 MPa), while G BOND presented a significantly lower mean value (10.9 +/- 2.9 MPa). Doubling the adhesive layers significantly reduced the mean strength of the total etch adhesive system; in contrast, it significantly increased the bond strength of both self-etch adhesives. The relatively thicker adhesive layer was seen with the total-etch adhesive when the application was doubled, while the hybrid layer appeared thicker with self-etch adhesives. CONCLUSIONS: Doubling the adhesive layer applications significantly improved the bond strength of the two self-etch adhesives (XENO IV and G BOND); however, it had a negative effect on the bond strength of the total-etch adhesive (Prime & Bond NT).

Systemic lupus erythematosus in Egyptian children.

The aim of the study was to study the characteristics of systemic lupus erythematosus (SLE) in the Egyptian population, comparing it to other populations. We retrospectively studied 207 patients with SLE diagnosed between 1990 and 2005. We obtained clinical features and laboratory data and analyzed them statistically. We studied 151 female and 56 male SLE patients. The female to male ratio was 2.7 to 1 and the mean age at presentation was 10 +/- 2.7 years (range 2-16). The mean disease duration was 6.47 +/- 3.74 years. At diagnosis, musculoskeletal, constitutional and mucocutaneous manifestations were the commonest features. During follow-up, the prevalence of nephritis (67%), hematological manifestations (44.9%), photosensitivity (44%), arthritis (39%), malar rash (38.2%), serositis (32.9%) and neuropsychiatric manifestations (24.25%) increased significantly. Those whose age of onset of the disease was

Doppler assessment of brachial artery flow as a measure of endothelial dysfunction in pediatric chronic renal failure.

Cardiovascular morbidity and mortality are highly prevalent among patients with chronic renal failure (CRF). Endothelial dysfunction is regarded as the initial reversible step in the development of atherosclerosis and has been demonstrated in all stages of renal failure. Non-invasive techniques to assess endothelial function have been recently developed and have been proven to predict future mortality in adults. We aimed to assess endothelial function in children with stage 4 chronic kidney disease (CKD 4) on conservative treatment, using a-non invasive, high-resolution, ultrasound Doppler study of the brachial artery flow, correlating it with other clinical and laboratory parameters. This study included 34 children with CKD 4 on conservative treatment who were compared with 30 healthy controls. Flow-mediated dilatation (FMD), nitroglycerin-mediated dilatation (NTG-MD) and FMD/NTG-MD ratio were estimated. FMD was abnormal (< 5%) in 24 patients (71%). FMD and FMD/NTG-MD ratio were significantly lower in patients than in controls (P = 0.001 and P = 0.01, respectively). FMD correlated positively with serum calcium and negatively with alkaline phosphatase. We concluded that endothelial dysfunction is present in children with CKD 4 on conservative treatment and may reflect increased atherogenic and thrombogenic properties of the endothelium, contributing to subsequent adverse cardiovascular outcome.