Postoperative Necrotizing Enterocolitis Following Inguinal Hernia Repair in an Infant: Case Report and Review of the Literature.

Necrotizing enterocolitis (NEC) following postoperative stress is a rare but life-threatening condition in infants. We report a 3-month-old infant who underwent bilateral inguinal hernia repair and developed NEC. This is the first reported case of an infant developing recurrent NEC with stricture formation after herniotomy. Timely recognition and management are vital due to potentially high mortality rates in severe cases. High index of suspicion is crucial for accurate diagnosis and appropriate management.

Pediatric and adolescent gynecology: Treatment perspectives in minimally invasive surgery.

Minimally invasive surgery (MIS) is widely utilized across multiple surgical disciplines, including gynecology. To date, laparoscopy is considered a common surgical modality in children and adolescents to treat gynecological conditions. Robotic surgical devices were developed to circumvent the limitations of laparoscopy and have expanded the surgical armamentarium with better magnification, dexterity enhanced articulating instruments with 5-7 degrees of freedom, and ability to scale motion thus eliminating physiologic tremor. There are well-documented advantages of MIS over laparotomy, including decreased post-operative pain, shorter recovery times, and better cosmetic results. Indications for MIS in pediatric gynecology are reported in this review and technical considerations are described to highlight new treatment perspectives in children and adolescents, which have already been described in the literature regarding adult patients.

Recurrent supraventricular tachycardia and necrotizing enterocolitis: A causative role or a simple association? A case report and literature review.

Insufficiency in mesenteric flow is a risk factor for the development of necrotizing enterocolitis (NEC). Recurrent episodes of supraventricular tachycardia (SVT) can lead to gut ischemia, with subsequent reperfusion injury. We present a term infant who developed NEC at 37 days of life after refractory SVT and reverted to sinus rhythm on day 23 and 25 of life. Resected stenotic ileum and transverse colon demonstrated inflammation with ischemia. This is the first case of NEC following SVT in a term infant without a congenital morphologic abnormality. In view of the temporal sequence of events, and in the absence of other risk factors for NEC, the SVT and NEC were likely causally related.

Spleen size evaluation in children: Time to define splenomegaly for pediatric surgeons and pediatricians.

BACKGROUND: We determined the range of normal spleen dimensions evaluated by ultrasonography (US) in children according to sex and age and the relationship between splenic measurements, auxological data and body proportions, in order to define splenomegaly parameters in support of the surgical mini-invasive approaches in pediatrics. METHODS: We prospectively examined 317 caucasian children of both sexes. The patients were divided into three groups: 0-3 years; 4-10 years; 11-18 years. Sex, weight, height/length, body mass index (BMI), waist circumference and xipho-pubic distance were determined for each child. US spleen evaluation included longitudinal/antero-posterior/transverse diameters, transverse area and volume. Spleen volume/abdominal volume, longitudinal spleen diameter/longitudinal left kidney diameter and longitudinal spleen diameter/xipho-pubic distance ratios were also calculated. RESULTS: For caucasian subjects, in different age groups spleen volume, transverse area and diameter increased while the spleen/abdominal volume ratio decreased significantly (p<0.001). A significant (p<0.001) decrease in longitudinal spleen diameter/xipho-pubic distance ratio was noted between the 0-3 years group and both 4-10 and 11-18 years group. Age and auxological data, except BMI, showed a high correlation with spleen dimension (r≥0.8). CONCLUSIONS: The current concept of splenomegaly is not applicable in pediatric surgery. A dedicated classification of splenomegaly is needed for children and would improve the safety and feasibility of treatment.

Isolated Ileal Perforation in Infancy: A Lethal Initial Presentation of Hirschsprung's Disease.

Arare case of ileal perforation, as a fatal initial presentation of total colonic aganglionosis (TCA) in infancy is reported. A 10-week-old boy, was brought to the emergency department with symptoms of complicated intestinal obstruction. He looked ill, was lethargic, markedly dehydrated and had a severely distended abdomen. An abdominal X-ray revealed multiple air fluid levels seen in a distended small intestine. During exploratory laparotomy the ileum was massively dilated with distal segment perforation. Ileal perforation repair was performed. A totally collapsed microcolon was identified. Biopsies were taken from the high rectum, sigmoid and hepatic flexure. Appendectomy and ileostomy were performed. All biopsies, as well as the appendix, showed absence of ganglion cells. Despite this procedure the patient progressively deteriorated and later died due to sepsis. Ileal perforation in infants is a rare, but potentially fatal initial presentation of TCA. Early detection is essential to prevent life-threatening complications.

Longstanding Presence of a Vaginal Foreign Body (Battery): Severe Stenosis in a 13-Year-Old Girl.

BACKGROUND: In recurrent/unresponsive vaginal discharge a vaginal foreign body should be considered. CASE: We present the case of a 13-year-old girl who suffered from a foul-smelling dark brownish and malodorous vaginal discharge since the age of 7 years. When the girl was 11 years old, a foreign body was discovered on ultrasound imaging; removal by colposcopy failed, because of the complete battery inclusion in the posterior vaginal wall. Two years later, at our institution, radiological images yielded the general position of the foreign body; colposcopy showed complete vaginal stenosis. Forced dilatation allowed surgical removal of the battery which was embedded in the vaginal fundal wall. SUMMARY AND CONCLUSION: Early removal of foreign objects prevents ulceration, necrosis, stenosis, perforations, and late serious sequelae such as pelvic inflammation and infertility.

Bruises Around the Umbilicus in an Infant.

The most common causes of umbilical discharge in infancy are infection and umbilical granuloma that may be treated by antibiotics and topical application of silver nitrate subsequently. If the umbilical discharge persists or if there is any abnormal discoloration around the umbilicus, it is important to investigate for underlying congenital abnormality that may be cured by surgical intervention. Unusual presentation of omphalomesenteric duct cyst has been reported in literature. We report, for the first time as far as our search is concerned, a case of a 16-month-old infant who presented initially with persistent umbilical discharge and finally with bruising around the umbilicus in keeping with Cullen's sign. A diagnosis of omphalomesenteric duct cyst containing pancreatic tissue was made on histopathological examination. This case emphasizes that, a persistent umbilical discharge and or discoloration around the umbilicus should be further investigated and an omphalomesenteric duct cyst can present as Cullen's sign.

Five millimetre-instruments in paediatric robotic surgery: Advantages and shortcomings.

PURPOSE: The study was designed to assess the utility and controversies surrounding the usage of 5-mm instruments in paediatric robotic surgery. Adequate, delicate instruments for surgery in very narrow spaces are still lacking. MATERIAL AND METHODS: Thirty children underwent elective abdominal robotic surgery. Working sites, assembly and operative time, hospital stay, advantages, complications and shortcomings are reported. RESULTS: Interventions were performed in the following anatomical sites: 11 upper abdominal, nine pelvic, ten renal procedures. The majority of procedures required two operative trocars. A 2-3 mm accessory port was necessary for operations in the renal area and upper abdomen. The ports had to be placed at least 3 cm from the costal margins and superior iliac spines and at an angle of at least 130° with respect to the camera trocar. This configuration allowed intra-corporal knotting, vessel ligation and dissection with instruments in the inverted position. Operative times and hospital stays were similar to those reported for 8 mm-instruments. CONCLUSION: The use of 5-mm instruments was advantageous in renal and pelvic sites. The benefits in upper abdominal surgery need further evaluation, particularly in patients weighing <10 kg. Smaller sized instruments with high endowrist dexterity would resolve the problems encountered in paediatric robotic-assisted surgery using 5-mm instruments.

Metabolomic profile of amniotic fluid to evaluate lung maturity: the diaphragmatic hernia lamb model.

BACKGROUND: Tracheal occlusion (TO) stimulates lung growth in fetuses affected with congenital diaphragmatic hernia (CDH) although the processes involved in lung maturation still remain unknown. The objective of this study was to evaluate the metabolomic profile of amniotic fluid (AF) following TO in fetal lamb model in order to obtain an indirect view of mechanisms involved in pulmonary reversal hypoplasia and biochemical maturity in response to fetal TO. METHODS: Liquid Chromatography Mass Spectrometry was performed on lamb AF samples at: age I (70 days' gestation); age II (102 days' gestation); age III (136 days' gestation). CDH was induced at age I and TO at age II. RESULTS: Betaine, choline, creatinine were found significantly increased during gestation in the control group. The CDH group showed choline (p =0.007) and creatinine (p =0.004) decreases during pregnancy. In the TO group choline and creatinine profiles were restored. CONCLUSIONS: Alveolar tissue and fetal global growth ameliorated after TO. Metabolomics provided useful information on biochemical details during lung maturation. Metabolomic profiling would help to identify the best time to perform TO, in order to increase survival of CDH affected patients.

Malnutrition in pregnancy following bariatric surgery: three clinical cases of fetal neural defects.

OBJECTIVE: Bariatric surgery results in decreased food intake and a variable degree of malabsorption. Without adequate supplementation, the most common complications of this surgery are nutritional disorders. Pregnancy following surgery for obesity is a particular condition requiring strict monitoring of nutrient intake necessary for fetal development and a favourable neonatal prognosis. PATIENTS: Malnutrition in pregnancy and congenital neural malformations are reported in three women who had previously undergone bariatric surgery (1, 5 and 18 years before pregnancy, respectively). Two patients underwent the Roux en Y bypass and one bilio-pancreatic diversion with gastroplasty. None of the three received pre-conceptional nutritional counselling. Patients 1 and 2 did not undergo postoperative nutritional surveillance; nutrient supplementation was started at 22 and 20 weeks gestation, respectively. In patient 3, supplementation was stopped at six weeks gestation. RESULTS: Newborns 1 and 2 presented with dorsal myelomeningocele and ventricular dilation. Both underwent surgery and a ventriculo-peritoneal shunt was inserted in the first month of life. Newborn 3 had microcephaly, bilateral microphthalmia and sensorineural deafness. CONCLUSIONS: Diet and nutritional status, before and during pregnancy, play an important role in the early processes of fetal development and neonatal outcome. Women of childbearing age who have had bariatric surgery, should be encouraged to follow a well-balanced diet as part of a weight management strategy. They should be advised to take recommended maternal supplements.

Robotic-assisted surgery approach in a biliary rhabdomyosarcoma misdiagnosed as choledochal cyst.

Rhabdomyosarcoma is a soft tissue malignant tumor affecting 1% of children from 0 to 14 years. Preoperative imaging may not always be diagnostic for hepatobiliary rhabdomyosarcoma and differential diagnosis with choledochal cyst (CC) could be difficult. We report a case of 2-years-old girl with a strange CC pattern of presentation. A grapelike lesion involving the choledochal and biliary ducts was easily and completely resected by robotic assisted surgery. Since no previous reports were available about oncologic safety of robotic approach, the porto-enterostomy was performed in open surgery. On histologic examination, the specimen revealed a botryoidembryonal rhabdomyosarcoma affecting both the common bile duct and the common hepatic duct. One year postoperatively the child is safe of tumor relapse. Robotic approach seems to be safe and advantageous to obtain a radical excision of the tumor at the porta hepatis, even in case of misdiagnosed malignant lesion mimicking a CC.

Pediatric robotic pyeloplasty in patients weighing less than 10 kg initial experience.

AIM: To report the feasibility and safety of a robotic-assisted laparoscopic pyeloplasty (RALP) in patients weighing <10 kg. MATERIALS AND METHODS: Three patients weighing between 5 and 8 kg who were affected by severe congenital ureteropelvic junction obstruction, including a child with solitary kidney, were subjected to RALP. Three trocars were placed; sutures and pyeloplasty remodeling were performed with interrupted stitches. A double J stent was inserted through a 2-mm transparietal angiocatheter to protect the pyelic suture. RESULTS: The procedures were all completed within 90 minutes, the "docking" time requiring 20 minutes. The patients were discharged on postoperative day 2, without any complications. CONCLUSIONS: Comprehensive assessment of pyelic suture in a very narrow field with 2 operative instruments is feasible and safe. Robotic pyeloplasty provides all the advantages of mini-invasive surgery with the added advantage of higher magnification and excellent surgical navigation in very small spaces and on fragile infant tissues.

Chemotherapy and surgical approach with repeated endovascular embolizations: safe interdisciplinary treatment for kasabach-merritt syndrome in a small baby.

INTRODUCTION: Kasabach-Merritt syndrome (KMS) is a life-threatening disease. We describe a combined medical and multistep, endovascular embolization that was successfully performed with surgery. CASE REPORT: A 40-day-old female baby was referred because of an infiltrating pelvic mass. Blood tests showed severe anemia and thrombocytopenia with consumptive coagulopathy. The clinical aspect was pathognomonic for KMS. Administration of steroids and chemotherapy were started and coagulation parameters were normalized with tumor volume regression. Three months later, elective surgical treatment became possible, and dissection and ligature of the left internal iliac artery and sacral artery were performed. The main arterial supply of the mass was embolized with Spongostan®. Two months later, a second hybrid approach was adopted for embolization of the main vascular supply of the tumor occupying the left thigh. Via a third contralateral hybrid femoral approach and under fluoroscopy, selective catheterization of the left profunda femoris artery was performed. The arterial feeder of the tumor, localized at the left gluteus, was seen on a CT scan 2 months later and was embolized with Onyx-18. CONCLUSION: Chemotherapy and a tailored hybrid approach comprising repeated endovascular embolizations seem to be a safe treatment in cases of unresectable and life-threatening tumors in small babies.

Central precocious puberty and granulosa cell ovarian tumor in an 8-year old female.

Ovarian tumors associated with hormonal changes of the peripheral iso-sexual precocious puberty are of common presentation. We describe here a rare case of juvenile granulosa cell tumor in a female with central precocious puberty (CPP). An 8-year old girl with CPP presented with vaginal bleeding four months after the diagnosis and before starting treatment with gonadotropin-releasing hormone (GnRH)-analogs. Suppression of basal follicle-stimulating hormone (FSH) level, elevation of serum estradiol, progesterone and Cancer Antigen-125 were documented. Abdominal ultrasound examination (US) and magnetic resonance imaging showed a pelvic mass affecting the left ovary. A left salpingo-oophorectomy was performed and the mass was totally resected. Juvenile granulosa cell ovarian tumor was diagnosed. One month post surgery, estradiol and progesterone decreased to values of the first evaluation and FSH increased; Cancer Antigen-125 resulted normal while ultrasound pelvic examination showed absence of pelvic masses. In our patient, the tumor had grown very quickly since hormonal data demonstrated a CPP without any evidence of ovarian mass on US only four months before diagnosis. The overstimulation of the FSH or aberrant activation of FSH receptors may have contributed to the development of the mass.

Hunger hormone profile monitoring after gastroplication in an adolescent.

INTRODUCTION: Gut and adipose tissue hormones play an important role in energy balance control, particularly through the regulation at either short- or long-term food intake after bariatric surgery. CASE: A 15-year-old obese female (BMI 42.2) who was unresponsive to medical treatment underwent gastroplication. Hunger hormone levels (leptin, ghrelin and insulin), weight loss and eating behavior were monitored at 3, 6 and 12 months after surgery. RESULTS: Weight loss was obtained, progressively achieving a loss of 45.6 kg 1 year after surgery. A strong reduction in insulin concentration and insulin resistance was documented. At 3 months after the operation, a surprising leptin level drop was observed. During the following months a progressive increase in leptin levels and leptin/kg of fat mass were documented. Fasting ghrelin levels increased in the first 3 months, then fell over the next 6 months. Up to 6 months after gastroplication, we observed a less marked drop in plasma ghrelin after meal ingestion, while the values after 1 year showed a substantial fall in the postprandial period despite a further fasting ghrelin increased level. Early achievement of satiety was found. CONCLUSION: Hunger hormones level changes seem to be involved in weight loss and eating behavior after gastroplication in adolescents.

Intrascrotal lipoblastoma in a ten year old: case report and review of literature.

Lipoblastoma is a rare benign soft tissue tumor encountered almost exclusively in infancy and early childhood. The location of tumors varies, but most occur in the extremities, trunk, head and neck. Less frequently, lipoblastomas have been reported in the mediastinum, the retroperitoneum and the inguinal region. Only 7 cases of lipoblastoma in the scrotum have been reported so far in the English literature, with none of the patients older than 8. We report an intrascrotal lipoblastoma in a 10 year-old boy. The differential diagnosis is discussed with reference to the literature.

Portal Hypertension in Childhood Bilateral Wilms' Tumor Survivor: An Excellent Indication for TIPS.

Introduction. Increased pressure in portal venous system is relatively a rare complication after chemoradiotherapy for Wilms' tumor (WT). In paediatric population, feasibility and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in portal hypertension nonresponsive to medical or endoscopic treatment have been recently advocated. We report a case of TIPS positioning in a 15-year-old girl with portal hypertension as a long-term sequel of multimodality therapy in bilateral WT. Case Report. Two-year-old girl was diagnosed for bilateral WT. Right nephrectomy with left heminephrectomy and chemoradiotherapy were performed. At 7 years of age, the first gastrointestinal bleeding appeared, followed by another episode two years later, both were treated successfully with beta-blockers. At 15 years of age, severe unresponsive life-threatening gastroesophageal bleeding without hepatosplenomegaly was managed by TIPS. Reduction of the portosystemic pressure gradient was obtained. Conclusion. TIPS positioning for portal hypertension in long-term tumors' sequel is feasible and could be considered as an additional indication in paediatric patients.

Isolated colon ischemia with norovirus infection in preterm babies: a case series.

INTRODUCTION: Norovirus infection with necrotizing enterocolitis has so far been reported as a specific tropism of the small bowel in premature newborns. CASE PRESENTATION: Three cases of premature newborns presenting with extensive isolated colonic ischemia due to norovirus infection are reported.Patient 1 was a Caucasian girl with a gestational age of 29+2 weeks. She had sudden onset of abdominal distension on the 30th day of life. Radiological signs of colonic pneumatosis were present 48 hours before perforation and stool analysis was positive for norovirus. On the 34th day, free air was detected on plain abdominal X-ray. At laparotomy, stenosis, necrosis and perforations involved the whole colon. The patient underwent ileostomy. A large colon resection and ileosigmoid anastomosis were done 3 months later.Patient 2 was a Caucasian boy with a gestational age of 28+3 weeks. On the 19th day, bloody stools with abdominal distension appeared. Stool analysis resulted positive for norovirus. A plain abdominal X-ray showed distended bowel loops. Antibiotic treatment was started. On the 32nd day due to the progressive deterioration of clinical conditions and the appearance of colic pneumatosis, a laparotomy was performed. Severe damage of the transverse colon and multiple areas of necrosis were found. Terminal ileostomy was performed. Six months later surgery consisted of mid-transverse colon resection as far as the splenic flexure, colocolic anastomosis and closure of ileostomy.Patient 3 was a Caucasian boy with a gestational age of 30 weeks. On the 44th day bloody-mucous stools appeared and stool analysis was positive for norovirus infection. Even with institution of antibiotic therapy clinical abdominal radiologic signs of colonic pneumatosis of the upper right quadrant were found. At the 70th day an explorative laparotomy showed dilated bowel loops and stenotic right colon and ileostomy was mandatory. Partial colectomy was later necessary and ileocolic anastomosis was performed. CONCLUSION: We hypothesize that norovirus infection may be responsible for severe, distinctive colonic lesions, even in premature newborn infants.

Robotic assisted surgery in pediatric gynecology: promising innovation in mini invasive surgical procedures.

STUDY OBJECTIVE: Robotic assisted surgery is not yet widely applied in the pediatric field. We report our initial experience regarding the feasibility, safety, benefits, and limitations of robot-assisted surgery in pediatric gynecological patients. DESIGN: Descriptive, retrospective report of experience with pediatric gynecological patients over a period of 12 months. SETTING: Department of Pediatric Surgery, IRCCS Policlinico San Matteo Foundation. PARTICIPANTS: Children and adolescents, with a surgical diagnosis of ovarian and/or tubal lesions. MAIN OUTCOME MEASURES: Robot assembly time and operative time, days of hospitalization, time to cessation of pain medication, complication rate, conversion rate to laparoscopic procedure and trocar insertion strategy. RESULTS: Six children and adolescents (2.4-15 yrs), weighing 12-55 kg, underwent robotic assisted surgery for adnexal pathologies: 2 for ovarian cystectomy, 2 for oophorectomy, 1 for right oophorectomy and left salpingo-oophorectomy for gonadal disgenesis, 1 for exploration for suspected pelvic malformation. Mean operative time was 117.5 ± 34.9 minutes. Conversion to laparatomy was not necessary in any of the cases. No intra- or postoperative complications occurred. CONCLUSION: Initial results indicate that robotic assisted surgery is safely applicable in the pediatric gynecological population, although it is still premature to conclude that it provides better clinical outcomes than traditional laparoscopic surgery. Randomized, prospective, comparative studies will help characterize the advantages and disadvantages of this new technology in pediatric patients.