Conversion and retention of pteroylmonoglutamate by jejunum. The protruding gut is foreshortened, matted, thickened and covered with a peel. Education in Nazi Germany. Since risk of incarceration is high in children, repair should be undertaken shortly after diagnosis. Germany West Presse- und Informationsamt For example, Germany refused to share their formula for synthetic oil from coal until late in the war.
Office of Dietary Supplements Web site. Accessed October 20, Vitamin B 12 is a cofactor in two reactions: Therefore, a decrease in vitamin B 12 can result in higher levels of methylmalonic acid and homocysteine. If physicians are persistent about checking vitamin B 12 levels and, if necessary, methylmalonic acid levels, then serious deficiencies can often be prevented in patients.
If a vitamin B 12 deficiency is found, then replacement with oral or injection forms of vitamin B 12 may be necessary. As one of the organic building blocks necessary for all human cells, folate is needed by the body to make new cells such as neurons and red blood cells. Folate and its synthetic form, folic acid, are found in various foods, including fortified cereals, legumes, leafy vegetables, and other fruits and vegetables.
Folate absorption depends primarily on carrier transport mechanisms across the intestinal wall, pH level, and saturation points, with maximum folate absorption occurring at lower pH concentrations.
Processes that interfere with the intestinal wall, such as intestinal surgery, bacterial overgrowth, and celiac sprue, can reduce zinc absorption and cause zinc deficiency. Another essential nutrient for the human body is iron, a metallic element found in red meat and vegetables.
The recommended daily allowance of iron is about 8 mg to 18 mg daily, depending on age and sex. Although the precise mechanism of iron absorption is unclear, the duodenum and jejunum of the small intestine appear to play a primary role. Iron deficiency remains the most common known cause of anemia in addition to being the most common known nutritional deficiency among adults. As a nutrient involved in various enzymatic reactions, copper is a strong antioxidant essential to the human body.
It is found in meat, vegetables, legumes, and whole grains. Copper deficiencies may accompany iron deficiencies. Individuals with copper deficiency may develop progressive difficulty walking, increased muscle tone or spasticity, heart enlargement, skin changes, or neuropathy. Absorption of copper occurs primarily in the small intestine. There have been multiple reports 35 , 36 of calcium and vitamin D deficiency and hyperparathyroidism in patients who have had recent gastric bypass surgery.
Aggressive supplementation of calcium and vitamin D has been recommended around the time of the operation to combat these adverse effects. Calcium absorption and metabolism are carefully regulated by levels of calcium, vitamin D, and parathyroid hormone. Because these levels may be altered after bariatric surgery, careful monitoring is needed to maintain homeostasis. Good sources of calcium include dairy products such as cheese or milk.
Additional supplementation may be needed for these patients. At this time, consensus—including specific postoperative recommended daily allowance guidelines—is needed to ensure the proper postsurgical treatment for patients who have had bariatric surgery—especially gastric bypass surgery. To prevent surgical and other postoperative complications, one study 41 described the implementation of a multidisciplinary team—consisting of a primary care physician, dietician, gastroenterologist, and nursing staff—for patients undergoing bariatric surgery and the subsequent limited complications in patients.
Combined, multidisciplinary teams and consensus guidelines could help physicians provide better nutritional care for this patient population. Metabolic and bariatric surgery [fact sheet]. Accessed September 22, Spanakis E, Gragnoli C. Bariatric surgery, safety and type 2 diabetes [review] [published online ahead of print October 2, ].
Gastric banding or bypass? A systematic review comparing the two most popular bariatric procedures. National trends in use and outcome of laparoscopic adjustable gastric banding [published online ahead of print August 19, ]. Surg Obes Relat Dis. Seven cases of gastric perforation in Roux-en-Y gastric bypass patients: Nutritional deficiencies following bariatric surgery: Noncompliance with behavioral recommendations following bariatric surgery.
Prevalence of iron, folate, and vitamin B12 deficiency after laparoscopic Roux-en-Y gastric bypass [published online ahead of print January 23, ].
Dietary supplement fact sheet: Nutritional consequences of adjustable gastric banding and gastric bypass: Reversible peripheral neuropathy induced by vitamin B12 deficiency [in French] [published online ahead of print January 25, ].
Iverson D, McKenzie M. Neurologic complications of gastric bypass surgery for morbid obesity [letter]. Nutritional and metabolic complications of bariatric surgery [review]. Am J Med Sci. Oh R, Brown DL. Vitamin B12 deficiency [review].
Dietary intake pattern relates to plasma folate and homocysteine concentrations in the Framingham Heart Study. Accessed September 9, A new approach to total aganglionosis of the colon.
Surg Gynecol Obstet Cass DT, Myers N: Pediatr Surg Int 2: Total colonic aganglionosis with or without ileal involvement: A review of 27 cases. J Pediatr Surg Festen C, Severijner R, v. The absorptive function of colonic aganglionoic intestine: Are the Duhamel and Martin procedures rational? Ikeada K, Goto S: Total colonic aganglionosis with or without small bowel involvement: An analysis of patients. A new surgical approach to extensive aganglionosis. Further experience with the colonic patch graft procedure and long-term results.
Suction biopsy in Hirschsprung's disease. Arch Dis Child A possible cause of anastomotic failure following repair of intestinal atresia. Can J Surg Surgical management of Hirschsprung's disease involving the small intestine. Improvements in the management of total colonic aganglionosis.
Pediatr Surg Int 5: The importance of oral sodium replacement in ileostomy patients. Progr Pediatr Surg Diagnosis of congenital megacolon: J Pediatr Surg 7: Imperforate anus IA is a congenital anomaly in which the natural anal opening is absent.
Diagnosis of IA is usually made shortly after birth on routine physical examination. The incidence of IA is approximately 1 in live births and it is more common in males. Its etiology is unknown and it runs equally through all racial, cultural and socio-economic groups. IA is classified as either "high" or "low" depending on the termination of the distal rectum. When the rectum ends above the levator muscles the malformations are classified as high, and when the rectum ends below the levator muscles the malformations are classified as low.
High lesions are more frequent in males, low ones in females. Determination of the level of the lesion by abdominal x-ray or perineal ultrasound is critical for appropriate management. Children who have IA may also have other congenital anomalies.
Vertebral defects, Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal anomalies, and Limb anomalies. Repair of low IA is relatively simple and is usually treated with perineal anoplasty; however, repair of high IA is more complex. Patients are initially given a temporary colostomy and time is given to allow the child to grow. A pull-through operation is completed at a later date.
Independent of the level of the lesion, the goal of the surgery is the creation of adequate nerve and muscle structures around the rectum and anus to provide the child with the capacity for bowel control.
Male patients will benefit from perineal inspection to check for the presence of a fistula wait hours of life before deciding. During this time start antibiotherapy, decompress the GI tract, do a urinalysis to check for meconium cells, and an ultrasound of abdomen to identify urological associated anomalies. Perineal signs in low malformations that will NOT need a colostomy are: These infants can be managed with a perineal anoplasty during the neonatal period with an excellent prognosis.
Meconium in urine shows the pt has a fistula between the rectum and the urinary tract. Flat "bottom" or perineum lack of intergluteal fold , and absence of anal dimple indicates poor muscles and a rather high malformation needing a colostomy. Patients with no clinical signs at 24 hours of birth will need a invertogram or cross-table lateral film in prone position to decide rectal pouch position. Those cases with high defect are initially managed with a totally diverting colostomy.
Diverting the fecal stream reduces the chances of genito-urinary tract contamination and future damage. These infants require a colostomy before final corrective surgery. The colostomy can be done electively before discharge from the nursery while the GI tract is decompressed by dilatation of the fistulous tract. A single orifice is diagnostic of a persistent cloacal defect usually accompany with a small-looking genitalia. Cloacas are associated to distended vaginas hydrocolpos and urologic malformations.
This makes a sonogram of abdomen very important in the initial management of these babies for screening of obstructive uropathy hydronephrosis and hydroureter. Hydrocolpos can cause compressive obstruction of the bladder trigone and interfere with ureteral drainage.
Failure to gain weight and frequents episodes of urinary tract infections shows a poorly drained urologic system. A colostomy in cloacas is indicated. Radiological evaluation will be of help along with a diverting colostomy in this cases. Perineal fistulas can be managed with cutback without colostomy during the neonatal period. The most important prognostic characteristic is the severity of the IA. Patients with low IA have a good probability of having normal stool patterns. Patients with high IA report more problems such as fecal incontinence and constipation.
For patients who cannot maintain normal bowel function, the use of a special diet, underpants liners, enemas and drugs have ameliorated their lives. Long-term follow up with both qualitative and quantitative quality of life considerations of these patients is very important.
References 1- Chen CJ: The treatment of imperforate anus: J Pediatr Surg 34 Inheritance of familial congenital isolated anorectal malformations: Am J Med Genetics Do children with repaired low anorectal malformations have normal bowel function? J Pediatr Surg 32 6: Semin Pediatr Surg 4 1: Management of anorectal malformations during the newborn period. World J Surg 17 3: Posterior sagittal approach for the correction of anorectal malformations. Surgical treatment of high imperforate anus. World J Surg 9 2: J Pediatr Surg 17 5: Although intussusception can occur at any age, the greatest incidence occurs in infants between months of age.
Over half of the cases are in the first year of life. Frequently occurs after a recent upper respiratory infection, by Adenovirus type 3 that causes a reactive lymphoid hyperplasia that act as lead point of Peyer's patch. Meckel's diverticulum, polyps, Henoch's Schonlein purpura, hematoma, lymphoma, foreign bodies, and duplications. Most children have no lead point and it is felt that enlarged mesenteric nodes or swollen Peyer's patches may be the cause. The baby has intermittent periods of severe discomfort with screaming, stiffening and drawing up of the legs, followed by periods of rest.
Vomiting may occur and bloody, mucoid currant jelly stool may be passed. The baby may become dehydrated and appear acutely ill. Frequently, lethargy may be an early sign. To be successful, the barium must reflux into the terminal ileum. The surgeon should be notified before an attempt at barium reduction, and should be present at the time of study.
Recently the use of gas enema reduction has been successful in patients with: Ultrasonography can be used as a rapid sensitive screening procedure in the initial diagnosis of intussusception.
Previous adverse clinical features that precluded barium reduction can be replaced during gas reduction. Predictors of failure of reduction are: Air reduction pneumocolon is a very effective alternative method since it brings less radiation shorter flouroscopy time , less costs and less morbidity in cases of perforations.
Failure of hydrostatic reduction requires urgent operation through a right lower quadrant horizontal incision. The intussusception is reduced by pushing on the distal bowel like a tube of toothpaste rather than pulling the proximal bowel. Most cases are ileo-colic intussusception, and a few are jejuno-jejunal or ileo-ileal intussusception. The traditional method of diagnosing and managing ileo-colic intussusception is barium enema contrast reduction. In China where this is the most common surgical emergency in childhood, pneumatic reduction has been used for more than 25 years.
A recent tendency toward this approach is seen in recent years in Occident. Small bowel aeration is a sign of complete reduction. Gas enema reduction is very successful in patients with: The condition can occur in an isolated form either localized to colon or disseminated throughout the bowel , or associated to other diseases such as Hirschsprung's HD , neurofibromatosis, MEN type IIB, and anorectal malformations. Clinically two different types of isolated IND have been described: Type A shows symptoms of abdominal distension, enterocolitis, bloody stools, intestinal spasticity in imaging studies Ba Enema since birth, is less common and associated with hypoplasia of sympathetic nerves.
Type B is more frequent, symptoms are indistinguishable from that of HD, with chronic constipation, megacolon, and repeated episodes of bowel obstruction. Management depends on clinical situation; conservative for minor symptoms until neuronal maturation occurs around the 4th year of life, colostomy and resectional therapy for life threatening situations.
The most common congenital diaphragmatic hernia CDH is that which occurs through the postero-lateral defect of Bochdalek. It is caused by failure of the pleuroperitoneal membrane to develop adequately and close before the intestines returning to the abdomen at the tenth week of gestation. The intestines then enter the pleural cavity and cause poor lung development leading to pulmonary hypoplasia a reduced number of alveoli per area of lung tissue.
This defect is postero-lateral in the diaphragm and may vary in size. Stomach, liver or spleen may be partly in chest as well. The clinical presentation is that the newborn becomes rapidly cyanotic, acidotic, and has poor ventilation.
Major findings relate to the degree of pulmonary maldevelopment. Chest films will show intestines in the chest. Placement of a radiopaque nasogastric tube may show the tube coiled in the lower left chest. Higher risk factors are: Treatment consist of rapid intubation and ventilation with use of muscle relaxants, placement of a nasogastric tube to prevent gaseous distension of the intestines and preoperative stabilization of arterial blood gases and acid-base status.
Surgery can be undertaken when one of the following objectives are met: Operative management consist of abdominal approach, closure of hernia by primary repair or use of mesh, and correction of malrotation. Postoperative management is very difficult. Due to hypoplastic lungs, there is frequently pulmonary hypertension leading to right-to-left shunting and progressive hypoxemia, hypercarbia, and acidosis that worsens the pulmonary hypertension.
The use of chest tubes may cause overstretching of the already hypoplastic alveoli causing: Postoperatively, the infant should be kept paralyzed and ventilated and only very slowly weaned from the ventilator. The severity of pulmonary hypoplasia, both ipsilaterally and contralaterally, is the main determinant of outcome. ECMO extracorporeal membrane oxygenator has come to reduce somewhat the mortality of this condition. The mortality of CDH is directly related to the degree of lung hypoplasia associated.
Death is caused by persistent pulmonary hypertension and right ventricular failure. Prospective studies of prenatally diagnosed fetus prior to 25 wk.
This unsolved problem has prompted investigators to develop new treatment options such as preoperative stabilization, jet-frequency ventilation, and ECMO. Another area of development is intrauterine fetal surgical repair. To achieve success fetal surgery should: Intrauterine repair has meet with limited success due to herniation of the fetal liver into the chest through the defect. Disturbance of the umbilical circulation during or after liver reduction causes fetal death.
Positive-pressure ventilation after birth reduces the liver before the baby comes for surgical repair. Harrison USFC Fetal Treatment Center has devised separate fetal thoraco-abdominal incisions to deal with this problem "two-step dance" , reducing or amputating the left lateral segment of the liver.
Another less invasive approach is enlarging the hypoplastic lungs by reducing the normal egress of fetal lung fluid with controlled tracheal obstruction called PLUGS Plug Lung Until it Grows.
Infants and children will present with either respiratory or gastrointestinal symptoms such as: Occasionally the child is asymptomatic. A rise intrabdominal pressure by coughing or vomiting transmitted to any defect of the diaphragm makes visceral herniation more likely. Diagnosis is confirmed by chest or gastrointestinal contrast imaging.
Management consists of immediate surgery after preop stabilization. Most defects can be closed primarily through an abdominal approach. Chest-tube placement in the non-hypoplastic lung is of help. Surgical results are generally excellent. A few deaths have resulted from cardiovascular and respiratory compromise due to visceral herniation causing mediastinal and pulmonary compression.
First described in , Morgagni Hernias MH are rare congenital diaphragmatic defects close to the anterior midline between the costal and sternal origin of the diaphragm. Almost always asymptomatic, typically present in older children or adults with minimal gastrointestinal symptoms or as incidental finding during routine chest radiography mass or air-fluid levels.
Infants may develop respiratory symptoms tachypnea, dyspnea and cyanosis with distress. Cardiac tamponade due to protrusion into the pericardial cavity has been reported. US and CT-Scan can demonstrate the defect. Trans-abdominal subcostal approach is preferred with reduction of the defect and suturing of the diaphragm to undersurface of sternum and posterior rectus sheath.
Large defects with phrenic nerve displacement may need a thoracic approach. Results after surgery rely on associated conditions. Two types of esophageal hernia recognized are the hiatal and paraesophageal hernia. Diagnosis is made radiologically always and in a number of patients endoscopically. The hiatal hernia HH refers to herniation of the stomach to the chest through the esophageal hiatus.
The lower esophageal sphincter also moves. It can consist of a small transitory epiphrenic loculation minor up to an upside-down intrathoracic stomach major. HH generally develops due to a congenital, traumatic or iatrogenic factor. Most disappear by the age of two years, but all forms of HH can lead to peptic esophagitis from Gastroesophageal reflux.
Repair of HH is determined by the pathology of its associated reflux causing failure to thrive, esophagitis, stricture, respiratory symptoms or the presence of the stomach in the thoracic cavity.
In the paraesophageal hernia PH variety the stomach migrates to the chest and the lower esophageal sphincter stays in its normal anatomic position. PH is a frequent problem after antireflux operations in patients without posterior crural repair.
Small PH can be observed. With an increase in size or appearance of symptoms reflux, gastric obstruction, bleeding, infarction or perforation the PH should be repaired. The incidence of PH has increased with the advent of the laparoscopic fundoplication. A hernia is defined as a protrusion of a portion of an organ or tissue through an abnormal opening. For groin inguinal or femoral hernias, this protrusion is into a hernial sac.
Whether or not the mere presence of a hernial sac or processus vaginalis constitutes a hernia is debated. Inguinal hernias in children are almost exclusively indirect type. Those rare instances of direct inguinal hernia are caused by previous surgery and floor disruption.
An indirect inguinal hernia protrudes through the internal inguinal ring, within the cremaster fascia, extending down the spermatic cord for varying distances. The direct hernia protrudes through the posterior wall of the inguinal canal, i. The embryology of indirect inguinal hernia is as follows: During the third month of gestation, the processus vaginalis extends down toward the scrotum and follows the chorda gubernaculum that extends from the testicle or the retroperitoneum to the scrotum.
During the seventh month, the testicle descend into the scrotum, where the processus vaginalis forms a covering for the testicle and the serous sac in which it resides. At about the time of birth, the portion of the processus vaginalis between the testicle and the abdominal cavity obliterates, leaving a peritoneal cavity separate from the tunica vaginalis that surrounds the testicle. The typical patient with an inguinal hernia has an intermittent lump or bulge in the groin, scrotum, or labia noted at times of increased intra-abdominal pressure.
A communicating hydrocele is always associated with a hernia. This hydrocele fluctuates in size and is usually larger in ambulatory patients at the end of the day. If a loop of bowel becomes entrapped incarcerated in a hernia, the patient develops pain followed by signs of intestinal obstruction. If not reduced, compromised blood supply strangulation leads to perforation and peritonitis. Most incarcerated hernias in children can be reduced. Associated to these episodes of incarceration are chances of: Symptomatic hernia can complicate the clinical course of babies at NICU ill with hyaline membrane, sepsis, NEC and other conditions needing ventilatory support.
Repair should be undertaken before hospital discharge to avoid complications. Postconceptual age sum of intra- and extrauterine life has been cited as the factor having greatest impact on post-op complications.
These observation makes imperative that preemies with post conceptual age of less than 45 weeks be carefully monitored in-hospital for at least 24 hours after surgical repair of their hernias.
Outpatient repair is safer for those prematures above the 60 wk. The very low birth weight infant with symptomatic hernia can benefit from epidural anesthesia. At times, the indirect inguinal hernia will extend into the scrotum and can be reduced by external, gentle pressure. Occasionally, the hernia will present as a bulge in the soft tissue overlying the internal ring. It is sometimes difficult to demonstrate and the physician must rely on the patient's history of an intermittent bulge in the groin seen with crying, coughing or straining.
Elective herniorrhaphy at a near convenient time is treatment of choice. Since risk of incarceration is high in children, repair should be undertaken shortly after diagnosis. Simple high ligation of the sac is all that is required. Pediatric patients are allowed to return to full activity immediately after hernia repair. Bilateral exploration is done routinely by most experienced pediatric surgeons.
Recently the use of groin laparoscopy through the hernial sac permits visualization of the contralateral side. Testicular feminization syndrome TFS is a genetic form of male pseudohermaphroditism patient who is genetically 46 XY but has deficient masculinization of external genitalia caused by complete or partial resistance of end organs to the peripheral effects of androgens.
This androgenic insensitivity is caused by a mutation of the gene for androgenic receptor inherited as an X-linked recessive trait.
In the complete form the external genitalia appear to be female with a rudimentary vagina, absent uterus and ovaries. The incomplete form may represent undervirilized infertile men. This patients will never menstruate or bear children. Early gonadectomy is advised to: Vaginal reconstruction is planned when the patient wishes to be sexually active. These children develop into very normal appearing females that are sterile since no female organs are present.
A hydrocele is a collection of fluid in the space surrounding the testicle between the layers of the tunica vaginalis. Hydroceles can be scrotal, of the cord, abdominal, or a combination of the above. A hydrocele of the cord is the fluid-filled remnant of the processus vaginalis separated from the tunica vaginalis.
A communicating hydrocele is one that communicates with the peritoneal cavity by way of a narrow opening into a hernial sac. Hydroceles are common in infants. Some are associated with an inguinal hernia. They are often bilateral, and like hernias, are more common on the right than the left. Most hydroceles will resolved spontaneously by years of age. After this time, elective repair can be performed at any time. Operation is done through the groin and search made for an associated hernia.
Aspiration of a hydrocele should never be attempted. As a therapeutic measure it is ineffective, and as a diagnostic tool it is a catastrophe if a loop of bowel is entrapped. A possible exception to this is the postoperative recurrent hydrocele.
The undescended testis is a term we use to describe all instances in which the testis cannot be manually manipulated into the scrotum. The testes form from the medial portion of the urogenital ridge extending from the diaphragm into the pelvis. In arrested descent, they may be found from the kidneys to the internal inguinal ring.
Rapid descent through the internal inguinal ring commences at approximately week 28, the left testis preceding the right. Adequate amounts of male hormones are necessary for descent. The highest levels of male hormones in the maternal circulation have been demonstrated at week Thus, it appears that failure of descent may be related to inadequate male hormone levels or to failure of the end-organ to respond. The undescended testes may be found from the hilum of the kidney to the external inguinal ring.
The undescended testis found in 0. Testes that can be manually brought to the scrotum are retractile and need no further treatment. Parents should know the objectives, indications and limitations of an orchiopexy: To improve spermatogenesis producing an adequate number of spermatozoids surgery should be done before the age of two.
Electron microscopy has confirmed an arrest in spermatogenesis reduced number of spermatogonias and tubular diameter in undescended testis after the first two years of life.
Other reasons to pex are: The management is surgical; hormonal Human Chorionic Gonadotropin treatment has brought conflicting results except bilateral cases. Surgery is limited by the length of the testicular artery. Palpable testes have a better prognosis than non-palpable. Laparoscopy can be of help in non-palpable testis avoiding exploration of the absent testis. Viens, MS University of Toronto. An umbilical hernia is a small defect in the abdominal fascial wall in which fluid or abdominal contents protrude through the umbilical ring.
The presence of a bulge within the umbilicus is readily palpable and becomes more apparent when the infant cries or during defecation. The actual size of the umbilical hernia is measured by physical examination of the defect in the rectus abdominis muscle, and not by the size of the umbilical bulge.
The size of the fascial defect can vary from the width of a fingertip to several centimetres. Embryologically, the cause of an umbilical hernia is related to the incomplete contraction of the umbilical ring. The herniation of the umbilicus is a result of the growing alimentary tract that is unable to fit within the abdominal cavity.
Umbilical hernias are more prevalent in females than in males and are more often seen in patients with African heritage. The increased frequency of umbilical hernias has also been attributed to premature babies, twins and infants with long umbilical cords. There is also a frequent association with disorders of mucopolysaccharide metabolism, especially Hurler's Syndrome gargoylism.
Most umbilical hernias are asymptomatic; the decision to repair the umbilical hernia in the first years of life is largely cosmetic and is often performed because of parental request, not because of pain or dysfunction. In the past, some parents use to tape a coin over the umbilical bulge, however, manual compression does not have an effect on the fascial defect.
Treatment of umbilical hernia is observation. However, surgical repair is recommended if the hernia has not closed by the age of five. The incidence of incarceration trapped intestinal loop is rare, even in larger defects.
Females should especially have their umbilical hernia corrected before pregnancy because of the associated increased intra-abdominal pressure that could lead to complications. The procedure is simple and incidence of complication such as infection is extremely rare. The repair is usually done as outpatient surgery under general anesthetic. Inguinal and umbilical hernia repair in infants and children. Surg Clinics of North Am 73 3: Swenson's Pediatric Surgery - 5th edition.
The developing human - 4th edition. Philadelphia, WB Saunders, pp. Some observations on umbilical hernias in infants. The comparative incidence of umbilical hernias in colored and white infants. J Natl Med Assoc The three most common abdominal wall defect in newborns are umbilical hernia, gastroschisis and omphalocele.
Omphalocele is a milder form of primary abdominoschisis since during the embryonic folding process the outgrowth at the umbilical ring is insufficient shortage in apoptotic cell death. Defect may have liver, spleen, stomach, and bowel in the sac while the abdominal cavity remains underdeveloped in size. The sac is composed of chorium, Wharton's jelly and peritoneum.
The defect is centrally localized and measures cm in diameter. A small defect of less than 2 cm with bowel inside is referred as a hernia of the umbilical cord. Epigastric localized omphalocele are associated with sternal and intracardiac defects i.
Cardiac, neurogenic, genitourinary, skeletal and chromosomal changes and syndromes are the cornerstones of mortality. Cesarean section is warranted in large omphaloceles to avoid liver damage and dystocia. After initial stabilization management requires consideration of the size of defect, prematurity and associated anomalies. Primary closure with correction of the malrotation should be attempted whenever possible. Antibiotics and nutritional support are mandatory. Manage control centers around sepsis, respiratory status, liver and bowel dysfunction from increased intraabdominal pressure.
The protruding gut is foreshortened, matted, thickened and covered with a peel. The IA might be the result of pressure on the bowel from the edge of the defect pinching effect or an intrauterine vascular accident.
Rarely, the orifice may be extremely narrow leading to gangrene or complete midgut atresia. In either case the morbidity and mortality of the child is duplicated with the presence of an IA. Alternatives depend on the type of closure of the abdominal defect and the severity of the affected bowel.
With primary fascial closure and good-looking bowel primary anastomosis is justified. Angry looking dilated bowel prompts for proximal diversion, but the higher the enterostomy the greater the problems of fluid losses, electrolyte imbalances, skin excoriation, sepsis and malnutrition. Closure of the defect and resection with anastomosis two to four weeks later brings good results.
Success or failure is related to the length of remaining bowel more than the specific method used. Initially do an Apt test to determine if blood comes from fetal origin or maternal origin blood swallowed by the fetus. If this coagulation profile is normal the possibilities are either stress gastritis or ulcer disease. If the coagulation profile is abnormal then consider hematologic disease of the newborn and manage with vitamin K. The apt test is performed by mixing 1 part of vomitus with 5 part H2O, centrifuge the mixture and remove 5 ml pink.
If the coagulation profile is abnormal give Vit K for hematologic disorder of newborn. If it's normal do a rectal exam. A fissure could be the cause, if negative then consider either malrotation or Necrotizing enterocolitis. The stress includes prematurity, sepsis, hypoxia, hypothermia, and jaundice.
These babies frequently have umbilical artery, vein catheters, have received exchange transfusions or early feeds with hyperosmolar formulas. The intestinal mucosal cells are highly sensitive to ischemia and mucosal damage leads to bacterial invasion of the intestinal wall. Gas-forming organisms produce pneumatosis intestinalis air in the bowel wall readily seen on abdominal films. Full-thickness necrosis leads to perforation, free air and abscess formation.
These usually premature infants develop increased gastric residuals, abdominal distension, bloody stools, acidosis and dropping platelet count. The abdominal wall becomes reddened and edematous. There may be persistent masses and signs of peritonitis. Perforation leads to further hypoxia, acidosis and temperature instability. The acid-base status is monitored for worsening acidosis and hypoxia. The white blood cell count may be high, low or normal and is not generally of help. Serial abdominal films are obtained to look for evidence of free abdominal air, a worsening picture of pneumatosis intestinalis, or free portal air.
Therapy consist initially of stopping feeds, instituting nasogastric suctioning and beginning broad-spectrum antibiotics ampicillin and gentamycin. Persistent or worsening clinical condition and sepsis or free air on abdominal films require urgent surgical intervention.
Attempts to preserve as much viable bowel as possible are mandatory to prevent resultant short gut syndrome. Complicated NEC is the most common neonatal surgical emergency of modern times, has diverse etiologies, significant mortality and affects mostly premature babies. Consist of a right lower quadrant incision and placement of a drainage penrose or catheter under local anesthesia with subsequent irrigation performed bedside at the NICU.
Initially used as a temporizing measure before formal laparotomy, some patient went to improvement without the need for further surgery almost one-third.
They either had an immature fetal type healing process or a focal perforation not associated to NEC? Some suggestion made are: PPD should be an adjunct to preop stabilization, before placing drain be sure pt has NEC by X-rays, persistent metabolic acidosis means uncontrolled peritoneal sepsis, do not place drain in pts with inflammatory mass or rapid development of intraperitoneal fluid, the longer the drainage the higher the need for laparotomy.
In the initial evaluation a history should be obtained for bleeding disorders, skin lesions, and aspirin or steroid ingestion. The physical exam for presence of enlarged liver, spleen, masses, ascites, or evidence of trauma or portal hypertension. Labs such as bleeding studies and endoscopy, contrast studies if bleeding stops. Common causes of Upper GI bleeding are: Esophagus a Varices- usually presents as severe upper gastrointestinal bleeding in a year old who has previously been healthy except for problems in the neonatal period.
This is a result of extrahepatic portal obstruction portal vein thrombosis most commonly , with resulting varices. The bleeding may occur after a period of upper respiratory symptoms and coughing. Management is initially conservative with sedation and bedrest; surgery ir rarely needed.
Treatment consist of antacids, frequent small feeds, occasionally medications and if not rapidly improved, then surgical intervention with a fundoplication of the stomach. This was thought to be uncommon in children because it was not looked for by endoscopy.
It probably occurs more often than previously thought. Treatment initially is conservative and, if persistent, oversewing of the tear through an incision in the stomach will be successful. They bleed when there is ectopic gastric mucosa present. Total excision is curative. Occasionally requires surgical intervention with local repair or ligation of hepatic vessels. Anal fissure is the most common cause of rectal bleeding in the first two years of life.
Outstretching of the anal mucocutaneous junction caused by passage of large hard stools during defecation produces a superficial tear of the mucosa in the posterior midline. Pain with the next bowel movement leads to constipation, hardened stools that continue to produce cyclic problems.
Large fissures with surrounding bruising should warn against child abuse. Crohn's disease and leukemic infiltration are other conditions to rule-out. The diagnosis is made after inspection of the anal canal. Chronic fissures are associated with hypertrophy of the anal papilla or a distal skin tag. Management is directed toward the associated constipation with stool softeners and anal dilatations, warm perineal baths to relax the internal muscle spasm, and topical analgesics for pain control.
If medical therapy fails excision of the fissure with lateral sphincterotomy is performed. Meckel's diverticulum MD , the pathologic structure resulting from persistence of the embryonic vitelline duct yolk stalk , is the most prevalent congenital anomaly of the GI tract. MD can be the cause of: Diagnosis depends on clinical presentation. Rectal bleeding from MD is painless, minimal, recurrent, and can be identified using 99mTc- pertechnetate scan; contrasts studies are unreliable.
Persistent bleeding requires arteriography or laparotomy if the scan is negative. Obstruction secondary to intussusception, herniation or volvulus presents with findings of fulminant, acute small bowel obstruction, is diagnosed by clinical findings and contrast enema studies. The MD is seldom diagnosed preop.
Diverticulitis or perforation is clinically indistinguishable from appendicitis. Mucosal polyps or fecal umbilical discharge can be caused by MD. Overall, complications of Meckel's are managed by simple diverticulectomy or resection with anastomosis.
Laparoscopy can confirm the diagnosis and allow resection of symptomatic cases. Removal of asymptomatic Meckel's identified incidentally should be considered if upon palpation there is questionable heterotopic gastric or pancreatic mucosa thick and firm consistency present. Histology features a cluster of mucoid lobes surrounded by flattened mucussecreting glandular cells mucous retention polyp , no malignant potential.
Commonly seen in children age with a peak at age As a rule only one polyp is present, but occasionally there are two or three almost always confined to the rectal area within the reach of the finger. Most common complaint is bright painless rectal bleeding. Occasionally the polyp may prolapse through the rectum.
Diagnosis is by barium enema, rectal exam, or endoscopy. Removal by endoscopy is the treatment of choice. Rarely colotomy and excision are required. Wilms tumor WT is the most common intra-abdominal malignant tumor in children affecting more than children annually in the USA. It has a peak incidence at 3.
WT present as a large abdominal or flank mass with abdominal pain, asymptomatic hematuria, and occasionally fever. Other presentations include malaise, weight loss, anemia, left varicocele obstructed left renal vein , and hypertension.
Initial evaluation consists of: The presence of a solid, intrarenal mass causing intrinsic distortion of the calyceal collecting system is virtually diagnostic of Wilms tumor.
Doppler sonography of the renal vein and inferior vena cava can exclude venous tumor involvement. Metastasis occurs most commonly to lungs and occasionally to liver. Operation is both for treatment and staging to determine further therapy.
Following NWTSG recommendation's primary nephrectomy is done for all but the largest unilateral tumors and further adjuvant therapy is based on the surgical and pathological findings. Important surgical caveats consist of using a generous transverse incision, performing a radical nephrectomy, exploring the contralateral kidney, avoiding tumor spillage, and sampling suspicious lymph nodes.
Nodes are biopsied to determine extent of disease. Stage I- tumor limited to kidney and completely resected. Stage II- tumor extends beyond the kidney but is completely excised. Stage III- residual non-hematogenous tumor confined to the abdomen. Stage IV- hematogenous metastasis. Stage V- bilateral tumors.
Further treatment with chemotherapy or radiotherapy depends on staging and histology favorable vs. Non-favorable histologic characteristics are: Prognosis is poor for those children with lymph nodes, lung and liver metastasis. They tend to occur in younger patients. Routine abdominal ultrasound screening every six months up to the age of eight years is recommended for children at high risk for developing WT such as the above-mentioned syndromes.
It was originally thought that WT developed after the two-hit mutational model developed for retinoblastoma: When the first mutation occurs before the union the sperm and egg constitutional or germline mutation the tumor is heritable and individuals are at risk for multiple tumors.
Nonhereditary WT develops as the result of two-postzygotic mutations somatic in a single cell. The two-event hypothesis predicts that susceptible individuals such as familial cases, those with multifocal disease and those with a congenital anomaly have a lower median age at diagnosis than sporadic cases. It is now believed that several genes' mutations are involved in the overall WT pathogenesis. Loss of whole portions of a chromosome is called loss of heterozygosity LOH , a mechanism believed to inactivate a tumor-suppressor gene.
Children with the WAGR association shows a deletion in the short arm of chromosome 11 band 13 11p13 but a normal 11p15 region. Up to a third of sporadic WT have changes in the distal part of chromosome 11, a region that includes band p The region of the deletion has been named the WT1 gene, a tumor suppressor gene that also forms a complex with another known tumor-suppressor, p WT1 gene express a regulated transcription factor of the zinc-finger family proteins restricted to the genitourinary system, spleen, dorsal mesentery of the intestines, muscles, central nervous system CNS and mesothelium.
The important association of WT1 mutation and WAGR syndrome with intralobar nephrogenic rests immediately suggest that WT1 expression be necessary for the normal differentiation of nephroblasts. Inactivation of WT1 only affects organs that express this gene such as the kidney and specific cells of the gonads Sertoli cells of the testis and granulosa cells of the ovary.