PSU Volume 58 NO 01 JANUARY 2022

Completion Thyroidectomy

Thyroid nodules in children are managed based exclusively on the results of fine-needle aspiration (FNA) biopsy. Benign FNA results can be observed depending on the size, complexity and symptomatology of the thyroid nodule. FNA results with positive pathology for papillary carcinoma is best managed with total thyroidectomy with central lymph node dissection. With results of FNA reported as indeterminate, suspicious, insufficient or a follicular neoplasm, removal of the affected lobe (hemithyroidectomy) might be performed. During removal of the affected lobe, the contralateral neck and remaining lobe should not be explored or violated.  In cases in which a hemithyroidectomy is performed and the final pathology is reported as a well-differentiated papillary or follicular thyroid cancer, a completion thyroidectomy is performed. Completion thyroidectomy reduces locoregional recurrence, distant metastasis as well as low-risk carcinoma. The overall need for completion thyroidectomy in the era of FNA is generally less than 5-10% after lobectomy. The current major indications for completion thyroidectomy are: gross extrathyroidal extension on the ipsilateral side, gross residual disease on the esophagus, recurrent laryngeal nerve or the tracheal wall, major vascular or capsular invasion and poorly differentiated carcinoma or aggressive Hurthle cell carcinoma. Whenever a completion thyroidectomy is to be performed, the surgeon should study the gland previously removed to determine the status of the parathyroid glands that might be removed with the specimen. The rates of both temporary and permanent hypoparathyroidism are considerably higher in patients in whom the parathyroid glands were removed with the specimen. At the tine of completion thyroidectomy when there is already a parathyroid gland in the initial hemithyroidectomy specimen, the surgeon must make every effort to identify and preserve both parathyroid and in the event of a suspected devascularization, the gland should be autotransplanted. Hemithyroidectomy followed by completion thyroidectomy does not appear to be associated with an increase operative risk of hypocalcemia or recurrent laryngeal nerve injury. The lower rate of temporary hypoparathyroidism and hypocalcemia seen in the completion thyroidectomy group can be attributed to the fact that the interval between operations allowed for recovery of any reversible injury caused at the initial hemithyroidectomy. Devascularized parathyroid glands require approximately four weeks to return to full function. Compared with total thyroidectomy, completion thyroidectomy has been associated with similar rates of recurrent laryngeal nerve injury and lower rates of hypoparathyroidism. After performing completion thyroidectomy, serum thyroglobulin levels tend to be an adequate prognostic follow-up marker. Beside the effectiveness of radioactive iodine for ablation of the remaining normal tissue or residual microscopic disease is enhanced after completion thyroidectomy. Recurrent laryngeal and superior laryngeal nerve monitoring during completion thyroidectomy is associated with a decrease risk of injury. Completion thyroidectomy is a safe procedure with acceptable morbidity in the hand of experience surgeons.

Hematocolpos after Cloacal Repair

In females the most common anorectal malformation is an imperforate anus with a rectovestibular fistula, followed by rectoperineal fistula and the cloacal anomaly. Cloacal repair entails reconstruction of the urethra, vagina and rectum which end in a common channel. The aim of vaginal reconstruction is to provide a cosmetically satisfactory introitus, a conduit for normal menstruation and pain-free penetrative intercourse. There is a strong association of gynecologic anomalies (60%)  associated with cloaca. A high rate of menstrual obstruction (40%) at puberty is well described. In less complex malformations such as rectovestibular and rectoperineal fistula a vaginal septum is the most common associated finding and can be managed most effectively during the initial repair of the rectum without trauma to the hymen or introitus. Vaginoscopy allows evaluation of the vaginal anatomy during infancy and puberty by documenting of vaginal duplication with 2 hemivaginas, and a septum, documentation of the septal length and total vaginal length. During vaginoscopy, perhaps at colostomy closure or creation of an appendicostomy, documentation of the presence of mucus at the outer part of the cervix can be evaluated. Also cannulation of the distal fallopian tube with instillation of saline can be performed to visualize the egress of saline from the vagina and confirm patency of the Müllerian system. Hematocolpus is a medical condition of blood retained in the proximal vagina due to an outflow tract obstruction or blockage of menstrual flow. The most common cause of hematocolpus in children without anorectal malformation is an imperforate hymen. In cases of repaired cloaca a longitudinal or transverse vaginal septum, congenital or acquired vaginal atresia or severe vaginal stricture, uterus didelphys and septate uterus can cause hematocolpus. Ovarian function is normal in girls with repaired cloaca so pubertal and breast development occurs as expected. Thelarche (breast development) occurs between 9-10 years of age with menstruation occurring at 12-12.5 years of age. Confirmation of the patency of the reproductive tract before menarche is important to avoid obstruction, pain, and risk to reproductive organs with infertility. It is during this time that repaired cloaca can be studied further to determine if there could exist the possibility of menstrual flow tract obstruction and development of hematometrocolpos by performing serial ultrasound studies. Almost 20% of children born with cloaca develop amenorrhea due to absence of or underdeveloped Müllerian structures. US surveillance of the reproductive structures should begin 6-9 months after thelarche and continue every 6 months through menarche. If an obstruction to menstrual flow is detected by visualization of a thickened endometrium with hematocolpos, medical intervention should be initiated immediately to minimize adverse sequelae. Hormonal suppression of menses and endometrial stimulation should be started if there is menstrual flow obstruction to prevent continued accumulation of blood. Surgery is usually necessary to establish an adequate outflow tract either by resection of a vaginal septum, introitoplasty, posterior vaginoplasty or vaginal replacement with bowel in cases of absent vagina. Other gynecologic concern for pubertal females include the development of adnexal cysts, hydrosalpinges, endometriosis and chronic pelvic pain. Women who had a history of a cloacal anomaly should be delivered by cesarean section.  

Apple Peel Atresia

Jejunoileal atresias are classified as type I characterized by a transluminal septum; type II involves a fibrous cord connecting two blind ending pouches; type IIIA has a V-shaped mesenteric defect; and type IV exhibits multiple atetric segments. The term Apple peel atresia (or Type IIIB intestinal atresia), occurring in less than 10% of all jejunoileal atresias, refers to the development of a high jejunal atresia with discontinuity of the small bowel and a wide gap in the mesentery. The distal segment of jejuno-ileum is shortened and assumes a helical configuration around a retrograde perfusing vessel. The appearance is similar to a Christmas tree, hence the synonym Christmas tree deformity. An intrauterine vascular accident after emergence of the middle colic artery to the affected proximal bowel during late gestation has been accepted as the cause of apple-peel atresia presenting with a wide spectrum of occlusions of one or more branches of the superior mesenteric artery. The bowel distal to the atresia is precariously supplied in a retrograde fashion by anastomotic arcades from the ileocolic, right colic or inferior mesenteric artery. Most of these children have less than half of the normal length of the small bowel and a physiological short bowel. Apple peel atresia is usually reported as an isolated malformation, but has also been related to malrotation, situs inversus and polysplenia. Not all distal apple-peel atresias are associated with a proximal bowel atresia since a few scattered reports of a mesenteric defect associated with a marginal artery may cause the coiling defect of the apple peel as the bowel outgrows it blood supply causing problems of ischemia later in life associated with mesenteric internal hernias. Also, not all apple-peel atresia are from the proximal jejunum, since very few have been described arising after a proximal duodenal atresia related to the second portion of the duodenum with absence of the third and fourth portions of duodenum and superior mesenteric artery. Infants born with high jejunal atresia have considerable dilatation of the proximal bowel, while the distal segment is small and collapsed. Anastomosis between two discrepant bowel sizes can cause functional bowel obstruction. The resulting peristalsis is incapable of producing an adequate upstream pressure gradient. As alternative, an antimesenteric reduction-tapering proximal jejunoplasty can be used to perform anastomosis reducing the caliber of the proximal bowel to fit an almost end to end anastomosis with the distal microbowel. Serial transverse enteroplasty of the dilated proximal jejunal atresia can also be performed to reduce the caliber while lengthening it appropriately without significant loss of absorptive area. Apple-peel atresias frequently have a high incidence of prematurity, short gut, multiple atresias and associated anomalies which constitute potential prognostic factors. Antenatal US may suggest the diagnosis of jejunoileal atresia with the presence of dilated fluid-filled loops of bowel and polyhydramnios. Surgery is the preferred mode of treatment for jejunoileal atresias and the goal of treatment is to establish bowel continuity while preserving as much bowel length as possible. Postoperative complications associated with apple-peel atresia include anastomotic dysfunction (most common), sepsis (from leak or TPN), short bowel syndrome, necrotizing bowel, stenosis and even death. Survival is above 90%. 

PSU Volume 58 No 02 FEBRUARY 2022

Pectus Arcuatum

Anterior chest wall deformities are not rare. They consist of pectus excavatum as the most frequent form of chest deformities, and by protrusion deformities such as pectus arcuatum and carinatum. Pectus arcuatum, also known as pouter pigeon chest, Currarino-Silverman syndrome, chondro-manubrial deformity or type two pectus carinatum, is a rare and complex congenital chest wall deformity whose main feature is protrusion and early ossification of the sternal angle of Lewis associated with bilateral deformity of the 2nd to 4th cartilages. It may also be associated with a depressed lower sternum.  It also involves a wavelike deformity, a mixed form of excavatum and carinatum features, either along a longitudinal or along a transverse axis. The visual appearance of pectus arcuatum is formed by the costal cartilage protrusion. In most cases pectus arcuatum is a cosmetic defect. Though the diagnosis is established by physical exam, chest films and CT-Scan with 3-D reconstruction are needed if repair of the sternal defect is warranted. Imaging using thoracic CT scans and customized aided design virtual simulation allows the surgeon to predetermine the specific cutting angle for each patient and therefore design a cutting template tailored to the individual deformity.  The surgical repair of this rare deformity requires a modified open Ravitch technique. Patients are usually young adults without comorbidities and no special preparation is needed. The basic steps in the surgical correction described by Ravitch consist of bilateral parasternal and subperichondrial resection of the deformed costal cartilages, detachment of the xiphoid process, transverse wedge osteotomy at the upper edge of the sternal depression, and bending of the sternum to straighten its course, securing the corrected position of the sternum. Satisfactory overall results occur in 98% of patients. The Ravitch technique has a risk of growth limitation to the thoracic cage due to a wide resection of the rib cartilages, the reason that the repair is not undertaken until the child has acquired a rigid skeletal structure later in life. Pectus arcuatum can be successfully corrected by Ravitch-type of chondrosternoplasty. Due to necessity to resect cartilages, late puberty or adulthood is preferred, since by that age the growth of ribs have finished. Repair of pectus deformity in children that might need future cardiac surgery has revealed that concomitant surgery is contraindicated before adolescence because pectus deformities may spontaneously disappear or recur after early sternal surgery. Congenital heart defects are reported occasionally as well as simultaneous Poland syndrome. Concomitant surgery of cardiac defects and pectus deformity is a reliable strategy in adolescent and adults offering long-term results. The modified Ravitch technique is more adequate as it can be used in all types of deformities and in concomitant surgery allowing optimal operative exposure during cardiac procedures, easy postoperative reentry and resuscitation maneuvers if needed.  

Imposter Syndrome

Imposter syndrome refers to a feeling of self-doubt or innate fear of being discovered as a fraud or non-deserving professional, despite their demonstrated talent and achievements. Imposter syndrome is characterized by a chronic sense of self-doubt coupled with a constant worry of being discovered as a fraud. Imposter syndrome is more prevalent in high achievers, women, and under-represented racial, ethnic, and religious minorities. Impostor syndrome is increasingly recognized as a condition between physicians and physicians in training. Despite remarkable academic and professional achievements affected individuals  beliefs that they were unintelligent. For affected individuals, imposter syndrome can lead to burnout, psychological distress, emotional suffering, and serious mental health disorders, including chronic dysphoric stress, anxiety, depression, drug abuse and suicide. Most cases start early during high school or college. The true incidence of imposter syndrome is unknown in medical professionals. In the US, among medical students the rate of imposter syndrome was 49% in women and 24% in men, and among residents the rate was similar. The root cause of imposter syndrome is not known, but it has been related to depression and anxiety, which are both present among residents, with suicide being the second most common cause of death among residents. Imposter syndrome among general surgery residents is not only prevalent but severe with 76% of residents reporting either significant or severe imposter syndrome. Neither sex nor age correlates with the presence of, or level of, imposter syndrome in the general surgery resident population. It is believed that imposter syndrome is an incidentally protective mechanism encouraged by the hierarchical culture of surgical training by which residents are encouraged to self-regulate their decision-making process. By constantly downplaying their own accomplishments, those suffering from imposter syndrome may sabotage their own career. Institutions must address imposter syndrome by increasing the visibility of the problem, providing access to mental health coaching, and establishing supportive organization policies. Institutions and residency programs should provide training for mentors to help them recognize the negative consequence of the imposter syndrome. Medical educators must recognize that it is not just the underperforming or failing learners who struggle and require support, and medical culture must create space for physicians to share their struggles. The Accreditation Council for Graduate Medical Education (ACGME) requires residency programs to support residents  well-being via established policies and programs. Imposter syndrome has been linked to burnout and suicide in residents and understanding how to combat it may help improve resiliency in residents. Imposter syndrome has been linked to resident burnout and discussing imposter syndrome is viewed as an effective intervention to promote resident wellness and resiliency. When creating wellness interventions, residency programs should consider addressing imposter syndrome.

Short Bowel Syndrome

Short bowel syndrome (SBS) refers to a compromised bowel absorptive capacity due to severely reduced mucosal surface resulting in diarrhea, water-electrolytes imbalances, and protein malnutrition. SBS is the most common cause of intestinal failure in children. Most underlying conditions that lead to major loss of intestine in neonates have their origin in intrauterine life. SBS usually occurs after extensive bowel resection, either congenital or acquired, such as that associated with small bowel atresia, complex gastroschisis, midgut volvulus and necrotizing enterocolitis. The most common acquired cause of SBS is NEC with 30% in most reported series. Factors influencing outcome in SBS include underlying diagnosis, type of segments preserved, stoma vs primary anastomosis, presence of ileocecal valve and the age of the child at the time of surgery.  Massive resection stimulates modification in thickness and length of the muscle layer and villi crypts. Distension of the remaining bowel is the most common consequence after massive resection. Massive jejunal resections are better tolerated than significant ileal resections. Ileal resections are associated with impaired resorption of Vitamin 12, bile salts and fatty acids. Three anatomical subtypes of SBS: (1) small bowel resection with anastomosis and intact colon; (2) small bowel resection with partial colon resection; (3) small bowel resection with high output jejunostomy. Type 1 has the best potential for adaptation, while type 3 the least. With the advent of parenteral nutrition support survival of SBS improved significantly. Management of SBS aims to promote adaptation of the remnant bowel. Parenteral nutrition (PN) provides nutrition while the bowel achieves intestinal autonomy. The bowel should be use for feeding as much and early as possible to stimulate adaptation. Oral feeding maintains sucking and swallowing functions, promotes release of epidermal growth factor from salivary glands and increases GI secretion of trophic factors. Breast feeding should be encouraged. Long term PN leads to sepsis, cholestasis due to liver failure and death. Key predictors of mortality in SBS include cholestasis (conjugated bilirubin > 2.5 mg%) and percentage of small bowel length. A small bowel length greater than 10% of expected for a given gestational age is highly predictive of survival (see normal bowel length in accordance with gestational age graph). Presence of an ileocecal valve and percentage of small bowel length are primary predictors of weaning PN.  Surgical approaches to maximize bowel digestive and absorptive function are important in the management of SBS. These include stoma closure, bowel continuity restoration, resection of strictures and closure of fistula. When the bowel is short, dilated, and static children might benefit from longitudinal intestinal lengthening and tapering (Bianchi) or serial transverse enteroplasty (STEP) procedures. The UGIS provide accurate estimates of bowel diameter and length use to operative planning. Surgical bowel lengthening should be considered in any chronically PN-dependent child when there is substantial bowel dilatation and symptoms of small intestinal bacterial overgrowth regardless of remaining bowel length. Medical approach to SBS include antidiarrhea/ antimotility agents and controlling acid/base balance. Hypersecretion of gastrin and gastric acid occurs in children after extensive small bowel resection which should be managed. Octreotide inhibits gastrin and diarrhea prolonging transit time. Intestinal bacterial overgrowth frequently seen in SBS is managed with probiotic and antibiotic therapy. Promising hormonal therapy include glucagon-like peptide 2 hormone (Teduglutide) produced by the L-cells of the terminal ileum. Teduglutide has a trophic effect on the bowel, promotes absorption and adaptation. The future of SBS might lie in an artificial grown and engineered harvested intestine. Micronutrient deficiencies are frequent during intestinal rehabilitation for SBS. The most common micronutrient deficiency include zinc, copper, vitamin D and phosphorus after the transition to enteral nutrition. With liver failure and reduced venous access, bowel transplantation becomes the treatment of choice. 

PSU Volume 58 No 03 MARCH 2022

Extremity Compartment Syndrome

Extremity compartment syndrome (ECS) in children is a potential cause of permanent disability. Sustained increased pressure in the limb fascial compartments compromises circulation causing ischemia and necrosis of the contents within. Early recognition is critical in avoiding further disability. Diagnosis of compartment syndrome in children can be challenging due to poor cooperation, difficulty with communication and difficulty measuring compartment pressures of the affected limb in the conscious child. The most common risk factors for ECS in the pediatric age comprise tibial diaphysis fractures, soft-tissue injury, distal radius fracture, radius and ulna diaphysis fracture and crush injury. Extremity fractures causes most ECS in children (75%) followed by vascular injury, tibial osteotomy, and soft-tissue injury. ECS can also arise from extrinsic causes that exert pressure such as compressive casts or bandages, pneumatic antishock garments, or intrinsic factors that increase the volume inside the fascial envelops such as septic arthritis, intraosseous infusions, toxic venom, burns, intramuscular hematomas, hereditary bleeding disorders and viral diseases. An open fracture of the forearm or leg significantly increases the risk for ECS. Nontraumatic causes of limb compartment syndrome in children include ischemia-reperfusion events after arterial injury, thrombosis, burns, bleeding disorders and blunt injury. The pathogenesis of ECS is tissue damage leading to increased intracompartmental pressure way above the closing pressure of venules. Continued arterial inflow increases the pressure until the arterioles develop stasis and ischemia occurs. Prolonged ischemia beyond a six-hours period results in ischemic muscle which may result in myonecrosis, chronic contracture and permanent nerve damage. Compartment syndrome is a clinical diagnosis. The affected patient develops paresthesia, numbness, swelling and pain out of proportion or with passive movement of the extremity. Diminished pulses, pallor and progressive neurologic deficit are late findings less commonly seen. Pain is one of the earliest symptoms of ECS. Sensory deficit occurs before motor dysfunction. Paresthesia in the affected extremity is one of the first signs of hypoxia to nerve tissue within a compartment. Blood flow in the capillary circulation ceases when compartment pressure exceeds 35 mm Hg. The sensory nerves are affected first, followed by the motor nerves and muscle, fat and skin become involved later. ECS can be confirmed by the measurement of tissue compartment pressure greater than 30 mm of Hg. The normal pressure in a muscle compartment is less than 10-12 mm Hg. This diagnostic method is essential in uncooperative, altered mental status, very young or children with inconsistent clinical symptoms. Measurement of compartment pressure can be performed using a slit catheter, wick catheter, needle manometer, electronic arterial pressure transducer, or a solid-state transducer intra compartment catheter. Management of symptomatic ECS with pressures above 30 mm Hg is urgent decompressive fasciotomy. Favorable outcomes are found in children who had a fasciotomy less than six hours from the time of diagnosis. After fasciotomy, limb compartment pressures should be monitored as progressive muscle swelling may continue as a result of toxic effects of infection. The lower leg is the most common location of acute ECS with the anterior and lateral compartments most frequently affected. Children tolerate increased intracompartmental pressure for longer periods of time than adults before tissue necrosis becomes irreversible. The most common complications after ECS in children is an unpleasant scar since wound closure after upper or lower extremity fasciotomies require split thickness skin graft. Silent compartment syndrome is defined as confirmed compartment syndrome without significant pain or absence of marked pain on passive motion. Pediatric patients generally achieve good outcomes even when presenting in a delayed fashion and undergoing fasciotomies after 24 hours of the initial event. Decompressive fasciotomy is recommended even if there is prolonged time from injury to diagnosis.

Diverticulitis in Children

The most common diverticulum in children causing surgical problems is the Meckel's diverticulum. In very rare occasion the pediatric patient can develop diverticular disease of the colon similar to that occurring in the adult. Such diverticular disease can lead to colonic diverticulitis. Diverticulitis is predominantly a disease of adults older than 50 years of age, being extremely rare in children. Low quantity dietary fiber, obesity, constipation, decreased physical activity, steroids, and smoking all predispose individuals to diverticulosis. Chronic increase intraluminal pressure leads to formation of pseudo-diverticular outpouching. Less than 5% of all patients with diverticula of any etiology develop diverticulitis. The prevalence in the population younger than 40 is only 10%. No matter the age group or etiology, diverticula can develop anywhere along the colon from the cecum and appendix to the sigmoid colon. When diverticular disease occurs in children, they are associated with alterations in the component of the colonic wall. Some genetic disorders in children are associated with diverticulitis due to weakening of the colonic wall by alteration of collagen or elastin synthesis within the tissues. They include cystic fibrosis, Ehlers-Danlos syndrome, Marfan syndrome and William-Beuren syndrome. Possible complications of colonic diverticulosis include bleeding, inflammation (diverticulitis), and perforation. Symptoms depend on localization of the diverticula. Differential diagnosis includes colon cancer, Crohn's disease, ischemic colitis, pseudomembranous enterocolitis, and pelvic inflammatory disease. CT-Scan or MRI is utilized to diagnose diverticulitis. Ultrasound and MRI can be useful alternatives in the initial evaluation of a patient with suspected acute diverticulitis when CT imaging is not available or is contraindicated. Pediatric colonic diverticulitis is often associated with a more complicated course than that seen in adults  patients. Most pediatric cases have been described in the cecum or ascending colon as a true diverticulum. Right sided diverticulitis is relatively rare inflammatory condition affecting the cecum and ascending colon. The incidence in children has not been determined since most cases are incorporated into adult series. They may be found as solitary lesion, multiple lesions, or parts of generalized diverticulosis of the colon. Right colonic diverticula are predominantly congenital and solitary being true diverticula consisting of all layers of the bowel wall. Most children complain of right lower quadrant pain with tenderness associated with nausea and vomiting. Since they can mimic appendicitis, the diagnosis is often difficult. Nonoperative management with antibiotics and bowel rest is advocated by most, leaving resection or diverticulectomy for recurrent episodes, obstructing mass, abscess, fistula, or perforation. The recurrence rate of children managed successfully with intravenous antibiotics is 18%. Classic findings related to sigmoid diverticulitis in adults include left lower quadrant pain, fever, and leukocytosis. Complicated diverticulitis is defined as diverticulitis associated with uncontained, free perforation with systemic inflammatory response, fistula, abscess, stricture, or obstruction. Micro-perforation in the absence of a systemic inflammatory response is not considered complicated diverticulitis. Symptomatic uncomplicated disease is defined as diverticulosis with associated chronic abdominal pain in the absence of clinically overt colitis. CRP above 150 mg/L is associated with complicated diverticulitis. Elevated procalcitonin is associated with diverticulitis recurrence.  Most cases resolved with antibiotics. Patients with recurrent symptoms, diverticular abscess, fistula, obstruction, or stricture will need surgery. An elective resection based on young age at presentation is not recommended. Management of pediatric patients with diverticulitis should be multidisciplinary, including GI, surgery, genetics, cardiology, and ophthalmology.  

Inguinal Lymphadenopathy

Inguinal lymphadenopathy (IL) refers to the condition in which peripheral inguinal or groin lymph nodes become abnormally enlarged, sometimes tender to palpation causing concern to caretakers. Lymphadenopathy is a common clinical manifestation in the pediatric age group. It may be part of normal age-related physiology or in response to any local or generalized infection in the body.  Inguinal adenopathy may be a symptom to several disease process, most commonly of infectious origin. The history of the child with IL should be studied carefully since it provides clues to the underlying disease. Most groin adenopathies are self-limited infections in young patients. When confronted with a child with IL laboratory tests, imaging studies and tissue diagnosis might be needed unless there is a clear explanation for the sudden growth of the lymph node. Ultrasound, a non-invasive and non-radiating imaging study, is the best study to assess lymph nodes in the groin and neck. CT-Scan should be avoided with peripheral lymphadenopathies to reduce radiation injury associated with this imaging modality. CT-Scan is more helpful with central lymph nodes in the thorax or abdominopelvic cavities. Fine needle aspiration (FNA) biopsy can be used as initial management.  FNA biopsy is easy, safe, rapid and a cost-effective tool, but will need a cooperating child to be performed. Excisional biopsy of the enlarged lymph node is the gold standard procedure and in the groin the procedure is usually simple, fast, and free from major complications. In fact, the most common complication is seroma which resolves spontaneously in most cases. In the groin lymph nodes larger than 1.5 cm in children are abnormal. It must be determined if the lymphadenopathy is localized or generalized. Generalized lymphadenopathy is defined as the enlargement of two or more groups of noncontinuous groups of lymph nodes. It results from systemic illness like infections (viral, bacterial, fungal, and protozoan), malignancies, autoimmune disease, drugs reactions, histiocytic disorders, disseminated neoplastic diseases and storage disorders. If the child does not present with overt signs of malignancy, the lymph node can be safely watch for three to four weeks before considering biopsy. Benign lesions are more commonly encountered than malignant lesions in children and include benign reactive hyperplasia (by far the most common pathology; approximately 65-85%), chronic skin diseases, cat-scratch disease, toxoplasmosis, necrotizing granulomatosis, and non-necrotizing granulomatosis. Of the malignant conditions, non-Hodgkin lymphoma is the most common. FNA biopsy can be helpful in differentiating benign from malignant pathology but is often faced with failure due to quantity of tissue to provide a diagnosis. As mentioned previously, an open biopsy with removal of the whole adenopathy is almost always diagnostic. Chronic skin disorders are a cause of what is known as dermatopathic lymphadenopathy, an entity that represents a secondary immune response to a pathologic condition affecting primarily the skin.       

PSU Volume 58 NO 04 APRIL 2022

Brunner Gland Adenoma

Brunner gland adenoma (BGA), also known as an hamartoma, is a rare duodenal lesion comprising not more than 5% of benign duodenal lesions. Most Brunner gland adenomas are of small size without causing significant symptoms. When BGA grows, they can cause obstruction or bleeding. Brunner glands are submucosal mucin-secreting glands predominantly located in the posterior wall of the duodenal bulb and second portion of the duodenum segment and progressively decrease in size and number in the distal potions of the proximal bowel. Brunner's glands secrete an alkaline fluid composed of viscous mucin which protects the duodenal epithelium from acid chyme of the stomach. BGA are rarely larger than 2 cm in asymptomatic individuals, while larger than 5 cm in symptomatic patients. Brunner's gland could be classified into three types based on size: type 1 (diffuse nodular hyperplasia) confined to the mucosa with multiple sessile projections occupying most of the duodenum; type 2 (circumscript nodular hyperplasia), found in the bulb duodenum and usually smaller than 1 cm; and type 3 (Brunner's gland adenoma) stemmed with sized 1-2 cm, generally without clinical manifestations. Etiology of BGA is unknown. It is believed that gastric hypersecretion of acid results in hyperplasia of Brunner's gland resulting in adenoma formation and excrescence toward the bowel lumen. Others believe the loss of the alkaline protection of the exocrine pancreas leads to compensatory hyperplasia of Brunner's gland with increased production of mucus and alkali. Helicobacter infection has also been found culprit of BGA, though a clear relationship has not been met. It is believed BGA are hamartomas. The word adenoma might be a misnomer, since the mass is not a true neoplasm, but rather a hamartomatous or hyperplastic collection of mature glands with no known potential for malignant transformation. Patients with BGA are usually asymptomatic, or developed symptoms of nausea, vomiting, bloating, dyspepsia, vague abdominal pain, melena, or hematemesis. Pancreatitis (ampullary lesions), intussusception and diarrhea have also been reported. Those in the pylorus often presents with epigastric pain, dyspnea, or melena, whereas those in the posterior wall of duodenum often presents with postprandial fullness. When the adenoma grows it can cause symptoms of obstruction or gastrointestinal bleeding. Chronic bleeding with ulceration is found in most symptomatic patients.  On rare occasion BGA can cause gastric outlet obstruction. The most common laboratory finding in symptomatic patients is anemia. Abdominal imaging (Ultrasound/CT-Scan/MRI) or endoscopy can detect the lesion. Upper endoscopy localizes the lesion and biopsy can provide the diagnosis of a BGA. Endoscopic biopsy shows involvement of the mucosa and submucosa layers without deeper extension, variable echogenicity, and cystic changes within the lesion. Cytologically, Brunner's gland shows loose clusters of flat, two-dimensional cells with minimal overlapping or atypia showing abundant, finely granular, and vacuolated cytoplasm. In most cases, diagnosis is confirmed after endoscopic or surgical resection. Endoscopic resection is recommended to avoid developing symptoms with time. When endoscopic resection is not possible, surgical resection is indicated. Recurrence rate after both modalities of management is very low. BGA have been reported very rarely in the pediatric age and can be associated with surgical repair of duodenal atresia. BGA are benign and the prognosis after endoscopic or surgical management is good.

Hirschsprung's Associated Enterocolitis (HAEC)

Hirschsprung's disease (HD) occurs in approximately one in 5000 live births with most babies presenting with failure to pass meconium in the first 24 hours of life. Failure to recognize HD early in infancy place them at high risk of developing HirschsprungÕs associated enterocolitis (HAEC). HAEC is a serious life-threatening inflammatory complication of HD. HAEC occurs preoperatively in 6-26% of cases, and post pull-through surgery in 5-42%. HAEC is histologically characterized by cryptitis, the appearance of neutrophils in intestinal crypts. They progress to crypt abscess, mucosal ulceration and fibrinopurulent debris. In severe cases ischemia, transmural necrosis and perforation can occur leading to shock and hypoperfusion. Pathophysiologic mechanism associated with HAEC includes partial mechanical obstruction (bacterial translocation), unbalanced microflora (dysbiosis), insufficient immunoglobulin secretion, abnormal mucin production (impaired mucosal barrier), and dysfunction of the enteric nervous system. Mortality of HAEC ranges between 1% and 10%. Several factors contribute to an increased risk of HAEC, these include family history of HD, Down syndrome, long-segment HD involvement, obstruction from any cause (retained aganglionosis, transitional zone pull-through, dysmotility following pull-through, anastomotic stricture, twist in the pull-through or tight muscular cuff following surgery), and prior episodes of HAEC. Some authors believe no patient or clinical characteristic is associated with the risk of postoperative HAEC. The risk of developing HAEC increases with the length of the aganglionic segment and delay in diagnosis. The diagnosis is suspected when a child with constipation develops abdominal distension, pain, vomiting, explosive watery diarrhea, fever, lethargy, rectal bleeding, and shock. Initial images consist of plain radiographs which can demonstrate a cutoff sign in the rectosigmoid colon with absent distal air, dilated proximal bowel loops, air fluid levels, pneumatosis intestinalis, "sawtooth' appearance with irregular intestinal lining, or pneumoperitoneum from bowel perforation. Barium enema and colonoscopy are contraindicated in the acute setting due to the risk of perforation, while CT-Scans are of little value in the diagnosis and treatment of HAEC. Chronic HAEC symptoms include persistent diarrhea, soiling, intermittent abdominal distension, and failure to thrive. Should this occur after surgical management mechanical obstruction from aganglionosis should be suspected and confirmed with rectal biopsy. The diagnosis of HD is made histologically by the absence of ganglion cells, presence of nerve hypertrophy and absent calretinin immunohistochemistry. For prophylactic prevention of HAEC routine rectal irrigation or diverting colostomy is indicated in selected patients. Rectal irrigations reduce fecal stasis and bacterial load, limiting colon distension. Children with HD and severe congenital heart disease should be diverted to avoid HAEC. Probiotics management has controversial results as a preventive measure. The clinical suspicion and severity of HAEC have been graded based on history, physical examination, imaging studies and laboratory findings similar to Bell's criteria for neonatal enterocolitis. Four or more of 16 criteria seen in the Table below are diagnostic. Such score helps make the correct diagnosis of HAEC. Existing scoring systems perform poorly in identifying episodes of HAEC, resulting in significant underdiagnosis. Management of HAEC includes fluid resuscitation using isotonic solutions, broad spectrum intravenous antibiotics, decompression of the gastrointestinal tract and bowel rest. Systemic antibiotics are used empirically in HAEC, with metronidazole typically chosen to managed anaerobes. Those children with severe sepsis and acutely ill (Grade III) benefit with admission to the intensive care unit some of them needing vasopressor therapy and ventilatory support. Rectal washouts with warm saline are the mainstem of management and they should be performed two to four times daily at a rate of 10-20 ml/kg of weight each time or until the effluent is clear. In newborns presenting with severe HAEC, shock and sepsis immediate diversion (leveling colostomy) should be considered. Other risk factor associated with the need of diversion includes delayed presentation, co-morbid conditions, presence of HAEC or multiple risk factors for HAEC in the preoperative period. Diversion improves patient symptoms but does not resolve HAEC development later in life. HAEC can also occur in the postoperative period after pull-through definitive surgery. Rectal washouts are also effective to manage postoperative HAEC. There is a trend toward a higher incidence of enterocolitis in the primary endorectal pull-through group as compared with those with a two-stage approach. Postoperative HAEC can occur more than 18 months after definitive surgery.  The postoperative risk of developing HAEC after definitive surgery is highest in those operated with Swenson and Duhamel approach (20%), and lowest with Soave procedure (11%). Children with recurrent HAEC, occurring in 2-33% of patients, should be evaluated for anatomic or pathologic causes of obstruction. A water-soluble contrast enema can identify any mechanical cause of obstruction. Should the suspicion of persistent aganglionosis be considered a rectal biopsy should be performed. Risk factors for recurrent HAEC includes preoperative HAEC, history of central nervous system infection and congenital chromosome anomalies and does not include placement of an ostomy prior to pull-through and congenital cardiac anomalies. Recurrent postoperative HAEC does not have an impact on mortality. Children with previous episodes of HAEC are more likely to develop subsequent episodes.

Transanal Pull-Through

In 1998, De la Torre introduced a new one-stage surgical technique to manage Hirschsprung's disease (HD) where rectal mucosectomy, aganglionic segment colectomy, posterior myectomy, and normoganglionic colon pull-through is performed through the anus. HD aganglionosis affects the rectosigmoid (classic HD; 85%), long-segment (10%) and total aganglionosis (5%) of the colon with sporadic cases affecting the proximal small bowel. The transanal pull-through (TAPT) is mainly design for children with classic HD and uses a prone approach, though it can also be performed in supine position. Advantages of the TAPT include abdominal or intraperitoneal bowel opening is not necessary unless the child has a previous leveling colostomy, risk of adhesion is decreased, excellent cosmetic results, earlier full oral feedings, shorter hospital stay, less costs, less pain, reduced operating time, endorectal dissection preserves the anorectal sphincters and their blood supply along with innervation causing less damage to fecal and urinary incontinence. Should the ganglionic bowel need further dissection proximally laparoscopy can provide this mobilization. The procedure can be performed in newborns with potential benefits of avoiding a colostomy and establishing colonic continuity early in life improving continence results. A stoma in HD is considered appropriate in children with bowel perforation (usually cecal), severe malnutrition, severe enterocolitis, very dilated proximal bowel, total colonic aganglionosis or lack of adequate pathological support. Should the surgeon choose to wait beyond the neonatal period for final correction of HD the risk of developing enterocolitis should be minimized by ensuring adequate decompression of the proximal dilated bowel by appropriate daily irrigations, administration of prophylactic metronidazole or probiotics, and use of breast feeding or elemental infant formula. Using laparoscopy concomitantly with the transanal approach can help in the proximal bowel dissection, distal bowel dissection and obtaining a seromuscular biopsy for frozen section to determine the ganglionic bowel to be pull-through. A frozen full-thickness biopsy helps the pathologist see both submucosa and myenteric plexus in look for ganglion cell and nerve hypertrophy minimizing error. Whether using a supine or prone position for the procedure depends on the certainty that you are dealing with classic HD and proximal mobilization will not be required. The prone position has the advantage that mesenteric vessels can be send and controlled more effectively than in the supine or lithotomy position. The transanal dissection can be started 0.5-1 cm above dentate line in neonates and 1-2 cm above the dentate line in older children as the transitional epithelium must not be damaged to avoid loss of sensation and incontinence. Dissection on the outside of the rectum, as that used during laparoscopic or open Swenson procedures, may increase the risk of injury to pelvic nerves and vessels, and to the prostate, urethra, and vagina. Comparison between complete TAPT and laparoscopic-assisted TAPT do not differ between rates of major complications, including leaks, strictures, enterocolitis, fecal incontinence, postoperative obstructive symptoms, and mean length of stay. Comparison between the Duhamel procedure and the TAPT have shown they are similar in respect postoperative fecal incontinence and operation time, but the Duhamel is associated with longer hospital stay and lower rate of enterocolitis. When performing the TAPT in neonates, a higher incidence of enterocolitis has been reported due to increased risk of sphincter spasm and anastomotic strictures. Excising the entire posterior rectal muscle cuff (myectomy) has been effective in reducing the incidence of enterocolitis. Current guidelines suggest doing the TAPT between two and three months of age if the child is growing well and the bowel is sufficiently decompressed. Whether performing the procedure totally transanally or laparoscopy assisted does not affect long-term bowel function. More than 20% of patients develop at least one complication within the 30-days following a TAPT. Older age at time of surgery increases the risk of developing a postoperative complication. They include anastomotic leakage, abdominal abscess, and anastomotic strictures. Ischemia and increased tension on the colon anastomosis play an important role in leakage and stricture. This is one reason to consider perioperative diverting stoma in older patients. Older age at time of surgery, laparotomy-assisted and long segment disease increases the risk of developing a postoperative complication. Long term problems with pull-through surgery for HD include obstructive symptoms (30%), persistent constipation, soiling, enterocolitis and descending aganglionic bowel. Persistent bowel obstructive problems might be caused by mechanical obstruction, recurrent or acquired aganglionosis, disordered motility in the proximal colon, nonrelaxation of the internal anal sphincter, or stool-holding behavior. Mechanical obstruction might also be caused by an anastomotic stricture, twisted descended pull-through colon, or rolling of a long muscular cuff left behind. They are managed by sequential dilatations or redo pull-though surgery. Soiling can be caused by damage sphincter function (incontinence), abnormal sensation and pseudo-incontinence. Anorectal manometry is indicated to determine the cause. Soiling not associated with constipation is caused by true fecal incontinence and does not improve with time. Thus, to preserve the anal canal and avoid sphincter damage are of vital importance during the TAPT. Enterocolitis is managed with bowel rest, colonic irrigations, and systemic antibiotics. Most children have an excellent quality of life going into adulthood after TAPT. Besides the management of HD, the TAPT can be utilized for children born with rectal atresia, for severe chronic idiopathic constipation associated with megarectosigmoid, idiopathic rectal prolapse, and children with rectal prolapse after anorectal malformation correction.   

PSU Volume 58 NO 05 MAY 2022

Slipping Rib Syndrome

Slipping rib syndrome (SRS) is a rare cause of lower rib and abdominal pain in children and adults. SRS accounts for approximately 5% of all musculoskeletal chest pain in primary care. SRS is caused by a hypermobility of the anterior false ribs that allows the 8th to 10th ribs to slip or click as the cartilaginous rib tip abuts under the rib above. The costal cartilage slips out of its normal anatomical position, the anterior false ribs (8th through 10th) slide out of orientation and become pinned underneath their adjacent superior ribs. This displacement is caused by a congenital anomaly, damage to the fibrinous articulation, or hypermobility of unknown origin. Impingement of the intercostal nerve along the adjacent surface of the adjacent ribs during this slip occurs causing acute pain. Failure to diagnose this condition might lead to unnecessary tests in the child. The pain is caused when the lower costal cartilages 8th to 10th which are not connected to the sternum lose their fibrous or cartilaginous attachments to each other. Pain can extend to the floating ribs (11th and 12th). Movements in the child such as twisting, simple lumbar flexion, bending, deep breathing, laughing, sitting, sneezing, or coughing results in irritation of the intercostal nerve hence pain. Pain is sharp, intermittent, stabbing lasting for a few minutes followed by a dull or burning sensation for several hours. Sometimes associated with nausea and vomiting. Slippage may produce a clicking or popping sensation. Pain can range anywhere from the midline to the lateral flank and from the xiphoid process to as inferiorly as the umbilical line. Due to interconnections between intercostal and somatic visceral nerves the pain might be interpreted as upper abdominal (subcostal). The lack of significant radiographic findings makes the diagnosis of SRS difficult using imaging. Time to diagnosis is often years. Differential diagnosis includes rib fracture, chondritis and pleuritic pain. There is usually one dominant affected side, though the syndrome can occur bilaterally. SRS can occur in any age group and females athletes (swimmers) are more commonly affected than males. Many of these patients have hypermobile joints with laxity and subluxation without symptoms. Diagnosis is established through history and physical examination as imaging are not very useful. Dynamic US can be of help in the diagnosis by demonstrating an overlapping movement of the lower rib above the upper rib. Less thickness of the ipsilateral rectus abdominis muscle has also been found. Pain is intermittent and localized to the lower ribcage with some trigger point of tenderness palpable. Popping or grinding sensation with movement can be elicited. At physical exam the hook maneuver, the examiner slides his finger under the costal margin and lift anteriorly and superiorly, reproducing a click and pain and diagnosing the condition. Careful palpation can identify the disconnected cartilage, or the cartilage curling beneath the overlying ribcage causing point tenderness. Compressing on the sides of the ribs simultaneously may reproduce the pain at the affected costal margin. As a temporary and localizing measure, a local anesthetic rib block with Bupivacaine can be performed which provides temporary or complete symptoms relief. Repeated anesthetics and steroid rib block, manipulative techniques, acupuncture, Botox injections, prolotherapy, and topical anesthetics produce long-lasting results. Should conservative measures fail, the management should consist of removing the slipping, disconnected cartilages through a small incision at the affected lower subcostal margin. The hypermobile cartilage is removed all the way to the costochondral junction in most cases including the perichondrium to avoid regrowth of the cartilage. During this maneuver, the intercostal neurovascular bundle is preserved. Injury to the neurovascular bundle can cause acute blood loss or chronic neuropathic pain. The use of a vertical bioabsorbable plate to stabilize the hyperflexible subluxing bony ribs used to stabilize fractures is also highly recommended to be incorporated with resection of the cartilages as recurrence rate are decreased significantly. If the patient develops recurrent symptoms after surgery, this might be due to missed slipping cartilages during the initial procedure, regrowth of the cartilage, or new symptoms on the contralateral side. Most patients are satisfied after surgical resection of the slipping ribs.          

Encapsulating Peritoneal Sclerosis

Peritoneal dialysis is the preferred chronic dialysis modality in the pediatric age. Encapsulating peritoneal sclerosis (EPS) is a rare but most serious complications of continuous ambulatory peritoneal dialysis (CAPD) in end-stage renal patients. The incidence of EPS in children receiving CAPD is 2-15% depending on the long-term use of CAPD, while the mortality exceeds 30% with most survivors requiring long-term parenteral nutrition. EPS is characterized by progressive fibrosis of the peritoneum resulting in reduced ultrafiltration capacity, dysfunctional peristalsis of the bowel, and partial or complete bowel luminal obstruction. EPS involves both the visceral and parietal peritoneum. EPS is not specific to CAPD children and can be seen secondary to drug therapy (Beta-blockers), sarcoidosis, systemic lupus erythematosus, abdominal tuberculosis, gastrointestinal malignancies, protein S deficiency and ovarian luteinized thecomas. EPS is a clinical syndrome that continuously, intermittently, or repeatedly presents with symptoms of intestinal obstruction due to adhesions of diffusely thickened peritoneum. The diagnosis of EPS is based on clinical, histologic, and radiographic findings. The pathophysiological event in the development of EPS is an inflammatory process resulting in loss of the mesothelial layer of the peritoneum and fibroconnective tissue proliferation. Fibrin deposition and fibrinolysis with hyalinization of the superficial stromal collagen possibly tanned through nonenzymatic glycosylation by the dialysate glucose plays an important role in causing excessive fibrogenesis in children with CAPD. Contributing events include duration of CAPD (single most significant risk factor), recurrent episodes of bacterial or fungal peritonitis, the acetate dialysis solution, chlorhexidine, plasticizers, and cumulative exposure to hypertonic glucose-based dialysis solutions. The rate of developing EPS increases with the years receiving CAPD. If a child receives CAPD for more than five years and shows poor ultrafiltration with peritoneal calcifications on CT-Scan, a peritoneal biopsy should be performed to rule out the presence of severe EPS. Peritoneal biopsies of EPS show loss of normal mesothelial cells, massive expansion of the submesothelial compact zone and increased vascularization, mononuclear cell infiltration, calcifications, and low peritoneal mast cell number. EPS occurs insidiously with vague presenting symptoms initially. Symptoms of EPS include weight loss, malnutrition, low-grade fever, nausea, vomiting, hemorrhagic effluent, ultrafiltration failure, ascites, and recurrent bouts of abdominal pain to subacute or acute intestinal obstruction with bowel necrosis. Imaging might show dilated small bowel loops, air-fluid levels, and calcific plaques. Ultrasound is the most sensitive modality to detect EPS demonstrating thickened bowel wall with a trilaminar appearance and adhesion of bowel loops to the anterior abdominal wall. CT-Scan demonstrates peritoneal thickened, bowel teetering, thickened bowel wall, loculated ascites, peritoneal calcification, and clouding of mesenteric fat. The cocoon appearance is secondary to the presence of a thick fibrous layer encapsulating the small bowel. Some recommendations to stop peritoneal dialysis in cases with EPS include ultrafiltration failure, bloody deacylate with calcifications of the peritoneum, duration longer than eight years of dialysis, persistency elevated C-reactive protein and recurrent peritonitis. EPS can occur up to five years after withdrawal of CAPD, when on hemodialysis, or after transplanted. These cases are all characterized by an acute presentation with a rapid clinical course. Management of EPS includes termination of peritoneal dialysis, immunosuppression, steroids (anti-inflammatory), tamoxifen (antifibrotic agent), and surgical debridement.  Surgical therapy is required when the child does not respond to medical therapy or presents with complete bowel obstruction, bowel perforation or hemoperitoneum. Total enterolysis and Noble plication are the methods suggested in the literature. Preventing EPS includes minimizing dialysate glucose exposure, preventing acute peritoneal dialysis peritonitis and using a neutral-pH solution.

Burnout Syndrome

Burnout syndrome is a psychological syndrome arising from the continued response to chronic interpersonal stressors while at work. Defined as a state of physical and mental exhaustion related to caregiving activities or work is a severe problem affecting medical personal and healthcare organizations. Emotional exhaustion and irritability in the work environment leads to development of psychiatric problems characterized by emotional exhaustion, depersonalization, and diminished personal accomplishment. The three main components of burnout are overwhelming exhaustion, feeling of cynicism or depersonalization, and a sense of ineffectiveness and lower efficacy. Burnout is the most common mental issue faced by residents and junior attending. The prevalence among residents is over 50% comparable between medical and surgical specialties. Older age and male prevalence, especially married male residents, are associated with higher prevalence. Systematic review studies have revealed that women, young surgeons, single status, increased workload, and conflict with other colleagues is associated with a higher risk of burnout. In either case, men manifested more depersonalization symptoms, whereas women report more emotional exhaustion symptoms. Women are more likely to adopt emotionally based strategies (self-blame, seeking comfort, getting help and advice from others), while men often resort to ego-defense mechanisms like depersonalization which might impact the ability to provide care. The consequences of developing burnout syndrome between physicians and residents in-training include physical illness, increase feelings of hopeless, irritability, impatience poor interpersonal relationships, risk of medical errors, reduced medical service, depression, and adverse effect in patient safety. In the case of residents, they are subjected to sleep deprivation, high workload, unsatisfactory salaries, and high responsibility. Symptoms of burnout can originate from causes such as: bureaucratic requirements, continued changing work environment, micro-management by administrations, poor clinical supervision, sensationalist media reports of medical errors, limited healthcare resources, litigious environment, and poor work-life balance. Residents and interns in urgency and surgical specialties such as general surgery, anesthesiology, obstetrics & gynecology, and orthopedics have the highest prevalence of burnout syndrome. A plausible explanation is that residents in these specialties deal with emergency routines dealing directly with life-threatening situations with overload shifts. Specialties with less burnout syndrome include otolaryngology, plastic surgery, and neurology. To deal with the problem of burnout in residencies it is necessary to recognize that the problem exists and describe its impact. By addressing burnout, you can increase your personal wellness, improve patient safety, satisfaction, and quality of life. Burnout syndrome needs evaluations of the prevalence and intensity using validated instruments such as the Maslach Burnout inventory and apply these to students, interns, residents and attending periodically. The Maslach burnout inventory toolkit asses the level of burnout by measuring: 1) emotional exhaustion caused by work, 2) depersonalization, translated into unfeeling and impersonal response toward recipients of our service, care or treatment, and 3) personal accomplishment, measure by the feeling of competence and successful achievement in our work toward patients. In addition, the work environments should be asses by using the Areas of Worklife Survey which measures workload (amount of work to be done), control (opportunity to make choices and decisions to solve problems), reward (recognition, financial and social you receive for contributing into the job), community (quality of the social context in which you work), fairness (extent to which organizations has consistency and equitable rules for everyone) and values (what matters to you in your work). Burnout is prevalent in more than half of US physicians, makes us less happy with our professional choice and less effective in our roles as clinicians, teachers, mentors, and role models. Neglected burnout leads to alcohol and substance misuse, anxiety, depression, discontinuation of residency, fatigue, impaired interpersonal and marital relationship, insomnia and even suicide. Working more than 60-80 hours per week and taking two or more nights on call per week promote burnout. Managing burnout syndrome can be divided into preventive and therapeutic measures. Actions must be concentrated on risk factors, improvement in the relationship between professionals and the promotion of healthy behavior in physicians. Therapeutic strategies consist in management of negative emotions and relaxation technique. To alleviate and prevent burnout we should fix what we control and advocate for the rest. These include aligning with patients providing high-quality care, develop expertise in disease and not technique, redesign the practice, developing progressive employment policies, and respect toward our own choices. 

PSU Volume 58 NO 06 JUNE 2022

DIC

Disseminated intravascular coagulation (DIC) is a state of hemostatic dysregulation which causes microvascular clotting and consumptive coagulopathy and is seen in a variety of conditions including sepsis (most common), trauma, malignancy, liver diseases, and toxins. These conditions can cause cytokine-induced endothelial and mononuclear cell release of tissue factors and generation of excessive thrombin extending outside the local area of injury along with release of fibrinolytic proteins. Excessive thrombi production leads to microvascular thrombi with consumption of platelets, procoagulant and anticoagulants proteins, and inhibition of fibrinolysis. All these factors contribute to multiorgan failure. Diagnosing DIC is difficult since it includes a wide range of clinical presentations, including mild to excessive bleeding and systemic thromboembolic phenomenon associated with multiorgan failure. The International Society on Thrombosis and Hemostasis (ISTH) diagnostic scoring system for overt DIC is widely utilized in intensive care units for diagnosing DIC, including sequential testing of components of the ISTH scoring system such as prothrombin time, platelet count, fibrinogen, and D-dimer. Fibrinogen does not seem to have a significant impact on the prediction of DIC. Among individual DIC components evaluated, prolonged PT is the most predictive of increased vasopressor use, followed by elevated D-dimer. Pediatric patients presenting with suspected sepsis to the emergency department who have scores greater than three are more likely to have outcomes including increased vasopressor use, increase mortality, prolonged hospital and intensive care unit lengths of stay, increased rates of mechanical ventilation, and increase mortality. The predominant condition leading to DIC is sepsis affecting 75,000 children per year in the USA with mortality rates ranging between 7010% among all age groups. Sepsis is a syndrome with a variety of clinical manifestations including organ dysfunction caused by a dysregulated host response to the inciting infection. Septic shock produces profound circulatory, cellular, and metabolic abnormalities associated with high-risk morbidity and mortality. Coagulation activation can be triggered with septic shock leading to activation and inhibition of physiological anticoagulation mechanisms and fibrinolytic system leading to intravascular fibrin formation and consumption of procoagulant leading to DIC. DIC then results in hemorrhage or thrombotic occlusion of vessels leading to inadequate blood and oxygen to various organs leading to multiple organ dysfunction syndrome. DIC results from the acceleration of the clotting cascade, inactivation of endogenous anticoagulants, and modification of fibrinolysis, leading to hypercoagulability and augmented fibrinolysis. This event causes the formation of multiple microthrombi in the systemic circulation, which consequently manifests as multiorgan failure. The main management of DIC is early identification and treatment of the underlying condition, hemodynamic support, frequent monitoring of laboratory and clinical parameters, and replacement of consumed coagulation factors and blood components via transfusion of platelets, fresh frozen plasma, or cryoprecipitate. Prophylactic transfusion of these blood products is not recommended unless there is clinical bleeding or impending invasive procedure (surgery). There is a demonstrated efficacy of antithrombin and protein C concentrates, recombinant activated protein and recombinant thrombomodulin for the management of DIC in children. Activated protein C inactivates coagulation factors V and VIII, and ultimately causes inhibition of thrombin formation and has anti-inflammatory properties. Recombinant thrombomodulin activates protein C leading to the inactivation of factor Va, which ultimately leads to the inhibition of thrombin generation.  

Extracolonic Manifestations of FAP

Familial adenomatous polyposis (FAP) is a hereditary syndrome of autosomal dominant inheritance caused by a germline mutation in the adenomatous polyposis coli (APC) tumor suppressor gene in the long arm of chromosome 5 characterized by 100-1000 adenomatous polyps in the colon and rectum with progression to cancer if left untreated. The less aggressive variant termed attenuated FAP exhibits fewer colorectal adenomatous polyps (10-100), later age of adenoma appearance and a lower cancer risk. FAP accounts for 1% of all colorectal cancers in the USA. Many of these hereditary syndromes have extracolonic manifestations, including the development of benign and malignant tumors. These extracolonic manifestations can be the first sign of the inherited syndrome helping establish an early diagnosis of FAP in high-risk patients who have not yet developed colorectal polyposis. Extracolonic cancer in FAP has become of significant concern since affected patients are living longer and are increasingly being diagnosed earlier. The majority of FAP patients (over 70%) present with some level of extracolonic manifestation during the course of the disease. Extracolonic manifestation can be classified according to the tissue of origin into ectodermal, endodermal, and mesodermal. Ectodermal lesions include epidermoid or sebaceous cysts which occur with increasing frequency at a younger age with predilection in the face, scalp, legs, and arms. Also, congenital hypertrophy of the retinal pigment is a well-recognized ocular sign and clinical marker occurring in more than 90% of FAP patients. Ocular lesions are discrete, darkly pigmented, round, oval or kidney shape raging in size from 0.1-1.0 optic disk diameter. Lesions of mesodermal origin include desmoid disease, lipomas, fibromas, osteomas, and dental abnormalities such as odontomas, dentigerous cysts and supernumerary teeth. Dentists should consider referral of patients with extranumerary teeth or jaw osteomas for FAP evaluation. Desmoid tumors are slow growing mesenchymal neoplasms composed of fibroblasts and myofibroblasts within a rich collagen matrix. Desmoid tumors are most commonly (80%) intraabdominal (mesentery of retroperitoneum). Other sites include abdominal wall, subcutaneous, and in the musculo-aponeurotic layer. They can reach massive size, cause intestinal obstruction, mesenteric vascular obstruction, and ureteric obstruction. They lack a metastatic potential but exhibit aggressive local behavior with infiltrative patterns of growth involving surrounding structures and a high local recurrence rate following resection. Desmoid tumor development can be exacerbated by surgical trauma or pregnancy. The frequency of developing desmoid tumors is higher for FAP with a lifetime risk of 8% for males and 15% for females. Management of desmoid tumors is complex and depends on location, symptoms, extent of disease and pattern of growth. Radiation therapy, NSAID's, antiestrogen and chemotherapeutic are options. Surgery is used for those unresponsive to medical treatment or if complications requiring emergency surgery occur. Recently, Imatinib has been shown to positively impact progression-free survival in patients with advance desmoids. Osteomas may occur in the mandible, maxilla, sinuses or calvarium of the skull. Exostosis may be found in the skull, digits, and long bones. Lesions of endodermal origin include gastrointestinal adenomas and carcinomas. After the colon and rectum, the duodenum is the second most common site of polyp development in patients with FAP. Non-adenomatous fundic gland polyps predominate in the stomach, while duodenal lesions were mostly adenomatous in nature. The most common extracolonic manifestations with FAP are upper gastrointestinal polyps, including gastroduodenal adenomas that can progress to cancer. Upper GI polyps in the setting of FAP are located in the stomach, duodenum and periampullary region. Gastric polyps are typically non-adenomatous benign fundic gland polyps considered hamartomas. They develop in almost 50% of FAP patients, are located in the antrum, and are not associated with cancer. Duodenal adenomatous polyps in FAP are found in 30-70% of individuals, have a predilection for the second and third portion of the duodenum, have a strong propensity toward developing into duodenal cancer and a genotypic/phenotypic correlation with mutation in exon 15 of the APC gene has been described. Duodenal cancer is the second most common cause of disease-related mortality in patients with FAP with a lifetime risk of approximately 3-5%. Is one of the leading causes of death in patients who have undergone prophylactic colectomy. Increasing age appears to be correlated with higher risk of progression to advanced polyposis. Endoscopic surveillance is recommended starting between age 25 and 30 or 5 years earlier than any affected family member with duodenal adenomatous polyps. The Spigelman classification is used to evaluate polyp severity based on number, size, histology, and presence of dysplasia. Management of duodenal polyps consists of pharmacologic (non-steroidal inflammatory drugs) which has been found ambiguous, endoscopic, or surgical removal. Among FAP patients the risk of death is higher than in the general population (3-fold); disease related mortality is caused more commonly by upper gastrointestinal malignancies followed by desmoid tumors and perioperative complications. Other less common extraintestinal malignancies associated with FAP include thyroid, brain (medulloblastoma), adrenal, hepatoblastoma and pancreatobiliary tumors. Thyroid cancer is the third most common malignancy associated with FAP. Is most commonly papillary histology, presents in the second or third decades of life, mostly in females. The cribriform-morular histologic variant of papillary thyroid cancer is highly suggestive of FAP. Periodic thyroid ultrasound screening should be considered in patients with FAP, and FNA in those with thyroid nodules. The risk of pancreatic adenocarcinoma is also increased in patients with FAP. Mutations in the APC gene are associated with several other extraintestinal manifestations including Turcot and Gardner syndromes. Gardner's syndrome is characterized by the typical manifestations of FAP and presence of osteomas, fibromas, and epidermoid cysts.

Delayed Separation Umbilical Cord

The umbilical cord connects the fetus to the placenta in the uterus, is made of blood vessels (two arteries and one vein), and connective tissue. The umbilical cord of newborn infants separates during the first two weeks of life, though there is a wide variation in the time this event takes place with regard to ethnicity, geographical location and proper care of the cord. The mean umbilical separation time is 6.6 days, with a median of seven days. The cord stump dries and falls off, and the wound heals. The cord separation is mediated by leukocytes. Histologically this process is characterized by granulocyte influx and phagocytosis at the base of the cord. Delayed separation of the umbilical cord is defined as separation that occurs after three weeks of life. Factors associated with delayed separation of the cord include the use of alcohol or chlorhexidine for cleaning purpose, prematurity, and infants born by cesarean section. Neonates delivered by cesarean section tend to have longer cord separation time due to less bacterial colonization after birth. The use of postpartum antibiotics, parenteral nutrition and phototherapy also delayed the separation of the umbilical cord. Sepsis delays the cord separation time by sixfold beyond the second week of life. Urachal anomalies rarely can be associated with delayed separation. According to the National Institute of Health Care and Excellence guidelines, parents should be advised how to keep the umbilical cord clean and dry, and that antiseptics should not be used routinely. A marked delayed in umbilical cord separation raises the suspicion of leukocyte adhesion deficiency (LAD), a rare autosomal recessive hereditary disorder leading to defective neutrophil function. LAD is a disorder of neutrophils due to a genetic defect in the beta subunit of the integrin molecule ITGB2 which encodes the integrin beta chain-2 protein CD18. This defect leads to dysfunction of leukocyte adhesion to the wall of blood vessels and migration of leukocytes to sites of infection and inflammation. Patients have a complete absence of neutrophils at the site of inflammation causing recurrent bacterial infections and sepsis. LAD is characterized by recurrent infections, impaired pus formation, delayed wound healing, omphalitis, and delayed separation of the umbilical cord as hallmark features of the disease. Patients are infected with common pathogenic agents but not opportunistic ones and respond well to antimicrobial therapy. Proteus, Klebsiella, Staphylococcus aureus, Pseudomonas aeruginosa, and enterococci are the most common pathogens affecting LAD patients. The severe form of this disease remains a life-threatening condition with limited 2-years survival in the absence of transplantation. The flow cytometric analysis of monocytic intracellular tumor necrosis factor-alpha production in response to lipopolysaccharide may be a useful method to screen for the disease. Management of LAD consists of allogeneic stem cell or bone marrow transplantation.