PSU Volume 52 NO 01 JANUARY 2019

Ventriculo-Pleural Shunts

Shunting of cerebrospinal fluid (CSF) into extracranial sites usually include the use of the peritoneum (ventriculo-peritoneal shunt) and atrium (ventriculo-atrial shunt) for palliation of symptoms associated with hydrocephalus. Another alternative includes the use of the pleura for absorption of the CSF using a ventriculo-pleural shunt in selected patient when conventional sites  not suitable either due to adhesions, infection, thrombosis or obliteration. Ventriculo-pleural shunts are an acceptable alternative for CSF decompression in children as well among adults patients. It is estimated that up to 50% of children will experience a failure of any shunt within the first year. Clinical manifestations of early shunt failure include nausea, vomiting, irritability, altered consciousness, bulging fontanelle among infants. Depress level of consciousness and loss of milestone are the main indication of late shunt failure. CSF shunts complications are either mechanical, functional or infectious in nature. Mechanical complications include obstruction of the shunt, fracture or disconnection of the device and migration. In-growth of portions of the choroid plexus or ependymal surface of the ventricles into the inlet holes of the proximal catheter accounts for the most common cause of obstruction. CSF malabsorption leads to abnormal accumulation of the fluid resulting in functional failure of the shunt. The most common complication of ventriculo-pleural shunts (VPLS) is pleural effusion. Only 20% of the pleural effusions are symptomatic requiring revision and most of these patients are infants. Anti-siphon devices seem to reduce the incidence of pleural effusion. The hydrothorax associated with VPLS is due to impaired pleural absorption and excessive drainage of CSF into the pleural space. The fluid is clear transudate with some mononuclear cells. In most cases of pleural effusion the fluid resolves spontaneously. Pleural CSF effusion might result in respiratory distress needing thoracentesis. Administration of acetazolamide reduces CSF production and reduces respiratory symptoms. With recurrent or worsening of symptom shunt replacement must be undertaken.

PET Scan Utility in Thyroid Cancer

Thyroid cancer in children carries a good prognosis. It is followed after total thyroidectomy with serial measurement of serum thyroglobulin (Tg), antithyroglobulin antibody (TgAb), neck ultrasound and radio-iodine (RAI) whole body scan. Elevated thyroglobulin with normal antithyroglobulin autoantibodies after surgery and radioiodine ablation therapy indicates recurrent thyroid carcinoma or metastatic disease. RAI scan is more than 90% specific for detecting recurrence or metastatic disease. 30-50% with recurrent/metastatic disease can have a negative RAI scan specially small size tumors or poorly differentiated carcinomas. PET Scan using fluorine-18-fluorodeoxyglucose (FDG) is an effective tumor imaging tool in thyroid cancer patients with high Tg levels specially in cases where RAI whole body scan cannot detect metastasis due to lack of accumulation by the tumor. Other potential indications for PET Scan are locating disease in positive RAI whole body scan patients looking for other sites of metastasis, high level of antithyroglobulin and determination of prognosis. Thyroid differentiated cancer cells express the sodium iodide symporter and concentrate radioiodine. As the disease becomes more aggressive, they lose that symporter ability making RAI whole body scan negative (non-functioning metastasis) but the remnant tumor/metastasis still secreting Tg. Due to the increase growth rate these cells are detected by PET 18-FDG Scan and are more amenable to surgical extirpation if identified. While this process of dedifferentiation between the tumor cells occurs, patients can have both RAI whole body scan and PET Scan with positive disease in different anatomical areas of the body. Combining both localizing studies increase the sensitivity of finding tumor to 94%. The tall cell variant of thyroid differentiated cancer associated with a poor prognosis is associated with a positive PET Scan more frequent that the classic cell type. PET Scans are more sensitive in patients with low TSH levels. In cases of high TgAb with negative RAI scan the Tg level even if normal carries a clinical problem of interpretation and PET Scan can detect recurrence/metastatic disease. Preoperative PET Scan is not useful for prediction of thyroid cancer recurrence. Lymph node stage is the only predictor of recurrence.

Acute Ileitis

The terminal ileum is the most distal segments of the small bowel and hosts many toxic substances including bacteria, viruses, parasites and digested food. The term acute ileitis describes episodes of right lower quadrant abdominal pain not identified as acute appendicitis but participation of the terminal ileum is identified or suspected. It occurs in 33 cases per 100000 presenting to the ER. The term ileitis became famous with the recognition of Crohn's disease terminal ileitis. The diagnosis of acute ileitis is suspected or established with US and/or CT-Scan. CT-Scan is needed when the child has atypical clinical findings, equivocal sonographic results or a pediatric surgeon ask for the study. The causes of acute ileitis include infectious (most common cause), inflammatory bowel disease and nonsteroidal antiinflammatory drug therapy. The most common infectious microorganisms causing ileitis include Yersinia enterocolitica (most common frequent organisms), Campylobacter jejuni, Salmonella enteritides and Anisakis simplex. They start with colicky right lower pain followed later by fever and diarrhea. Stool cultures are false negative. Anisakis infestation is associated with eating raw, marinated or smoked fish 48-72 hours before the onset of symptoms. One third of the patients with Crohn suffer from an acute episode of ileitis. Suspicion of Crohn's ileitis should arise on a combination of clinical and biochemical findings, imaging data, absence of alterative diagnosis and history of preceding episodes. With chronic symptoms of pain and diarrhea an ileal endoscopic biopsy is warranted. Exposure to nonsteroidal antiinflammatory drugs can induce ileitis. These lesions respond to discontinuation of the drugs that its causing it. Almost 20% of acute ileitis cases are nonspecific and a specific cause cannot be determined. In females gynecological conditions (uterine disorders, ovarian pathology and pelvic inflammatory disease) can mimic the symptoms of acute ileitis. US is the initial preferred imaging study in young females to avoid irradiating the pelvic organs. 

PSU Volume 52 No 02 FEBRUARY 2019

Pediatric Breast Cancer

Breast masses in female children are mainly benign with fibroadenoma being the most common diagnosis.  Breast cancer in the pediatric age is very rare occurring in 0.1% of all breast cancers with an estimated incidence of one case for every 125000 females under the age of 21 years. When breast cancer occurs in the pediatric age, the child presents with late stage disease, more nodal metastasis and lymphovascular invasion and few to none hormone receptor positive tumor. Breast cancer is the most common cancer in females between 15 and 40 years of age. The median age in the pediatric group with breast cancer is 17 years with 80% of cases in nonblack patients. Common symptoms include a lump, changing size of the breast, dimple of the skin, inverted nipple, and red or swollen skin changes in the breast. US and MRI suggest the presence of a malignancy and biopsy confirms the diagnosis. Suspicious lesions of any size with atypical findings on ultrasound or MRI (cystic areas, hyperlobulation, angularity, calcifications or irregular margins) or masses above 4 cm should undergo core or excisional biopsy. The most common histology in order of frequency is breast adenocarcinoma (50%), fibroepithelial/phylloides tumors (35%) and sarcomas (15%). Most cases undergo surgery as management of the tumor including modified mastectomy. Adenocarcinomas and sarcoma carry a worse survival when compared with fibroepithelial tumor. By stage fibroepithelial tumors is almost all early stage disease, whereas adenocarcinomas and sarcomas have both early and advanced stage tumors. High grade and advanced stage disease has a worse outcome than low grade and early stage disease. Fibroepithelial tumors are nearly as twice as common in black patients when compared to nonblack patients. This is probably caused by an early menarche age in black females with longer exposure to estrogen. Fibroepithelial tumors arise from the specialized connective tissue around mammary lobules and are often large (> 6 cm) estrogen-sensitive tumors with a high risk of local recurrence. Almost 20% of pediatric breast cancer dies of their disease.

Dog Bites Injury

More than 4.5 million dog bites occur in the USA annually requiring hospitalization in more than 80% of them. It is estimated that 60 million's dollars are spent annually for dog-related hospitalizations. Children are a specially vulnerable population at higher risk for sustaining dog bite injuries. The most common circumstance for injury involved a child playing with or while petting a dog. This benign initial encounter should be highly monitored during early interactions between a dog and child. Food-related encounters like a dog protecting its food or a child eating near a dog are common circumstances of injury. Dog bites leads to life and limb threatening infections in children due to the ischemic nature of tissue loss and mixed bacterial contamination. Infants are more than four times likely to be bitten by the family dog and most of these occurs in the head, face and neck region. Preschool children dog bites accounts for more than half of the injured population.  Most dog bites can be repaired in the emergency room with primary closure. Complex lacerations, avulsions and shearing injuries will need repair at the operating room. Pit bulls are the most common breed to inflict  dog bite injury requiring direct and reconstructive repair. Children between ages nine and 12 years are the most common victims of pit bulls attacks. Following pit bulls, Labrador retriever, German Shepard and Husky follow in as much as attacking dog breed. Location of dog bites in children is usually the head and face region due to child short stature, larger head circumference and underdeveloped evasive motor skills to protect the face. Simple puncture wounds and abrasions can be managed with wound irrigation and application of nonadherent dressing. Simple lacerations can be sutured in the emergency room under local sedation and analgesics. Complex lacerations, partial flap avulsions or complete full-thickness tissue loss requires operative repair and hospitalization. Broad-spectrum antibiotic coverage is recommended for all dog bite wounds that require operative intervention. Extended hospitalization is usually associated with local wound infection. Dog owners should be held legally and financially responsible for stray dogs that place people, particularly young children at risk for body harm.

Gilbert Syndrome

Gilbert syndrome (GS) is considered the most common inherited disorder of bilirubin metabolism due to reduction of uridine diphosphate-glucuronyl transferase 1A1 activity. The hepatic bilirubin glucurodination activity is approximately 50% lower than normal. It is characterized by asymptomatic unconjugated hyperbilirubinemia in the absence of liver disease or hemolysis. GS is mild and benign condition inherited as autosomal recessive that affect being either homozygotes or compound heterozygotes (two different recessive alleles at a particular locus). It may be precipitated by dehydration, fasting, menstruation, overexertion or stress due to intercurrent febrile illness or even vigorous exercise. Other than experiencing jaundice patients are typically asymptomatic. GS usually manifests itself after puberty, affects 3-7% of the population with male predominance. Children may report vague abdominal discomfort, general fatigue and malaise without evident cause. Caloric restriction raises the serum unconjugated bilirubin lever twofold in affected patients with Gilbert syndrome. Fasting, caloric restriction and the intravenous nicotinic acid (niacin) provocative test has been used to diagnosed GS. The oral rifampin test induces cytochrome P-450 isoenzymes and competes for the excretory pathways in the liver at the cellular level causing an exaggerated elevation in unconjugated total serum bilirubin level in GS. Liver biopsy is not indicated and is usually normal. Genetic testing for the A(TA)7TAA variant and Gly71Arg mutation is diagnostic of Gilbert syndrome. GS can present in the newborn period if there is concurrent hemolytic disease such as ABO incompatibility, hereditary spherocytosis or G6PD deficiency. GS has been found to be a precipitating factor for idiopathic cholelithiasis in children. There is no specific treatment except to avoid fasting and severe stress. The prognosis overall is excellent.   

PSU Volume 52 NO 0 MARCH 2019

Vanishing Testicular Syndrome

Testicular regression syndrome, also known as Vanishing testicular syndrome (VTS), refers to the disappearance of a normally developed testis during fetal life. The spermatic cord ends blindly as evidence of a testis that was present but disappeared. VTS occur usually unilateral the consequence of late antenatal or perinatal vascular thrombosis, torsion or infarction seen in less than 5% of undescended testes. The testis is non-palpable in almost 20% of cases of undescended testes in children and of these 50% are directly caused by VTS. VTS is more common than testicular agenesis. Due to earlier normal descent of the left testis anatomically this site is affected more commonly then the right testis. Most VTS are sporadic events while a few are associated with severe mental retardation or members of the same family. The clinical presentation is an empty scrotum with non-palpable testes in the inguinal canal or elsewhere. Once a child is found with a non-palpable undescended testis a diagnostic laparoscopy is in order to find an intraabdominal testis. When vas deferens and spermatic vessels are visualized exiting the internal inguinal ring on laparoscopy a groin exploration is usually carried out to identify and remove the testicular tissue remnant (nubbin) associated with the vanishing testis. Fibrosis, dystrophic calcification, giant cell reaction and hemosiderin deposition in association with identifiable testicular structure are features of VTS nubbin. Histopathological examination has confirmed the presence of germ cells in 10% of VTS testicular nubbin remnants. The pathological diagnosis of VTS can be established by the presence of the following diagnostic criteria: 1) a vascularized fibrous nodule with calcification and/or hemosiderin, and 2) a minimum of a vascularized fibrous nodule with cord elements in proximity. Intraabdominal testes are six times more likely to develop a testis malignant tumor than the palpable undescended testis in the inguinal canal. There is controversy whether to remove the nubbin from the VTS due to the low rate of viable germ cell tissue and almost negligible incidence of malignant transformation.    

Leiomyoma

Leiomyoma is a benign tumor usually encountered in the genitourinary (uterus) and gastrointestinal tract (small bowel and esophagus) in adults. Leiomyomas are slow growing tumors that can occur in any body part where smooth muscle is present. It is a very rare tumor in children. The skin is the second most common location for leiomyomas after the uterus. Depending on the site of origin leiomyomas can be classified into three types: piloleiomyoma (cutaneous), angioleiomyomas (vascular) and dartoic leiomyomas (deep soft tissue). Piloleiomyomas originate from the arrector pili muscles of the hair follicles. Angioleiomyomas come from the vascular smooth muscle, while dartoic leiomyomas originate from the smooth muscle of genital skin. Leiomyomas may present clinically as either solitary or multiple lesions. In the skin they can have a red-surface and most commonly located in the extremity. Piloleiomyomas are the most common type of leiomyoma in the skin ranging in diameter from two to 20 mm and they are typically painful. The pain may be spontaneous or the result of exposure to cold, pressure, emotional stress or growth of the tumor compressing surrounding nerve tissues. Piloleiomyoma are non-encapsulated circumscribed dermal tumors composed of multiple fascicles of smooth muscle in an interlacing whorled arrangement. Calcifications have been reported in isolated deep tissue leiomyomas. Differential diagnosis includes osteochondroma, desmoid tumor, spindle cell carcinoma and other soft tissue tumors from the adjacent region. Although leiomyomas are benign they have a low but definite malignant potential and should be removed. Atypical cells, necrosis and mitotic activity must be interpreted as a warning sign in terms of malignant transformation and post-surgical recurrence. Definitive diagnosis is established after surgical removal. The management of leiomyomas is surgical excision whenever the anatomic location permit. The prognosis is good for patients after complete surgical resection.    

Fracture CVC

As medicine advance and more invasive treatment options are available the number of children needing long-term central venous access increases. Long term venous access can be achieved using central venous catheters (CVC) such as Hickman, Broviac, percutaneous inserted central catheter (PICC), subcutaneous implantable ports or umbilical catheters. Most CVC are safe but complications do occur. Most complications can be categorized as infectious, thrombotic or mechanical. Fracture central venous catheter is a serious mechanical complication that occurs rarely. CVC fracture can be defined as a break in the sterility of the line that allows leakage as a result of a tear, hole, or shearing injury in the external line tubing. These fractures occur more frequently in peripherally inserted catheters. Factors that predispose CVC to break are related to the characteristic of the catheter, the insertion or removal technique, and the clinical problem for which they were used. Silicone catheters are prone to fracture at or near the entrance site where the caliber of the line narrows. Catheter damage may be caused by the introducer needle in PICC lines. Some catheters might rupture in the axillary area due to repeated stress caused by flexion and extension of the arm. Repeated compression of the catheter between the clavicle and first rib in the costoclavicular ligament area can lead to fracture of the CVC (Pinch off syndrome). If the catheter is occluded and high pressure infusion is utilized the catheter can rupture. Two primary methods are utilized to manage CVC fracture: line replacement or line repair. CVC comes with repair kit. The incidence of infection is not increased after repairing the fracture catheter. Line repair can be performed safely at the bedside. The major drawback of CVC repair over replacement is decreased durability of the catheter. Risk of leaving catheter fragments in the child body can include pulmonary embolism, sepsis, endocarditis, arrhythmias and cardiac perforation. Due to the risks associated with fragmented catheters they should be removed. This is performed using a percutaneous endovascular approach.

PSU Volume 52 NO 04 APRIL 2019

Grave's Disease and Urticaria

Chronic urticaria is a common clinical condition associated with itchy wheals and flare-hive skin reactions for more than six weeks with unknown etiology in more than 75% of patients. It is believed to be an autoimmune mediated condition with antibodies against the alpha subunit of the IgE receptor and IgG. Chronic urticaria can coexist with autoimmune thyroid diseases such as Hashimoto thyroiditis and Graves disease as a link has been reported several years ago. Hypothyroidism and Hashimoto's thyroiditis are more commonly associated than hyperthyroidism and Graves' disease. A reaction against a common antigen shared by the involved tissues, autoantibodies targeting similar epitopes or a cross-reaction between autoantibodies may be the origin of the problem in the development of both diseases. As thyroid function normalized with medical therapy (methimazole) toward Graves disease, the urticaria also improves. The amelioration of the urticarial symptoms observed may be the result of the reduction in sweating, itching and heat intolerance experienced by the patient when hyperthyroidism is corrected. Thyroid dysfunction is more common in adult patients with chronic urticaria than in children, and in females than in male patients with urticaria. The management of Graves hyperthyrotoxicosis with or without chronic urticaria in children includes medication (methimazole, propylthiouracil) or surgery (total thyroidectomy). The use of radioiodine therapy in children for managing Graves disease is indicated if the child has a contraindication for surgical management or poor medical management. Prompt treatment of the hyperthyroidism significantly improves the urticaria in the child. Screening for thyroid autoimmunity and function is advisable in all patients with chronic urticaria for the early identification of patients requiring either treatment of underlying thyroid dysfunction or follow-up.

Acute Scrotal Edema

Acute scrotal edema is a self-limited disease of unknown etiology characterized by edema and erythema of the scrotum and the dartos fascia without affecting the underlying layers of the scrotum, testicle or vas deferens. It is estimated to occur in almost 20% of all acute scrotal swelling in children. The condition is characterized by rapid onset and development of significant edema and erythema without tenderness or fever. It occurs between the four months and 18 years of age. Children are asymptomatic or complain of minimal scrotal discomfort. Palpation discovers a nontender testis. Affected scrotum is edematous and erythematous appearing from pink to violaceous. It can be unilateral (90%) or bilateral. Involvement of the groin, perineum and low abdomen is commonly observed. It is believed to be an allergic reaction as there is eosinophilia of 2-4% present and a response to antihistamines is occasional obtained. Some children have an associated allergic disorder such as asthma, eczema or dermatitis, though no allergen has been identified. Labs (WBC, urinalysis, urine culture), urethrogram, cystoscopy, and testicular biopsy are usually normal. Ultrasound findings include marked echogenic thickening of the skin and muscles of the scrotum with slightly increased blood flow to the scrotum on color Doppler. Color Doppler documents the equal arterial blood supply to both testis by the anterior and posterior scrotal artery along with scrotal hyperemia consistent with fountain's sign. Differential diagnosis includes testicular torsion, appendage torsion, epididymitis, trauma, incarcerated inguinal hernia, testicular tumor, cellulitis, and vasculitis. The duration of acute scrotal edema manifestation usually ranges from 6 to 72 hours. Management of acute scrotal edema is non-surgical as the condition resolves spontaneously. Depending on the clinical condition analgesia, nonsteroidal antiinflammatory drugs and antihistamines or antibiotics have been utilized, but mainstem of treatment is observation and reassurance, activity restriction, and scrotal support. Relapse can occur several months or even years after the initial event.

Longboard Injury

Since the 1960's longboarding has gained significant popularity along with the associated injuries that such a sport carries in our young people. Longboards are generally 42 to even 80 inches in length compared with regular skateboards that are 30 to 38 inches long. Longboards are perfect for street cruising, allowing for greater travel, higher speed, downhill cruising and carving. Longboarding lends itself to more severe injury than regular skateboard injury. Compared with skateboard injury, longboard patients are significantly older and female. The mean age of patients with longboard injury is 19.8 years with 58% of injuries in adolescents (10 to 16 years) and around 8% in children younger than 10. Dermal injuries and fractures are the most common type of injury followed by traumatic brain injury. Extremity fractures specially those of the clavicles are more commonly seen in longboard injury. Head fracture, traumatic brain injury and intracranial hemorrhage is also more common among longboard patients. In fact traumatic brain injury is the most serious consequence and most common injury pattern associated with longboarding trauma. Almost 10% of children with longboard injury will need an operation, primarily orthopedic in nature, followed by neurosurgical. The use of a helmet while longboarding is 10%. There is increase recommendation to use safety helmets during longboarding to reduce the severity and incidence of head injury. Almost 50% of children using longboard do not wear a safety helmet. It is estimated that 6% of the population using longboarding might sustain an injury described above. The use of a safety helmet should be promoted by longboard professionals, local longboard shops, promotional events and discounted or free distribution of helmet to reduce significantly the incidence of head injury. Use of safety helmets during skate and longboarding should be introduced as law to reduce the incidence of brain injury.

PSU Volume 52 NO 05 MAY 2019

Rapidly Involuting Congenital Hemangioma

Congenital hemangiomas are benign vascular tumors that have proliferated in utero, grown to their maximum size at birth and do not exhibit accelerated postnatal growth. They are further subdivided into: rapidly involuting congenital hemangioma, non-involuting congenital hemangiomas and partially involuting congenital hemangioma. Congenital hemangiomas are usually cutaneous but can be found in the viscera like the liver. Complications include hemorrhage, transient heart failure and transient coagulopathy. Rapidly involuting congenital hemangiomas (RICH) are large high-flow lesions that are completely formed at birth but rapidly involute by 12 to 15 months of age. RICH lesions are rare compared with other types of hemangiomas and tend to be located on the head, trunk and arms. The diagnosis of RICH is based on its clinical history of rapidly involution. MRI will show high intensity flow with areas of heterogeneity. Angiography reveals an arterial aneurysm with large and irregular feeding arteries in a disorganized pattern, large dilated draining veins direct arteriovenous shunts and intravascular thrombi. Biopsy histopathology will show that the RICH lesions do not express glucose transporter-1 protein. RICH usually resolves by the age of 14 months while non-involuting congenital hemangiomas continue to grow and often need to be surgically excised. Any rapidly growing mass that is firm on palpation, show signs of ulceration and has an atypical appearance should be considered for biopsy to rule out malignancy. A singular hepatic vascular lesion in infancy is almost always a rapidly involuting congenital hemangioma. RICH lesions do not respond to propranolol or any other medication. Thrombocytopenia is typical of RICH not due to Kasabach-Merritt phenomenon but due to central thrombosis in the lesion occurring after birth with transition from fetal to postnatal portomesenteric circulation. It is self limited within the first few weeks. Management of RICH lesions consists of observation during the involuting phase. Surgical excision or embolization is indicated for persistent ulceration, hemodynamic instability, thrombocytopenia or bleeding that does not respond to medical therapy. 

Axillary Lymphangiomas

Lymphangiomas are congenital malformations of the lymphatic system composed of single or multiple macro- or microcystic lesions with almost no communication with lymphatic system or drainage to the venous system. They are classified as capillary, cavernous or cystic. Microcystic lymphangiomas measure less than 2 mm in size, while macrocystic are more than 2 cm. The most common anatomic locations of lymphangiomas are cervicofacial (75%), followed by axilla (20%), mediastinum, groin and abdominal cavity. Lymphangiomas are painless cystic masses. Lymphangiomas usually present early in life or develop later, even in adult years. Axillary lymphangiomas present as painless swelling which is soft, compressible, nontender and transilluminate. Differential diagnosis includes lipoma, neurofibroma, hematoma, and dermoid cyst. It is difficult to get complete excision of axillary lymphangiomas without disrupting breast tissue in females. Axillary lymphangiomas can be complicated by infection associated to a respiratory tract infection. The lesion develops redness, pain and tenderness sometimes requiring incision and drainage. They can also develop spontaneous bleeding into the cystic cavity. Ultrasound is the initial imaging performed for diagnosing axillary lymphangiomas as they are seen as anechoic cavities with septa and debris. CT or MRI are better diagnostic tools in determining the relation of vessels and nerves with the axillary lymphangioma if surgical excision is planned. Complete surgical excision is the gold standard for management of all types of lymphangiomas if the anatomic location permit. The association with vital vascular and neural structures may preclude complete excision of lymphangiomas in neck, chest, axilla and retroperitoneum. Lymphangiomas can also be managed with sclerotherapy (OK-432, Bleomycin, doxycycline, acetic acid, alcohol and hypertonic saline), especially if they are macrocystic. Propranolol downregulate the Raf mitogen activated protein kinase signaling pathway reducing expression of vascular endothelial growth factor and decreasing lymphangiomas size in 30% of patients.      

Ectopic Breast

Ectopic (accessory) breast tissue can occur anywhere along what is known as the milk line from the axilla to the groin. May occurs unilaterally or bilaterally. The incidence of ectopic breast tissue is 1-6% in the general population. Axillary breast tissue is a subtype of ectopic breast that is found on 2-5% of women. Ectopic breast tissue has also been reported on the face, perineum and vulva. The same diseases that affect the normal positioned breast tissue occur in ectopic breast tissue, namely cysts, benign tumors such as fibroadenomas, inflammatory (mastitis), and neoplastic conditions (phyllodes and carcinoma). Breast tissue normally develops from the embryonic ectodermal thickening extending from the axilla to the groin region. It develops in the pectoral region and the rest of the milk line undergoing regression. Failure of such regression give rise to supernumerary breast (polythelia or polymastia). Ectopic breast tissue increases in size during puberty, pregnancy, birth and lactation due to hormonal stimulation. Native American women have a higher incidence of accessory breast compared with non-native American. The differential diagnosis in the axillary region would include lymphadenopathy, epidermal cyst or lipoma. Malignant transformation can occur in ectopic breast tissue mentioned above. The possibility of future complications in ectopic breast and axillary breast tissue along with the aesthetic and psychological involvement are the main reason for offering surgical excision of the accessory gland to affected patients. Axillary tail of Spence is axillary accessory breast tissue that is connected with outer part of normal thoracic breast tissue and is located deep. Diagnosis can be suggested using ultrasound and confirmed with fine-needle aspiration biopsy. Conservatory management (observation) is an option, though not commonly used among affected women.

PSU Volume 52 No 06 JUNE 2019                    

Laparoscopic Bladder Injury

Laparoscopy is a standard technique utilized in many surgical procedures proving itself important in as much as convalescence, less postoperative pain, better cosmesis and less hospital stay refers. Inserting trocar cannulas into the abdominal cavity can cause iatrogenic injury to intraabdominal organs, namely, blood vessels, viscera and bladder. Bladder injury during laparoscopy is very rare occurring with an estimated incidence of 0.5% of all general surgery laparoscopic procedures. The risk is increased in children due to smaller operative field. Most cases of bladder injury occur during emergency procedures performed in and toward the pelvis. Laparoscopic appendectomy is the procedure most commonly associated with bladder injury in children. Hollow organs should be decompressed to minimize damage to these structures during laparoscopy. Placement of a bladder catheter (Foley) during pelvic procedures, including appendectomy, reduces but does not eliminate the possibility of causing injury to the bladder. Bladder injury occurs during suprapubic trocar placement (most commonly) or while dealing with an inflammatory procedure near the bladder. Bladder injury can also occur if a urachal remnant is injured during suprapubic trocar insertion. In the immediate postop period a child with laparoscopic bladder injury will show suprapubic pain, hematuria, urinary retention, urinary leakage from wound sites, cystitis or even a subtle rise in creatinine. The systemic inflammatory response can include suprapubic cellulitis and crepitation from an underlying infection if the injury is delayed. Most trocar injuries produce a through and through perforation of the bladder tangentially or including two holes. The diagnosis of bladder injury is established with contrast cystogram. Findings could be of extraperitoneal or intraperitoneal bladder injury. Extraperitoneal injury can be managed conservatively with bladder drainage and antibiotics. Intraperitoneal bladder injury needs operative repair.     

Thyroid Lymphoma

Lymphoma arising primarily from the thyroid gland is a very uncommon malignancy comprising less than 5% of all thyroid cancers and less than 2% of extranodal lymphomas. Non-Hodgkin lymphoma of the thyroid gland has a female predominance. Thyroid lymphoma can be confused with anaplastic thyroid cancer in as much as both have rapid growth associated with dyspnea, dysphagia, pain, stridor, coughing, choking and hoarseness of voice. Most cases are euthyroid. Many cases of thyroid lymphoma have a ten-year prior history of Hashimoto thyroiditis. Large cell lymphoma probably evolves from persistent low-grade mucosa-associated lymphoid tissue (MALT) malignant lymphoma. Fine needle aspiration cytology has a limited role in diagnosing thyroid lymphoma since the cytological differentiation from lymphocytic thyroiditis and anaplastic carcinoma is difficult. A core needle or open biopsy is usually required to diagnose thyroid lymphoma. Thyroid lymphoma can be confused with anaplastic carcinoma needing differentiation using immunohistochemical assays with antibodies to cytokeratin and leukocyte common antigens. Most thyroid lymphomas are of B-cell origin predominantly diffuse large cell type. This does not means that Hodgkin's, Burkitt, plasmacytoma and T-cell lymphoma have also been reported in a lesser scale. Management of thyroid lymphoma is based in the histologic subtype, stage of disease and tumor bulk. In general treatment includes a combination of monoclonal antibodies, chemotherapy and radiotherapy. The presence of histopathological features of MALT constitutes a favorable prognostic factor for high grade lymphomas with five years survival of 90%. Lymphomas of non-MALT origin have a poorer prognosis with 5 year survival of less than 50%. Factors predicting worse prognosis include tumor size over 10 cm, advance stage, obstructive local symptoms, rapid tumor growth and mediastinal involvement. With this regimen of management the need for debulking and thyroidectomy has been reduced in this condition unless the patient has airway compromise. Secondary thyroid lymphoma originates from a disseminated non-thyroidal neoplasia that metastasize to the thyroid gland with widespread disease and higher mortality rates. 

Malfunctioning Peritoneal Dialysis

Dialysis using the peritoneal surface is an effective temporary therapy used widely in children and adults affected with end stage renal disease. A viable catheter allowing adequate inflow and outflow of dialysate fluid is essential for continuous peritoneal dialysis therapy to be successful. Malfunctioning of the peritoneal catheter is a common complication encountered during peritoneal Dialysis. Malfunctioning might occur due to kinking, catheter migration out of the pelvis, malpositioning of the tip, and obstruction of the catheter due to debris or fibrin deposition, blood clots, omental wrapping or intraperitoneal adhesions. The incidence of malfunctioning peritoneal catheters used for dialysis can occur in more than 50% of patients. Catheter related problems are blamed for up to 20% of patient transfers to hemodialysis. When there is obstruction to flow of dialysate fluid through the catheter some non-surgical maneuvers that can be accomplished to salvage the situation include infusion of urokinase to lyse the fibrin clot, forced flushing the catheter, the use of metal guidewire or Fogarty catheters to manipulate and clear the way to flow. Should this simple maneuvers failed to restore the catheter flow then surgical intervention is warranted. This usually requires open surgical removal and replacement of the catheter. Laparoscopy is a minimal invasive technique alternative to rescue malfunctioning catheters under direct vision. Using laparoscopy for reestablishment of flow through the catheter we can identify the real cause of malfunctioning whether is migration or obstruction and perform the necessary steps to correct the problem. Migration of the catheter tip from the pelvis is the most common laparoscopic finding found in malfunctioning peritoneal cannulas. This is managed with repositioning of the tip in the pelvis. This is followed by omental wrapping or dense adhesions as a cause of obstruction. Partial omentectomy and adhesiolysis is necessary in such situations to reestablish flow. Laparoscopy can also determine if the peritoneal absorptive capacity is overturned and the child needs removal of the catheter and commencement of hemodialysis. Catheter survival rate of 60-90% at one year can be achieved after laparoscopic salvage.