PEDIATRIC SURGERY UPDATE ©
VOLUME 05, 1995

VOL 05 NO 01 JULY 1995

LC vs. OC

During the past thirty-six month period (April 92 to April 95) we (Avilés, Más, & Lugo) have managed to do 40 cholecystectomies in children between the ages of 0.4 to 17.5 years (mean 10.2). Overall 16 patients underwent open cholecystectomy (OC) and 24 laparoscopic cholecystectomy (LC). Females were represented 60%. The most common symptom was right upper quadrant pain in 83% of cases. The etiology of the cholelithiasis was hemolytic in nine children (23%). Data between the two groups was analyzed using chi-square, ANOVA, and Fisher's exact test (a p<0.05 was considered statistically significant). We found that OC cases were younger and had a higher incidence of another simultaneous surgical procedure. Additionally, history of a prior surgical procedure, use of cholangiography, prophylactic antibiotherapy and a longer operating time was more commonly identified in OC children. Although not statistically significant, a trend in the development of postop complication in the OC group was observed. On the other hand, the LC patients showed a shorter hospital stay, more rapid diet resumption, less use of pain medication, and a shorter operating time. Our results conclude that LC is safe, feasible, and effective to do in children with sick gallbladder. The need of cholangiography should be a selective clinical decision. LC should be the standard method of management of gallbladder removal in children due to their immediate recovery, reduced morbidity, early discharge and lessened discomfort.

Gaucher's

Gaucher's disease (GD) is the most prevalent lysosomal sphingolipid storage disorder. It is an autosomal recessive disorder that results from a deficiency in ß-glucocerebrosidase with accumulation of the substrate glucosylceramide in monocytes and macrophages. Type I GD usually start in childhood with most patient presenting before the age of 10. GD causes growth retardation, hepatosplenomegaly, hypersplenism, bone involvement (avascular necrosis) and CNS involvement. IV enzyme replacement is the therapy of choice: has changed life expectancy, reduced organomegaly and improve hematological disorder. It is expensive (approx. $1500/Kg of weight annually). Indications for splenectomy in the era of enzyme replacement may be few: enzyme not available, recurrent symptomatic splenic infarction, life-threatening thrombocytopenia, severe restrictive pulmonary disease, and IVC syndrome. Partial splenectomy (removal of 85-90%) may improve severe hypersplenism and mechanical problems, but remaining spleen enlarges and preop condition recurs in most pts. Total splenectomy is postponed as far as possible in life due to risk of post-splenectomy sepsis, accelerated hepatic and bone lipid deposition with sooner appearance of osteolytic changes (painful crisis).

NB Ostomy

Ostomies in newborn babies are usually done on an emergency basis. Acquired situations such as gangrenous or perforated necrotizing enterocolitis is probably the most common indication for construction of an ostomy in newborns. It may be multiple, at jejunal, ileal or colonic level and placement varies according to the degree of gangrenous bowel found at surgery. Congenital lesions associated with the need of a colostomy during the neonatal period are mainly: Hirschsprung's and Anorectal disorders. Other less often conditions are complicated intestinal atresias, meconium ileus, and volvulus. Care of newborn ostomy must consider output, frequency, and size. The stoma must be protected from abrasion. Appliance fitting is critical; must not overlap the mucosa and little skin should be exposed to intestinal content. Change the appliance whenever it leaks. With time the stoma matures and the skin toughens. Proper training of parents is crucial before discharge from the hospital. Complications are frequent, due to urgent nature of construction and can be: bleeding, fluid and electrolyte losses, prolapse, stricture, and obstruction.

VOL 05 NO 02 AUGUST 1995

Groin Lap for IH

The issue of contralateral exploration in the pediatric inguinal hernia (IH) patient has been hotly debated. Proponents of routine contralateral exploration cite the high percentage of contralateral hernia a/o potential hernia found at exploration, the avoidance of the cost of another hospitalization, psychological trauma and anxiety to the child and parents over a second operation, and the added risk of anesthesia of a second procedure. Most pediatrics surgeons habitually explore the contralateral side. They disagree in opinions about exploration depending upon the primary site of IH, age, sex and the utilization of herniography or some intra-operative technique to check the contralateral side. Recently the use of groin laparoscopy permits visualization of the contralateral side. The technique consists of opening the hernial sac, introducing a 5.5mm reusable port, establishing a pneumoperitoneum, and viewing with an angle laparoscope the contralateral internal inguinal ring to determine the existence of a hernia, which is repaired if present. Requires no additional incision, avoids risk of vas deferens injury in boys, is rapid, safe and reliable for evaluating the opposite groin in the pediatric patient with unilateral IH. Children less than two years of age has a higher yield of positive contralateral findings.

Lap for NUT

The undescended testis identified in 0.28% of males can be palpable (80%) or nonpalpable (20%). It is difficult to determine either location or absence of the non-palpable undescended testis (NUT) by clinical examination. Imaging studies (US, CT, MRI, gonadal venography, etc.) are not reliable in proving its absence. Diagnostic laparoscopy is reliable in locating the NUT or proving its absence. Furthermore it can be combine to provide surgical management. After reviewing thirteen series of 613 children with NUT managed by laparoscopy the following three findings were identified: 1- The testis is present; either in an intra-abdominal (38%) or inguinal position (12%). Intrabdominal testis can be managed by first stage laparoscopic internal spermatic vessel clipping and cutting (Stephen-Fowler's), followed by second stage vas-based standard orchiopexy six to nine months later. Inguinal testes are managed by standard inguinal orchiopexy. 2- The testis is absent (vanishing testicular syndrome) as proven by blind ending vas and testicular vessels (36%). These children are spare an exploration. If the vas and vessels exit the internal ring, inguinal exploration is indicated to remove any testicular remnant as histologic evidence. The presence of a patent processus vaginalis may suggest a distal viable testis. 3- The testis is hypoplastic or atrophic (26%), in which case is removed laparoscopically. Exact anatomical localization of the testis by laparoscopy facilitates accurate planning of operative repair; hence is an effective and safe adjunct in the management of the cryptorchid testis.

AC

Acalculous cholecystitis (AC) is more commonly found in children than adults. Two-third of cases appear as a complication of other illness: trauma, shock, burns, sepsis, and operative procedures. Contributing causes mentioned are: obstruction, congenital tortuosity or narrowing of the cystic duct, decreased blood flow to the gallbladder, and long-term parenteral nutrition. Males are more commonly affected than females. Fever, nausea, vomiting, diarrhea, dehydration and marked subhepatic tenderness are the most common symptoms. Other less common sx are jaundice, and abdominal mass. Labs show leucocytosis and abnormal liver function tests. Recently (APSA 95), two distinct forms of this disease have been recognized: acute, with symptom duration less than one month and chronic, with sx greater than three months. US is diagnostic by demonstrating hydrops of gallbladder, increase wall thickness and sludge. HIDA scan with CCK stimulation may help diagnose chronic cases. In both situations management consist of early cholecystectomy which can be executed using laparoscopic techniques.

VOL 05 NO 03 SEPTEMBER 1995

Infantile Myofibromatosis

The fibromatosis are a heterogenous collection of tumors distinguished by proliferating fibroblast, minimal mitotic and inflammatory activity, limited local fat and muscle invasion that affect all age groups. They originate in the fascial sheath and musculo-aponeurotic tissues, and represent about 12% of soft-tissue tumors in children. Infantile myofibromatosis represent the most common fibrous tumor of infancy. It is characterized by either a localized or multicentric form, appearance during the first year of life, and myofibroblast cells as main pathological participants. A benign, self limited disease process demonstrating spontaneous regression. It includes the formation of palpable masses in skin, muscle, viscera, bone, and subcutaneous tissue. The solitary (localized) form is more commonly found in the head, neck and trunk region affecting mainly boys later during infancy. Is twice as common as the multicentric form. Lesions are usually found in skin, muscle and subcutaneous tissue. Prognosis is excellent with a very low recurrence rate after surgical excision. Positive surgical margins within the tumor predict a high probability of recurrence, especially if large number of slit-like blood vessels and undifferentiated mesenchymal cells are identified. The multicentric form favor lesions in soft tissue, muscle, bone and viscera. Infants are born with the lesions and the prognosis depends on the degree of visceral involvement. Excision is reserved for lesions that jeopardize vital structures.

Lipoblastoma

Tumors of adipose tissue origin are more commonly found in adult than children. They include lipoma, lipoblastoma and angiomyolipoma, to mention a few. Lipoblastoma is a benign tumor of fetal-embryonal fat tissue. Age at presentation is usually less than five years (mean 2.5 years) with a slight male predominance. The main clinical picture is that of a rapidly growing mass in a peripheral location, mainly the extremity (70%). It can arise within soft tissue. Other places identified are: shoulder, back, omentum, retroperitoneum, mediastinum and intrascrotal. Lipoblastomas are wellcircumscribed masses made up of immature fat cells. They have the capacity for differentiation. Microscopy will show lipoblasts with vacuolated cytoplasm present along with primitive mesenchymal cells in a myxoid stroma with plexiform capillaries. Skeletal muscle is sometimes involved. Mitotic figures are normal and rare. The lesion produces a bright signal in both the T1 and T2 weighted magnetic resonance images. A breakdown in the long arm of chromosome eight (8q11-13) is a consistent finding in this tumor. Management consist of complete surgical removal to prevent local recurrence, avoiding radical mutilating resections whenever possible. The tendency to recur is approximately 15%. The prognosis is usually excellent.

Breast

Most breast disorders in children of either sex are benign. Congenital lesions are: absent or multiple breast. Transplacental hormonal influence in neonates may cause hyperplasia of breast tissue with predisposition to infection (Mastitis neonatorum). Premature hyperplasia (thelarche) in females is the most common breast lesion in children. It occurs before the age of eight as a disk-shaped concentric asymptomatic subareolar mass. Remains static until changes occur in the opposite breast 6-12 mo later. It can regress spontaneously or stay until puberty arrives. Biopsy may mutilate future breast development. On the contrary, discrete breast masses in males cause concern and excision is warranted. Gynecomastia is breast enlargement cause by hormonal imbalance, usually in obese pre-adolescent boys. If spontaneous regression does not occur, it can be managed by simple mastectomy. Virginal hypertrophy is rapid breast enlargement after puberty due to estrogen sensitivity. If symptomatic, management is reduction mammoplasty.

VOL 05 NO 04 OCTOBER 1995

Surgical Responses in NB

The endocrine and metabolic response to surgical stress in newborns (NB) is characterized by catabolic metabolism. An initial elevation in cathecolamines, cortisol and endorphins upon stimulation by noxious stimuli occurs; a defense mechanism of the organism to mobilize stored energy reserves, form new ones and start cellular catabolism. Cortisol circadian responsiveness during the first week of life is diminished, due to inmaturation of the adrenal gland. Cortisol is responsible for protein breakdown, release of gluconeogenic aminoacids from muscle, and fat lipolysis with release of fatty acids. Glucagon secretion is increased. Plasma insulin increase is a reflex to the hyperglycemic effect, although a resistance to its anabolic function is present. During surgical stress NB release glucose, fatty acids, ketone bodies, and amino acids; necessary to meet body energy needs in time of increase metabolic demands. Early postoperative parenteral nutrition can result in significant rate of weight gain due to solid tissue and water accumulation. Factors correlating with a prolonged catabolic response during surgery are: the degree of neuroendocrinological maturation, duration of operation, amount of blood loss, type of surgical procedure, extent of surgical trauma, and associated conditions (hypothermia, prematurity, etc.). They could be detrimental due to the NB limited reserves of nutrients, the high metabolic demands impose by growth, organ maturation and adaptation after birth. Anesthetics such as halothane and fentanyl can suppress such response in NB.

Physiology of Pneumoperitoneum

A potential working space during video-laparoscopic abdominal procedures in adults and children is established with the help of a carbon dioxide pneumoperitoneum. Insufflation by either an open or closed (Veress needle) technique will cause an increase in intrabdominal pressure (IAP). Studies during abdominal wall defects closure has shown that the rise in IAP may cause decrease venous return, decrease renal perfusion, low splanchnic flow, and increased airway pressures. The cardiac afterload will increase, an effect magnified by hypovolemia. CO2 is absorbed into the bloodstream transperitoneally causing hypercapnia and acidosis, an effect controlled by increasing minute ventilation by the anesthesiologist. High risk children where this effect can be potentiate further are those with pre-existent cardio-respiratory conditions causing increase dead space, decrease pulmonary compliance and increase pulmonary artery pressure and resistance. Hypotension during the establishment of the pneumoperitoneum could be the result of vascular injury, arrhythmia, too much CO2, impending heart failure, gas embolism or pneumothorax.

Laparoscopic Splenectomy

Laparoscopic splenectomy is another safe and technically feasible video-endoscopic procedures in children. Indications are usually hematological disorders such as ITP, spherocytosis, and Hodgkin's staging. Technical considerations of the procedure are based on anatomical facts such as the variability in the splenic blood supply, the ligaments anchoring the organ and the size of the diseased spleen. Generally the avascular splenophrenic and colic ligaments are cauterized, the short gastric and hilar vessels are individually ligated with metallic clips or staplers (Endo-GIA), and the spleen is placed in a plastic bag and fracture until it is removed through the navel. Advantages of the procedure are: improved exposure, decreased pain, improved pulmonary function, shortened hospitalization, more rapid return to normal activities and excellent cosmetic appearance. Disadvantages are longer operating time, higher costs and the need to open 5-20% of cases due to uncontrolled hemorrhage.

VOL 05 NO 05 NOVEMBER 1995

GB Disorders

Gallbladder (GB) disorders in children are increasing and laparoscopic cholecystectomy (LC) is rapidly becoming the procedure of choice. From 1990 through 1995 eighty-three children between 21 mo. and 18 yrs. of age underwent cholecystectomy at San Pablo Medical Center. Mean age was 14.8 years and females with classic biliary symptoms predominated (RUQ pain and fatty food intolerance). 12% developed gallstone pancreatitis, and 7% jaundice. Indications for surgery were cholelithiasis in 71 (86%) children, GB dyskinesia in ten (12%), and sludge/polyp in 2. Fifty-nine cholecystectomies (71%) were done laparoscopically and 24 (29%) open. Choledocholithiasis (CBD stones) in six children (7%) was managed by open extraction with t-tube placement or endoscopic papillotomy followed by LC. No major ductal complication occurred. Predominant pathologic findings were chronic cholecystitis. LC is superior in post-surgical stay, length of stay, diet resumption, use of pain medication, operating time, and cosmesis to the open procedure. Modern diet, overweight and abnormal liver function chemistry are the main predisposing conditions of GB disorders in children during this decade. Females in their teenage years with typical symptoms continue to be the most commonly affected groups. Persistent biliary symptoms associated to low GB ejection fraction during hepatobiliary CCK stimulated scan may be cause by dyskinesia. The method of choice to remove the disease GB in children is LC: is safe, efficient, and superior to the conventional method. CBD stones can be managed with simultaneous endoscopic papillotomy. Costs of LC are reduced using reusable equipment and selective cholangiographic indications.

SBS

The short bowel syndrome (SBS) is a permanent malabsorption caused by loss of small bowel, leaving a residual jejuno-ileal length of less than 75 cm. During the pediatric age it most commonly results from neonatal necrotizing enterocolitis, intestinal atresia or midgut volvulus. Survival depends on remnant length and presence of ileo-cecal valve. Initial medical management consists of parenteral nutrition, elemental diet and predigested formulas. Management should focus on the rapid intestinal transit time, decreased mucosal surface area, ineffective peristalsis, and short bowel length of these patients. Despite significant morbidity, the general outcome is favorable and warrants aggressive nutritional support, medical treatment, and surgical intervention in selected patients. Patients with symptomatic dilated intestinal segments and stasis hooked on TPN may benefit from intestinal tapering or isoperistaltic lengthening (Bianchi) procedures. Other alternatives are home parenteral nutrition or bowel transplantation.

Umbilicus

Few conditions plague the umbilicus of the young infant. These are: hernias, umbilical granulomas, infectious process (omphalitis), patent urachus or omphalo-mesenteric (O-P) remnants. They can manifest as either purulent, urinary or intestinal discharge. Hernias can wait until the child is older (5 y/o), since most will disappear with time. Granulomas are generally well taken care with silver nitrate applications. Omphalitis during the neonatal period are caused by staph. or strep. organisms invading underneath the granulation tissue of the cord stump, should never be taken lightly, and spreading infection may involve the superficial lymphatic or lower abdomen. Treatment consists of local or systemic antibiotics. Urachal remnants have associated urinary tract abnormalities (distal lower obstruction) and are managed with excision after urinary investigation. O-P remnants clearly describe a communication between the bowel and umbilicus, manifesting as ducts, sinus, cysts or bands. Surgical excision and thorough search for discontinuous segments of the track are necessary. This may include a laparotomy.

VOL 05 NO 06 DECEMBER 1995

Rectal Prolapse

Rectal prolapse refers to a mucosal or full-thickness herniation of rectum through the anal canal that starts as an intussusception that fully develops. Most cases are seen in constipated preschool children after prolonged straining of stools. Other cases may be associated with acute diarrheal episodes, cystic fibrosis, malnutrition, parasitosis, and neurologic or anatomic anomalies. Initial management consists of manual reduction with buttock strapping for 24-48 hours. Recurrent episodes may be managed with outpatient submucous injection of sclerosing agents (5% phenol in almond oil). More aggressive surgical effort may be needed for recalcitrant recurrences and children with pelvic anatomic distortion caused by previous surgery. Surgical choice of procedure is controversial and may encompass simple encircling of anus (Thiersch's) with suture, posterior plication, mucosal stripping, and transsacral or resectional rectopexy, to mention a few. Prognosis is frequently excellent.

Echo in CDH

Congenital Diaphragmatic Hernia (CDH) management has evolved during the past few years. Immediate surgical repair of critically ill neonates with severe lung hypoplasia, increase pulmonary arterial pressure (PAP), and persistent fetal circulation is non-productive. Repair of the defect should be done after preoperative stabilization, no matter how long it takes to reach the objective of improving acid-base status and pulmonary mechanics. This stabilization takes the form of mechanical ventilation and muscle paralysis mostly (ECMO for persistent respiratory failure, where available). Although clinical judgement decides timing of surgical repair, a question often raised is for how long we should avoid surgery? Doppler echocardiographic studies have focused on methods to decide the degree of pulmonary hypoplasia as useful alternatives of answering that question and assessing prognosis. This has been done measuring changes in PAP, the bi-directionality of the patent ductus arteriosus (PDA), the left ventricular mass index, and recently the left to right main pulmonary artery ratio. Changes that favor surgical repair using these parameters are: evidence of reducing PAP, reducing pulmonary vascular resistance, and predominant left to right flow changes of the PDA. Poor prognosis is associated with reduced left ventricular mass index (also predicts use of ECMO) and decrease left to right main pulmonary arterial ratio.

Cat Scratch Disease

Cat Scratch Disease (CSD) is a self-limited condition transmitted by a Bartonella species (Rochalimaea henselae) present in unaffected kitten paws. Following inoculation by a scratch and one to two weeks of incubation period, malaise, fever, headache, anorexia and swelling of the regional lymph nodes follow. The adenopathy generally develops in the upper extremity (epitrochlear, axilla) or head/neck areas, is minimally tender and can develop fluctuation. Median age is 14 years with highest attack rate in children less than ten years of age. The diagnosis relies on the presence of symptoms, signs, physical exam (characteristic papule at the site of the scratch), history of exposure to a cat, and a positive immunofluorescent assay for bartonella antibodies. Most patients with clinically diagnosed CSD developed an immunologic response to Bartonella species. Conservative symptomatic management is recommended for most children since the node will eventually disappear spontaneously. In other cases' aspiration of fluctuant nodes is alleviating. Antibiotics are recommended during severe cases. Overall prognosis is good.

References?

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