Timing of colonic necrosis in hemolytic uremic syndrome.

Hemolytic uremic syndrome (HUS) consists of an acute onset of microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction. HUS-associated colitis can be seen in up to 100% of patients and is usually associated with severe abdominal pain and distention. Colonic perforation is a complication of HUS that has a reported incidence of 1%-2%, and although there are several case reports in the literature describing perforation of the colon, it is still very difficult to discern the abdominal symptoms associated with HUS colitis from perforation. Four cases of colonic perforation are reported here from a consecutive series of 57 patients, in which a trend in the length of time from the onset of symptoms of HUS to colonic perforation was determined. A review of the literature for cases of HUS-associated colonic perforation was also performed. The time from the onset of HUS symptoms to colonic perforation in our series was similar to that found in the literature review (11 +/- 5 vs 14 +/- 8 days). Awareness that this complication has a tendency to occur towards the end of the 2nd week during the course of HUS is essential to avoid an unnecessary and untimely surgical intervention.

Differentiated and medullary thyroid cancer in childhood and adolescence.

Thyroid cancer is rare in childhood and consists of several different histopathologic groups with widely varying clinical behavior. Major categories include differentiated, medullary, and anaplastic thyroid cancer. Non-Hodgkin's lymphoma and various sarcomas also can arise in the thyroid. This article discusses differentiated and medullary thyroid cancer in childhood and adolescence. Differentiated thyroid carcinoma is divided into three subtypes: papillary, papillary with follicular elements, and follicular. Medullary tumors may occur in isolation but more frequently are associated with one of the multiple endocrine neoplasia syndromes (MEN). A paradoxical observation concerning patients with differentiated thyroid cancer is that 70% to 80% present with regional lymph node involvement, and more than 20% have distant metastases at diagnosis. However, survival rates in series with median follow-ups of 10 to 20 years have been 90% to 100%. These data have implications for the surgical management of pediatric patients with differentiated thyroid cancer. The cellular and genetic factors that underlie this paradoxical behavior are not understood. Management of children with medullary thyroid cancer was revolutionized by the identification of specific mutations in the ret oncogene that predict for multiple endocrine neoplasia syndromes. This has allowed sensitive and specific diagnosis based on analysis of the patient's white blood cells. Because this leads to earlier diagnosis, total thyroidectomy can be performed at a much earlier age than if the increase in serum calcitonin was used to identify C-cell hyperplasia or early carcinoma. At present, genetic testing should be performed at birth in children suspected of having the MEN IIb syndrome and no later than 1 year of age for those with possible MEN IIa. If specific ret gene mutations are noted, total thyroidectomy is recommended as soon as the diagnosis is established. For MEN IIa patients, thyroidectomy probably should be performed before 5 years of age, whereas patients with MEN IIb may require surgery during the first 6 months to 1 year of life.

Transanal mucosectomy: a modification of the Soave procedure for Hirschsprung's disease.

In the Soave procedure for Hirschsprung's disease, the mucosectomy of the aganglionic segment of colon is performed transabdominally. The authors describe an innovative modification of the standard Soave procedure, in which a transanal mucosectomy is performed as the abdominal incision is made. The transanal mucosectomy technique was compared with the conventional approach. From 1974 to 1993, 51 patients underwent a Soave procedure for Hirschsprung's disease. Twenty-six (51%) had the standard Soave procedure (STD) (1974-1984), and 25 (49%) had the modified procedure (MOD) (1984-1994). In the latter, the entire mucosal dissection was performed through a transanal approach. Surgical data were accumulated by database analysis. Each case was entered into a database at the time of operation, and follow-up data were obtained through mail-in questionnaires or personal communication. The two groups were comparable with respect to number, gender distribution, and average age at the time of operation. There were two superficial wound infections in the STD group and two superficial wound infections and one death (secondary to cardiac arrest in a patient with trisomy 21 and a severe congenital cardiac anomaly) in the MOD group. The average postoperative stay for the STD group was 10.8 days; that for the MOD group was 6.8 days (t test: P < .0001). The operating time was shorter for the MOD procedure. Follow-up data were available for 25 (96.2%) patients in the STD group and 25 (100%) in the MOD group. The two groups were comparable in all respects except for the average length of follow-up, which was longer for the STD group. No patients had, urinary incontinence or sexual dysfunction (when applicable). Overall, patients/parents in both groups rated the outcome as "good." This modification of the Soave technique for Hirschsprung's disease is both safe and effective. Compared with the standard procedure, it is associated with shorter operating time (because the transanal mucosectomy is performed as the abdomen is opened) and a shorter stay postoperatively. The results have been excellent.

Surgical management of Crohn's disease in children.

Crohn's disease is a chronic, transmural inflammatory disease of the intestinal tract most frequently involving the terminal ileum and colon. It is a disorder of undetermined etiology that shares many pathophysiologic clinical aspects with chronic ulcerative colitis. Surgical treatment of Crohn's disease continues to be generally limited to the treatment of the complications of the disease. It is being reported with increasing frequency in children of all ages. The surgical principle generally adhered to is to remove only enough intestine to relieve the complication but to maintain as much intestinal length as possible. Because of this requirement, strictureplasty has become a worthwhile surgical adjunct, especially when dealing with multiple minimally inflamed fibrotic strictures in the small intestine. It has been as beneficial in children as in adults. Restorative proctocolectomy and the Kock pouch procedure are discouraged in patients with known Crohn's disease because of numerous subsequent complications. It is very important that surgeons and physicians realize that although the disease process cannot be eradicated by surgery, the health and well-being of a child or teenager can be restored to normal for prolonged periods, if not permanently.

Thyroglossal duct cysts and branchial cleft anomalies.

Thyroglossal duct cysts and branchial cleft anomalies are important in the differential diagnosis of cervical lesions. Both lesions are common and are seen more commonly in the child than in the adult. Considerable discomfort and morbidity may be associated with severe infections occurring in both of these lesions. Of importance is the observation that once these congenital cysts become infected, subsequent surgical removal is very difficult, and recurrence may result. For these reasons it is essential that the clinician become familiar with the embryology of these lesions, which is vital to the understanding of them. In addition, the development of skills in diagnosing and managing this condition is essential.

Papillary carcinoma of the thyroid in children.

Papillary carcinoma of the thyroid is the most common thyroid cancer in children. It usually occurs in the teenage female and presents as a 3-cm mass within the thyroid itself. Although the tumor is well differentiated, almost 80% of all children will present with local lymph node metastasis. Despite this metastasis, most children have a life expectancy that parallels those of their peers for 30 years. Treatment for well-differentiated papillary carcinoma includes surgical excision of all cancerous tissue and removal of the affected lymph nodes. Radioablation of residual disease has been effective.

Medullary thyroid carcinoma in children.

Medullary cancer of the thyroid (MCT) in children, although uncommon, represents not only a most intriguing problem but also one that is a model of early cancer detection that results in cure. MCT in children is usually a part of the multiple endocrine neoplasia (MEN) syndrome (MCT, pheochromocytoma, and hyperparathyroidism) and is detected by screening studies. MCT arises from C cells that secrete calcitonin, with C-cell hyperplasia representing the precancerous state. Both are diagnosed by measuring calcitonin levels both basally and after stimulation with pentagastrin. Screening studies should begin shortly after birth in infants at risk for MEN IIb and by age 1 year in children at risk for MEN IIa. Application of this approach should result in near elimination of the spread of cancer in children with MCT.

Current surgical management of Crohn's disease in childhood.

Crohn's disease is a chronic transmural inflammatory disease that may involve any portion of the gastrointestinal tract. It is being reported with increasing frequency in children of all ages. The diagnosis of Crohn's disease in children is dependent on the clinical, radiological, endoscopic, and pathological material that excludes other acute inflammatory disease of the gastrointestinal tract. Although medical therapy is instituted once the disease is diagnosed, surgery remains an integral part of the overall management of Crohn's disease. Surgical management continues to evolve. It is very important that both surgeon and physician realize that although the disease process cannot be eradicated by surgery, the health and well-being of a child or teenager can be restored to normal for prolonged periods of time, if not permanently.

Rectal mucosectomy: a definitive approach to extensive hemangiomas of the rectum.

Major rectal bleeding may occur in children and adults who have extensive hemangiomatous involvement of the pelvis and rectosigmoid colon, as in the Kleppel-Trenaunay syndrome (KTS). Conventional surgical techniques such as bowel resection and colostomy have often been associated with large blood loss and/or incontinence. We have used a new approach to this problem utilizing rectal mucosectomy to eliminate the bleeding rectal mucosa and to preserve anal function. Four patients born with KTS eventually developed major rectal bleeding and were successfully corrected by this surgical approach. Two were females and two males. Ages ranged from 4 to 25 years at the time of surgery. The patients developed rectal bleeding leading to chronic anemia during the first decade of life. Rectal bleeding gradually increased requiring multiple transfusions (2 to 20 units) prior to surgery. Patient 1 underwent resection of the rectosigmoid colon, with rectal mucosectomy and endorectal coloanal anastomosis. The Nd:YAG laser was used on 3 occasions on the distal remaining rectal mucosa. Patients 2, 3, and 4 underwent sigmoid resection, transanal rectal mucosectomy, and a coloanal anastomosis, with minor YAG laser therapy in one patient. Blood loss during surgery was minimal. Follow-up ranged from 1.5 to 4 years. All have excellent sphincter control with no incontinence. No strictures or sepsis occurred following surgery. Rectal bleeding was eliminated in all 4 patients. This sphincter-saving approach should be considered in patients with extensive hemangiomas of the rectosigmoid colon because of its remarkable effectiveness and safety.

Review of head and neck lesions in infancy and childhood.

Common congenital and infectious lesions of the head and neck in infancy and childhood have been discussed. Those that are present at birth, asymptomatic, and frequently cystic are clearly benign lesions that require operative management for the potential complications of enlargement and infection. Solid lesions, particularly those of the lymph nodes, must be differentiated from neoplasms, particularly Hodgkin's disease and non-Hodgkin's lymphoma. Suppurative lymphadenitis is obvious from its local inflammatory signs, but the subacute and chronic adenopathies may be difficult to distinguish from neoplasia. An attitude that unless the benign nature of a lesion is clearly demonstrated, early excisional biopsy for histopathologic evaluation must be the rule will avoid delays in diagnosing those neoplastic lesions that can be treated successfully if managed early and aggressively.

Giant epignathus. A case report.

We report the successful treatment of a 2.8 kg female infant born with a giant epignathus, and we present our current prenatal and neonatal recommendations for managing this problem. We recommend that the delivery be done by cesarean section, that an adjacent operating room be ready for the baby, and that a neonatologist, anesthesiologist, and pediatric surgeon be standing by. The term epignathus now commonly applies to a teratoma protruding from a newborn's mouth. The survival of these otherwise normal children has been very low. We discuss the perinatal, neonatal, and operative care necessary to improve survival, as evidenced by the case presented.

The surgical management of children with ulcerative colitis. The old vs. the new.

The authors' experience with children who have chronic ulcerative colitis was reviewed to compare their current surgical approach (ileoanal anastomosis) with earlier methods of management. Between 1960 and 1984, 137 children with chronic ulcerative colitis underwent surgery (mean duration of follow-up, 7.1 years). In 91 patients, the procedures were a total proctocolectomy with ileostomy or Kock pouch (66) or a lesser colectomy with either an ileostomy (16) or an ileorectal anastomosis (9) (group I). Forty-six patients underwent an ileoanal anastomosis procedure (group II). Children in group I were more likely to have significant preoperative loss of weight, a debilitated condition, and malnutrition. Urgent or emergency surgical intervention was required in 25 percent of patients in group I but in only 4 percent of patients in group II. Trends included 1) a younger age at operation in group II, 2) a higher mortality in group I (7.7 percent) than group II (0 percent), and 3) a higher perioperative mortality with emergency operations (23 percent) than elective procedures (1.6 percent). In group I, 98 percent of patients had an abdominal ostomy, but no patients in group II had an abdominal ostomy. The children with an ileoanal anastomosis had an average of 4.8 stools during waking hours and 1.3 stools each night. On the basis of this experience, the authors recommend use of the ileoanal anastomosis procedure in the surgical treatment of chronic ulcerative colitis in children.

Long-term follow-up of the ileoanal anastomosis in children and young adults.

The purpose of this study was to carry out a long-term study of the ileoanal anastomosis (IAA) in children and young adults, comparing the straight IAA to the J pouch. One hundred twenty-one young people who had undergone IAA were studied, with 114 available for long-term follow-up. One hundred one were 18 years and under. Forty-nine patients had a straight IAA and 72 had a J-pouch reservoir. There were no deaths. After surgery, three children had intraabdominal sepsis and one had pelvic sepsis, but it did not lead to excision of the IAA. The mean stool frequency in all 114 patients was 5.0 +/- 2.5 per day and 1.2 +/- 1.1 at night. The mean number of stools for the straight IAA was 6 per day and 2.1 at night. The mean number of stools for the straight IAA with balloon dilations was 5.8 per day and 1.2 at night, and for the J pouch it was 4 per day and 1 at night. Patients with both the J pouch and straight IAA had good to excellent sensation, with patients with the J pouch always able to distinguish flatus from stool in 87% of patients and almost always in 13%. Daytime continence was very good in both groups. Moderate nighttime loss of stool occurred in 10 patients, 6 with a straight IAA and 4 with a J pouch. Ninety-five percent of the 114 patients were satisfied or very satisfied, with most children with a J pouch very satisfied. The J pouch remains the procedure of choice in young people.

Carcinoid tumor of the appendix in the first two decades of life.

Carcinoid tumor of the appendix is the most common neoplasm of the gastrointestinal tract in childhood and adolescence. Sufficient long-term follow-up data after surgical treatment are not currently available for patients diagnosed during the first two decades of life. From 1936 to 1988, 23 patients were observed at this institution with histologically confirmed carcinoid tumors involving the vermiform appendix. In contrast to the adult experience, in which the tumor is most commonly encountered as the result of an incidental appendectomy, 18 of these patients presented with signs and symptoms of an acute abdomen directing the surgeon to the appendix. In the other five cases, surgery was performed for other reasons. Median age at presentation was 13.0 years (range, 6 to 20 years). Fourteen patients were female, nine were male. Simple appendectomy was the initial procedure for all patients. Tumor size ranged from "microscopic" to 2.5 cm in largest dimension. Three patients subsequently underwent right hemicolectomy, and one patient had removal of a residual appendiceal stump, but no residual or metastatic tumor tissue was found in any of the resected specimens. Nineteen patients underwent simple appendectomy alone. Eighteen available specimens were reviewed at the time of this study for confirmation of histology and degree of invasion. The tumor invaded to the serosa in nine of 23 (39%). The mesoappendix or periappendiceal fat was involved in seven of 23 (30%). Vessel invasion was not noted in any specimen. Our median follow-up time was very long, being 26 years (range, 9 months to 51 years). No patient has had evidence of recurrent or metastatic disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Evaluation of children with multiple endocrine neoplasia type IIb following thyroidectomy.

Medullary thyroid carcinoma (MTC) develops in all patients with multiple endocrine neoplasia type IIb (MEN IIb), a rare syndrome that either occurs sporadically or is inherited in an autosomal dominant pattern. The MTC in patients with MEN IIb has been reported to be biologically aggressive with onset at a young age and rapid progression as evidenced by widespread metastases and death, frequently in the teenage years. Seven children, aged 2 to 11 years (mean, 7 years), from three kindreds with MEN IIb were evaluated for evidence of tumor recurrence 3 to 10 years following thyroidectomy. In one child, age 11, a thyroid mass was palpable preoperatively. However, in the remaining six children (aged 2 to 10 years), the diagnosis of MTC was established by an increased concentration of plasma calcitonin (CT), either basally or following pentagastrin (Pg) stimulation. All patients underwent total thyroidectomy with removal of central lymph nodes from the neck. At the time of surgery, six children were found to have bilateral macroscopic MTC, five without and one with cervical metastases. One child (age 2 years) had C-cell hyperplasia, a premalignant precursor of MTC. Currently, five of the seven children are without evidence of recurrent disease clinically and have normal plasma CT levels (less than 0.3 ng/mL) following calcium (Ca) and Pg stimulation 3, 3, 10, 10, and 10 years after thyroidectomy. Two of the seven children have biochemical evidence of residual MTC.(ABSTRACT TRUNCATED AT 250 WORDS)

Pediatric urological complications with intraoperative radiation therapy.

Intraoperative radiotherapy with variable energy electrons has been used as a supplemental boost to treat 6 children with locally advanced retroperitoneal malignancies. Of the patients 4 had treatment-related injuries to portions of the urinary tract within the intraoperative and external radiation fields. Three patients had significant renal impairment requiring surgical correction. The pathogenesis and management of treatment-induced injury to the urinary tract are discussed.

Exercise cardiorespiratory function in adolescents with pectus excavatum. Observations before and after operation.

We studied 12 children with pectus excavatum (mean age, 13.8 years) using pulmonary function and exercise testing. Eight patients had surgical repair and were studied before and after repair. Four patients did not have repair and were tested twice. The degree of chest wall deformity in the two groups was similar. All patients reached 86% or more of their maximal predicted heart rate with exercise. Total lung capacity decreased 8% (p less than 0.01) after operation. In the nonoperated control group, total lung capacity was similar during both studies. The ratio of ventilation at maximal exercise to maximal voluntary ventilation was 68% +/- 12% before and 66% +/- 9% after operation in the operated group and 69% +/- 18% and 79% +/- 30% at two studies in the nonoperated group (nonsignificant changes). Maximal oxygen uptake was 36.1 +/- 4.4 ml/kg/min preoperatively and 38.1 +/- 8.1 postoperatively and 41.2 +/- 7.3 ml/kg/min and 43.0 +/- 6.9 on two studies in the nonoperated group (nonsignificant changes). Cardiac output and stroke volume increased appropriately with exercise both before and after operation. Operation had no physiologically significant effect on the cardiorespiratory response to exercise. Slight changes in cardiorespiratory function occurred in both operated and nonoperated subjects. These results emphasize the importance of studying patients before and after operation and of using a control group.

Medullary carcinoma in children. Results of early detection and surgery.

Early diagnosis and surgical treatment of medullary carcinoma of the thyroid (MCT) in children is essential to decrease the likelihood of metastatic spread. To determine the optimal timing of screening and surgery, we reviewed all children younger than 16 years old who were undergoing surgical treatment. From 1970 to 1988, 33 children 6 months old to 15.9 years old (mean, 9 years old) with MCT or C-cell hyperplasia or both underwent total thyroidectomy. Follow-up was 4 months to 15 years after surgery (mean, 9 years). Six of 31 patients presented with a neck mass. Basal immunoreactive calcitonin level was increased in 3 of 7 patients with C-cell hyperplasia alone, in 10 of 18 patients with nonmetastatic MCT, and in all 5 patients with metastatic MCT. At the time of surgery, 7 patients had C-cell hyperplasia, 19 patients had MCT without metastasis, and 7 patients had MCT with metastases. All children with metastasis were more than 12 years old. Medullary carcinoma of the thyroid was seen as early as age 6 months in a child with multiple endocrine neoplasia type IIb (MEN-IIb) and age 3 years in a child with MEN-IIa. At follow-up, 25 patients were free of disease and 8 had had recurrent disease. Two of the 8 patients with MEN-IIb had died. All 6 children with a neck mass at presentation had recurrent disease. Of 27 patients diagnosed by screening, 25 had no evident disease and 2 had persistent disease. Thus, in children with MEN-II syndromes, screening studies are highly effective in detecting MCT. All children with MEN-IIb should be studied shortly after birth, and those with MEN-IIa should be studied by 1 year old.

Clinical outcome in children with acute cerebellar encephalopathy and neuroblastoma.

Acute cerebellar encephalopathy (ACE)--ataxia often associated with opsoclonus, polymyoclonus, and irritability--may be associated with neuroblastoma and should be suspected in a child who presents with ACE. The survival in ten children with ACE associated with neuroblastoma was 100%. Most of the tumors were ganglioneuroblastomas. The abdomen was the most common location for the tumor in this study although it also can be found in the mediastinum. All patients with this syndrome had a localized tumor. With ACTH therapy, ACE may resolve early postoperatively, but it tends to recur in most patients, continuing for as long as 3 years after the initial operation. Treatment with ACTH or prednisone modifies and rapidly clears the symptoms during the acute episode of recurrent ACE as well as helps to resolve subsequent neurologic sequelae. Significant neurologic sequelae persisted in seven of ten patients and included deficits in cognition or intellect, hyperactivity, impulsivity, emotional lability, and mild motor deficits. Seizures were seen in one patient.