Robert L. Telander

Does rectal mucosa regenerate after ileonal anastomosis

Regeneration of rectal mucosa after rectal mucosectomy and ileoanal anastomosis (IAA) could jeopardize the long-term safety of the procedure. The aim of this study was to determine if rectal mucosal regeneration occurred after IAA. Pathologic specimens of the IAA and surrounding rectal muscular cuff were obtained from 29 patients who had required IAA excision 17±2 months (mean±SEM, range, 2 to 48 months) following construction. Multiple (≥6) coronal and sagittal sections of each specimen were made and examined histologically. The rectal muscle cuff was bound to ileal serosa by dense fibrous tissue. Small islets of residual rectal mucosa were identified between the denuded rectal cuff and the ileal pull-through in four patients (14 percent) and at the ileoanal anastomosis in two patients (7 percent). Active rectal muscosal disease, dysplasia, or reeplithelialization of the denuded rectal muscle were not seen. It is concluded that small islets of rectal mucosa may remain after IAA. Up to four years after IAA, however, no evidence of rectal mucosal regeneration could be documented.

Surgical implications of Klippel-Trenaunay syndrome.

Between January 1956 and July 1981, 40 patients with Klippel-Trenaunay syndrome were seen at the Mayo Clinic. Twenty male and 20 female patients presented with the classic triad of soft tissue and bony hypertrophy of the extremity, hemangioma, and varicosity without evidence of functional arteriovenous fistulae. The lower extremity was involved in 38 patients (95%), the upper extremity in six patients (15%). In four of these cases (10%), both the upper and lower extremities were affected. The disease was unilateral in 34 patients (85%), bilateral in five cases (12.5%), and crossed-bilateral in one case (2.5%). Surgery was done in 13 patients (32.5%), nine of whom were operated on at the Mayo Clinic and four of whom had had previous surgery elsewhere. Excision and stripping of varices were performed in three patients; of these three, a deterioration of symptoms was observed in one, but the procedure was beneficial in the other two. Partial varicectomy was performed in four cases, while resection of the angioma was attempted in eight cases, with good results in three cases of small angiomas. Femoral and tibial epiphysiodesis stopped the overgrowth and produced an excellent result in one case. In four cases of epiphysiodesis at the foot level and two cases of derotational tibial osteotomy, moderate improvement was achieved. The indication for vascular or orthopedic surgery should be carefully considered in each patient who has this syndrome. One patient (2.5%) died of a severe form of the disease, but the relatively benign course is documented by the 21 patients (52.5%) who are free of complaints without any treatment or with elastic support only.

Fate of the rectal mucosa after rectal mucosectomy and ileoanal anastomosis

The aim of our study was to determine if the rectal mucosa regenerates after rectal mucosectomy and endorectal ileoanal anastomosis for chronic ulcerative colitis. Such regenerated rectal mucosa could be the site of recurrent disease, leading to complications of the operation, and potential malignant degeneration. Pathologic specimens of the ileoanal anastomosis, surrounded by rectal muscular cuff, were obtained from eight patients who required takedown of their ileoanal anastomosis between one and 18 months after construction. Reepithelialization of the rectal cuff was not observed. In two patients, small islets of rectal mucosa and anal glands were identified. In all patients, the rectal muscularis propria was adherent to the serosa of the ileum by fibrous reaction. Three patients were diagnosed, both clinically and pathologically, as having chronic ulcerative colitis at the original ileoanal operation, but features suggestive of Crohns disease were noted in the subsequently resected neo-rectum. Our observations suggest that, although isolated rectal mucosal cells may remain after mucosectomy, extensive rectal mucosal regeneration does not occur, thus minimizing the risk of recurrent disease and potential malignant change. Failure of an ileoanal anastomosis is therefore most likely related either to technical factors or to the presence of unsuspected Crohns disease.

Physiologie Aspects of Continence After Colectomy, Mucosal Proctectomy, and Endorectal lleo-anal Anastomosis

We examined the physiology of continence in 12 patients at least four months after colectomy, mucosal proctectomy, and endorectal ileo-anal anastomosis for ulcerative colitis and familial polyposis. The mean fecal output (+/-SEM) was 598 +/- 60 gm, passed as 12 +/- 4 movements/24 hr, of which 4 +/- 1 were passed at night. The patients were generally continent during the day and could distinguish gas from stool, but 11 of 12 leaked stools at night. Anal sphincter resting pressures (71 +/- 8 cm H2O) and squeeze pressures (171 +/- 15 cm H2O) of patients were similar to those of ten healthy controls (P greater than 0.05), although the rectal inhibitory reflex was absent in the patients. After operation, the distal bowel had a pressure-volume curve of greater slope (0.15 +/- 0.05 ml/cm H2O) than it had in controls (0.07 +/- 0.01 ml/cm H2O, P less than 0.05) and a lesser maximum capacity (patients, 248 +/- 31 ml; controls, 406 +/- 26 ml; P less than 0.05). The greater the capacity of the neorectum, the fewer was the number of bowel movements/day (r = 0.91, P less than 0.001). We concluded that the operation preserved the anal sphincter, although it decreased the capacity and compliance of the distal bowel and impaired continence.

Primary mediastinal tumors in children

Mediastinal masses in children constitute a heterogeneous group of malignant and benign neoplasms. The majority of malignant tumors in our series of 188 children with a mediastinal mass were Hodgkins and non-Hodgkins lymphoma (87 patients) arising in the anterior and middle mediastinum. Ganglioneuroma (17) arising in the posterior mediastinum made up the bulk of benign tumors (52). Infants less than 2 yr old presented with symptoms of tracheal compression, whereas older children had fewer symptoms but a far greater likelihood of having a malignancy. In the assessment of patients with mediastinal tumors, the posteroanterior and lateral chest roentgenograms were most often diagnostic, and computed tomography of the chest provided the most information concerning preoperative resectability. The excellent survival of patients with stage I and II Hodgkins disease reflects the radiosensitivity of the tumor; in our series of 33 patients, survival was not increased by radical resection. Unlike lymphomatous tumors, neurogenic masses should be completely excised. When initial exploration reveals unresectability, biopsy followed by radiation therapy and second-look excision of the tumor can be accomplished. In patients with primary mediastinal sarcomas, total resection should be carried out if possible. In our series, adjuvant radiation therapy or chemotherapy was effective in only 1 of 15 children with sarcoma. Surgical treatment of all mediastinal masses except lymphoma should be complete excision. In cases of suspected lymphoma, cervical or supraclavicular node biopsy can yield adequate tissue for diagnosis.

Thyroglossal and Branchial Cleft Cysts and Sinuses

Cysts of the neck are usually congenital, with either thyroglossal or branchial cleft origin. The diagnosis is made on the basis of the physical findings. In both of these entities, total excision of the tracts is essential to minimize the likelihood of recurrence.

Crohn's disease in children: assessment of the progression of disease, growth, and prognosis.

There were 177 children less than 15 yr of age treatment for Crohns disease. Twenty percent had a family history of inflammatory bowel disease. Fifty-three percent had small bowel disease, 44% had both small and large bowel disease, and 3% had isolated colonic involvement. Fifty-eight children (33%) were treated medically. One hundred nineteen patients (67%) required operative therapy. Fity-nine percent had one operation, 22% had two operations, and 19% had three or more operations. Follow-up ranged from 1.3 to 30 yr. The probability of additional surgery was influenced by the length of the follow-up interval, beginning at the date of the last operation. In children requiring one operation, the likelihood of further surgery was 7% at 1 yr, 36% at 5 yr, and 48% at 10 yr of follow-up. Patients having both small intestinal and colonic involvement had a significantly greater chance of requiring reoperation. The mean height of all children with Crohns disease was significantly less than the mean of the normal population. Patients requiring surgery were significantly shorter than children treated medically. Operation resulted in a significant increase in growth immediately after surgery. The rate of growth in height from time of diagnosis to final adult follow-up was greatest for operated patients. The growth rate for medically treated patients was significantly less. Upon reaching adulthood, both surgically and medically treated patients were essentially the same size and were significantly shorter than average in height. At last follow-up, 71% of all patients were not limited in activity by their disease and 80% considered their state of health to be good to excellent. Survival of patients with Crohns disease compared very favorably with that of the normal population. After 5 yr the survival rate was 98% of expected. After 20 yr the survival rate was 89% of expected.

Pheochromocytoma in the pediatric age group: Current status

Pheochromocytoma is an unusual tumor in the pediatric age group. Several aspects serve to differentiate children with pheochromocytoma from their adult counterparts. Children have fewer malignant tumors, more extraadrenal tumors, and greater bilaterality and multiplicity of tumor. Similarly, they have an increased incidence of multiple endocrine neoplasia (MEN) and familial disease. The records of 16 children (age 17 years and younger) with pheochromocytoma were evaluated. One newborn died at 36 hours of age, and a 3-year-old died four days after exploratory laparotomy. Of the 14 survivors (with an average follow-up of 8 years), three had manifestations of multiple endocrine neoplasia type 2 and two had familial pheochromocytoma only. One patient had malignant paragangliomas. Three patients had bilateral tumors; one of these had MEN. Four patients underwent operation before the use of alpha- and beta-adrenergic blockade was routine at our institution. An additional patient received blockade in preparation for the removal of the pheochromocytoma but was not prepared for preoperative angiography. Hypertensive complications were encountered in four of these five children; in one, this complication was fatal. There were no intraoperative complications in the 12 patients who had received appropriate blocking agents. Eleven of the 14 survivors are currently normotensive without the need for antihypertensive medication. Two patients have medically controlled hypertension, and one continues to have severe hypertension. An overview of this series underscores the characteristic features of pheochromocytoma in the pediatric age group. Angiography has been replaced by high-resolution computed tomography for localization.(ABSTRACT TRUNCATED AT 250 WORDS)

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.

Results of early thyroidectomy for medullary thyroid carcinoma in children with multiple endocrine neoplasia type 2.

Children with multiple endocrine neoplasia type 2 (MEN2) often develop medullary carcinoma of the thyroid (MCT) or its precursor, C-cell hyperplasia. Survival results are improved if malignancy is diagnosed early from the results of plasma immunoreactive calcitonin (iCT) measurement. The effect of early detection and thyroidectomy in children with MEN2 syndrome was determined by reviewing the experience between 1975 and 1985. Seventeen children with MEN2 who were 12 years old or younger underwent a total thyroidectomy for MCT or C-cell hyperplasia. iCT was measured in all patients preoperatively and postoperatively. Of the 17 children, 14 (82%) had MEN2a and 3 (18%) had MEN2b. There were 14 (82%) female and three (18%) male patients; their mean age was 6.97 years (range 1.5 to 12 years). In all patients, the diagnosis of MCT was made from initial elevated levels of iCT after stimulation with pentagastrin. Three patients had clinical evidence of disease preoperatively. All patients underwent a total thyroidectomy and lymph nodes were removed from the central zone; a neck dissection was performed in the three with clinically obvious disease. MCT with C-cell hyperplasia was found in 11 children and C-cell hyperplasia alone in six. Of the 11 with carcinoma, eight had bilateral disease and three unilateral. Six children had bilateral C-cell hyperplasia. All 17 children were alive and feeling well at the time of this report; however, three had evidence of metastatic disease according to iCT measurements. None of the children had recurrent nerve injuries; one had evidence of hypoparathyroidism.(ABSTRACT TRUNCATED AT 250 WORDS)