Management of perianal abscess and fistula-in-ano in children.

INTRODUCTION: Perianal abscess (PA) and fistula-in-ano (FIA) are common acquired anorectal disorders in children, but their management is still controversial. This study was performed to evaluate our experience with the treatment of PA and FIA in children of different age groups. MATERIAL AND METHODS: A retrospective study was conducted of children below 16 years of age treated for PA/FIA in a pediatric surgery center between January 2002 and April 2006. The standard treatment for PA was incision and drainage (I&D). Judicious probing for fistulae was only performed in recurrent abscess or if a discharge of pus was identified from the anal verge at surgery. Fistulotomy was routinely performed in low fistulae not associated with inflammatory bowel disease (IBD). IBD associated fistulae were treated with topical tacrolimus in the absence of deep seated infection. Patients were divided into 3 age groups: <2 years, 2-8 years and >8 years. Mode of treatment, microbial organisms, recurrence, associated FIA and association with IBD were recorded. The median follow-up period was 6 months (8 weeks-3 years). Fisher's exact test was used for the analysis of categorical variables. RESULTS: A total of 78 (39 [<2 years]; 17 [2-8 years]; 22 [>8 years]) patients were treated for PA/FIA during the four year period. In children aged <2 years, 33 (85%) had I&D of PA and the other 6 (15%) had fistulotomy. Recurrence was seen in 9 (23%) children, of which 3 (8%) had FIA. In children aged 2-8 years, 13 (76%) had PA and 4 (24%) had a FIA and there were no recurrences. In children >8 years, 12 (55%) had I&D, 1 (4%) had a fistulotomy and 9 (41%) were treated non-surgically. Six of 7 patients with IBD associated FIA were treated successfully with topical tacrolimus. The recurrence rate after primary surgery was significantly higher for <2 years and >8 years age groups compared to the 2-8 years age group. The incidence of FIA identified either at primary operation or during exploration for recurrence was highest (50%) in >8 years age group and lowest (21%) in the <2 years age group. Lactose fermenting coliforms were the most common organisms isolated from pus. The presence of intestinal organisms in pus was associated with significantly higher recurrence rates in children aged <2 years. CONCLUSIONS: Surgery for PA/FIA in children aged <2 years resulted in low recurrence rates and should be considered as the primary treatment. Topical tacrolimus was found to be an effective treatment for IBD associated FIA.

Juvenile xanthogranuloma: a case report of a preterm baby.

This case report describes a preterm baby with juvenile extracutaneous xanthogranuloma that rapidly expanded. The tumor was excised because of rapid enlargement and extension into the thoracic inlet. To our knowledge, this is the first case of a preterm baby with extracutaneous juvenile xanthogranuloma. The case illustrates the need for careful surveillance of this lesion because it cannot always be managed conservatively.

Pulmonary metastasectomy for pediatric solid tumors.

Most pediatric thoracic malignancy is pulmonary disease secondary to solid tumors of childhood. The management of isolated pulmonary metastases in adulthood is well documented. Little has been published to document the long-term outcome of pulmonary metastasectomy in childhood. A retrospective study was undertaken to assess the results of surgery for isolated pulmonary metastases. Twenty children underwent surgery over 12 years (mean follow-up 8 years). Five had Wilms' tumor (mean age 51 months), eight had osteogenic sarcoma (mean age 141 months), three had rhabdomyosarcoma (mean age 92 months), two had hepatoblastoma (mean age 30 months) and two had teratoma (mean age 72 months). Four had bilateral synchronous metastases and thoracotomies, and one had bilateral metachronous metastases and thoracotomies. Nineteen children were discharged well within 10 days of surgery. There was one early complication: a death due to pneumonia. Four children subsequently died postoperatively with cranial metastases (mean 29 months postoperatively). The remaining 16 children remain alive and well. As part of the combined therapy, these results would support an aggressive surgical approach to this disease. Preoperative assessment should include contrast enhanced computed tomogram of the head and chest as well as chest X-ray taken immediately preoperatively to exclude metastases. Bilateral synchronous and metachronous thoracotomy is well tolerated in childhood.

A histological study of the hph-1 mouse mutant: an animal model of phenylketonuria and infantile hypertrophic pyloric stenosis.

AIM: To quantify the chronological sequence of changes in the morphology and immunoreactivity for neurotransmitters in the pylorus of an animal model of infantile hypertrophic pyloric stenosis and phenylketonuria. METHOD: Thirty specimens of pylorus from hph-1 mice and age/sex matched controls (age range: 10-180 days) were examined using conventional histology and immunohistochemistry for a variety of antigens: protein gene product 9.5, a pan neuronal marker; vasoactive intestinal polypeptide; nitric oxide synthase two antigens coalesced to the same inhibitory neurons in humans; substance P, a potent excitatory neurotransmitter; and calcitonin gene related peptide, a neurotransmitter implicated in the somatic afferent innervation of the stomach. The changes in the morphology of the muscle layers were quantified and statistically analysed for each age group (10, 20, 40, 90 and 180 days). RESULTS: Between 10 and 90 days of age, all muscle layers of the hph-1 mice were hypertrophied, for example, 10 days, hph-1 longitudinal muscle mean diameter = 3.4, control = 1.8; hph-1 circular muscle width = 11.5, control = 4.7. The hph-1 mice were significantly smaller during this period (40 days, hph-1 weight = 10 g, control = 25 g). There was no change in the pattern of expression of the antigens examined within the hph-1 mice compared with the controls. CONCLUSION: Hph-1 mice develop a transient smooth muscle hypertrophy of the pylorus attended by gastric distension and failure to gain weight. These changes resolve as the pyloric muscle hypertrophy resolves.

Cleft sternum: case report and literature review.

The isolated sternal cleft is a rare congenital anomaly that presents from birth to adulthood. We describe the late presentation and management in a child with this condition. The modern investigative and operative options are reviewed.

Axillary lipoblastoma--tumor recurrence in the right atrium.

This case report describes, for the first time, vascular invasion and recurrence of a lipoblastoma 6 months after the macroscopically complete excision of the initial cervico-axillary tumour. The importance of cytogenetics in the diagnosis of lipoblastoma is emphasized, as is the need to be wary of the diagnosis of lipoma in infancy.

A quantitative study of the neural changes underlying pyloric stenosis in dogs.

This study aimed to quantify the neural changes in congenital pyloric stenosis in dogs and to study the comparative anatomy between this condition in dogs and that in infantile hypertrophic pyloric stenosis. Eight specimens from the pylorus of dogs with pyloric stenosis and six control specimens were examined using conventional histology and immunohistochemistry for a range of neural antigens. The changes in the proportion of nerves immunoreactive for each antigen were quantified and analysed statistically. The morphology of the nerves in the diseased dogs was similar to that in controls. Only vasoactive intestinal peptide was reduced in expression in dogs (median proportion in control dogs 0.57, in diseased dogs 0.17; P = 0.065). This study demonstrates both morphological similarities and significant differences between closely related conditions in dogs, humans and other species.

Hunterian Lecture. The ontogeny of the peptide innervation of the human pylorus with special reference to understanding the aetiology and pathogenesis of infantile hypertrophic pyloric stenosis.

Infantile hypertrophic pyloric stenosis is the most common cause for urgent abdominal surgery in infancy. The aetiology of the condition is unknown. The ontogeny of the innervation and structure of the normal infant pylorus is unknown. A variety of differing histological features have been attributed to this condition and a number of animal models have been described. The histological changes in the human condition and those in the animal models have not been quantified and statistically verified. Thus, precise comparisons cannot be made. Immunohistochemistry was the principal technique employed in this study. Using this technique, the ontogeny and structure of the normal infant pylorus have been documented. The morphological and immunohistochemical changes underlying infantile hypertrophic pyloric stenosis have been quantified for the first time and compared with the quantified changes in natural and experimental animal models of this condition.

A quantitative study of the morphological and histochemical changes within the nerves and muscle in infantile hypertrophic pyloric stenosis.

PURPOSE: The aim of this study was to quantify changes in dimensions of nerves and muscle and the proportionate expression of neural antigens in infantile hypertrophic pyloric stenosis (IHPS). METHODS: Twenty specimens of pylorus from children with IHPS and age/sex-matched controls were examined using conventional histology and immunohistochemistry for a range of nerve and muscle antigens. The changes in the proportion of nerves expressing each antigen were quantified and statistically analyzed. RESULTS: The longitudinal muscle was found to be hypertrophic and protein gene product 9.5-stained nerves appeared longer and thicker in the myenteric plexus and shorter in the longitudinal muscle layer in IHPS. The proportion of nerves that expressed neural nitric oxide synthase (nNOS) was found to be diminished in all the IHPS tissues examined. In the circular muscle and myenteric plexus, the proportion of nerves that expressed vasoactive intestinal polypeptide (VIP) and nNOS was almost identically diminished. The expression of calcitonin gene-related polypeptide and substance P was proportionately reduced in the myenteric plexus. CONCLUSIONS: The results of this study represent the first quantitative analysis of nerves and muscle in IHPS. The muscle hypertrophy is not restricted to circular muscle layer. The changes in nerve morphology cannot be attributed to a dilutional effect of the muscle hypertrophy. The selective changes in nerve and ganglion morphology varies between tissue layers and neural antigen expressed. The findings of reduced proportions of nerves expressing, in particular, nNOS may shed some light on the etiology of this condition.

The ontogeny of innervation of the human pylorus.

PURPOSE: The aim of this study was to document the vagal innervation and expression of neuropeptides, neuronal nitric oxide synthase (nNOS), and neural cell adhesion molecule (NCAM) in the neuromuscular system of the developing human pylorus. METHODS: Specimens of human pylorus (n = 54; age range, 8 weeks' gestation to 6 months postnatal) were studied. Vagal innervation was determined by Dil autofluorescence. A wide range of neuropeptides, NCAM, and the neural isoform of NOS were examined by immunohistochemistry. RESULTS: Vagal innervation was first recognized in the myenteric plexus in the 12-week-old fetus as was vasoactive intestinal polypeptide (VIP) expression. Neuropeptides were present from 8 weeks' gestation and appeared to be expressed progressively from the adventitia toward the mucosa and showed an adultlike profile by 23 weeks' gestation. A craniocaudal pattern of expression was noted for VIP and nNOS. Alpha smooth muscle actin was expressed by muscle fibers of the muscularis propria from 8 weeks and the muscularis mucosae by 14 weeks. All the isoforms of NCAM examined were expressed from 8 weeks in the muscularis propria and by 12 weeks in the submucosa. CONCLUSION: The expression of the antigens studied correlated with the gestational age and development of the pylorus.

The ontogeny of the peptide innervation of the human pylorus, with special reference to understanding the aetiology and pathogenesis of infantile hypertrophic pyloric stenosis.

Pyloric stenosis (PS) is a common condition in infancy, which is associated with smooth muscle hypertrophy that results in pyloric outlet obstruction. The author examines the ontogeny of the peptide innervation of the pylorus in fetal tissues and an experimental model in mice and evaluates the histochemical and morphological changes in the pylorus. The data suggest that PS is an intrauterine lesion that occurs by 12 weeks' gestation. This is associated with diminished nitric oxide in human tissues and reduced enzyme activity (resulting from a deficiency in an enzyme cofactor) in mice. Increased vasoactive intestinal polypeptide expression in pyloric myenteric ganglia may be an intrinsic mechanism for resolving this condition.

Polytetrafluoroethylene graft for spontaneous coronary dissection: 7-year follow-up.

Spontaneous coronary artery dissection remains an exceedingly rare cause of myocardial ischemia. The patients are usually young and female, and the dissection is frequently fatal. The use of polytetrafluoroethylene as an aortocoronary conduit is generally followed by early occlusion. We report a case of spontaneous right coronary dissection in which a polytetrafluoroethylene graft was placed that was observed to remain patent by angiography at least 72 months after operation.

Leutic aortic aneurysm. Presentation of acute illness.

Leutic aortitis no longer is a forgotten clinical entity. A patient with an expanding aneurysm of the aortic arch required urgent surgical resection during the acute phase of the disease.

Giant right atrial myxoma with rheumatic mitral valve disease.

A patient with a 456 Gram right atrial myxoma is described. Associated rheumatic mitral valvular heart disease obscured the presence of the tumor and only with presentation of bradyarrhythmias leading to pacemaker implantation was the diagnosis suspected. Successful surgical removal of the tumor and correction of the intracardiac valvular dysfunction were performed. The role of echocardiography in the early detection of these tumors is reinforced.

A four-year experience with preoperative noninvasive carotid evaluation of two thousand twenty-six patients undergoing cardiac surgery.

From January 1979 through December 1982, 2026 patients scheduled to undergo open heart surgery were evaluated by a preoperative battery of noninvasive carotid tests including phonoangiography, oculopneumoplethysmography, pulse-timing oculoplethysmography, periorbital Doppler examination, and during the last 12 months, continuous-wave Doppler ultrasonography with spectral analysis. The incidence of hemispheric neurologic deficit following cardiac surgery in the 47 patients with carotid disease was 14.9%; the incidence in patients with no carotid disease was 1.9% (p less than 0.001). Fourteen of the 47 patients were not candidates for carotid surgery because of unilateral occlusion in 13 and bilateral occlusion in one. Three of the 14 (21.4%) had intraoperative strokes on the appropriate side. Thirty-three of the 47 had operable carotid disease. Four with unilateral stenosis had no carotid surgery; one had a postoperative deficit on the side referable to the nonstenotic artery. Eighteen with unilateral stenosis underwent simultaneous cardiac and carotid surgery; one (5.6%) had a transient deficit. Seven patients with bilateral stenosis underwent cardiac and unilateral carotid surgery; no deficits occurred. Four patients with unilateral stenosis and contralateral occlusion underwent combined surgery; one had a transient ischemic attack and one a fatal stroke, both referable to the hemisphere ipsilateral to the occlusion. It appears that the presence of carotid disease increased the risk of stroke during heart surgery. Proof that carotid endarterectomy lowers this risk awaits a prospective randomized trial.

Hemodynamic effects of intravenous 20% soy oil emulsion following coronary bypass surgery.

Because of its high caloric density, intravenous fat emulsions have been suggested as useful sources of calories and essential fatty acids in patients with serious heart disease in whom fluid restriction must be closely monitored. Previous studies in the experimental animal have suggested a myocardial depressant effect of intravenous fat emulsions at high infusion rates. In the present study, 19 adult patients, following uncomplicated isolated coronary artery bypass surgery, were divided into two groups. A constant infusion of 2 ml/min of soy oil emulsion (20% Intralipid) was administered to the first group. The second group received 20% Intralipid at 1 ml/min followed by a 2 ml/min infusion. In the group receiving the 2 ml/min infusion (averaging 5.25 mg/kg/min), significant decreases in cardiac output and increases in pulmonary capillary wedge pressure occurred. One patient suffered an adverse side effect which may have been related to myocardial ischemia. The second group of patients received an initial infusion of 1 ml/min (averaging 2.35 mg/kg/min) following which the rate was doubled. No significant hemodynamic changes or adverse side effects occurred in the second group. It is concluded that 20% soy oil emulsion can be administered safely to the recently postoperative cardiac surgical patient recovering from coronary bypass grafting, but the rate should not exceed the maximum clearance rate of 1 ml/min (2.67 mg/kg/min).

Continued propranolol administration following coronary bypass surgery. Antiarrhythmic effects.

One hundred consecutive patients requiring propranolol hydrochloride before undergoing isolated aortocoronary bypass procedures were examined. In half the patients, propranolol therapy was discontinued, whereas the other half continued to receive intraoperative and postoperative propranolol regardless of clinical events. Although there were no preoperative differences in the apparent degree of coronary arterial disease or left ventricular function in the two groups, postoperative supraventricular arrhythmias were less frequent in the propranolol-treated group, most noticeably in those receiving less than 320 mg preoperatively. In patients who had received large preoperative doses (greater than or equal to 320 mg/day), there were no significant differences in postoperative supraventricular tachycardias. Continued propranolol therapy following isolated coronary bypass surgery appears to be a safe and efficacious method of decreasing the incidence of postoperative supraventricular tachycardias.

Should nutritional status be assessed routinely prior to cardiac operation?

One hundred consecutive adult patients undergoing cardiac operations at a single institution were evaluated preoperatively with regard to their nutritional status. Anthropometric, biochemical, and immunologic characteristics were evaluated in addition to cardiac biopsy specimens to determine right atrial glycogen concentration. Although some positive anthropometric, biochemical, and cell-mediated immunity characteristics were observed to have "statistically significant" correlations with morbidity and mortality for the group as a whole, nearly all of the values remained near or at normal limits. Lighter weight men with a smaller arm muscle circumference and lower concentration of total body fat had more complications than their heavier counterparts. Serum transferrin and cell-mediated immunity also formed weakly positive statistical correlations. Anthropometric correlations in women were of no value. Myocardial glycogen concentrations did not correlate with postoperative morbidity and mortality. Because nearly all of the patients had arteriosclerotic heart disease, the series as a whole may have been skewed toward a group with values too close to normal to differentiate them adequately. It is concluded that routine nutritional assessment is of no value in guiding nutritional management for individual patients, although when patients are analyzed as a group, interesting epidemiologic observations can be made.