Biomedical subjects
Publications and source records attributed to A Wakhlu.
Ovarian neoplasms are unusual in the paediatric age group; the majority of them are of germ cell origin. Malignant epithelial tumours of the ovary occur infrequently in adolescent girls. Ovarian carcinoma in particular is extremely rare before puberty. The authors describe 3 cases of adenocarcinoma of the ovary in premenarchal girls and highlight the unique characteristics of this tumour in this age group.
Single dose vaccination was carried out with Mycobacterium habana vaccine, 31 lepromatous leprosy cases receiving 1.5 mg (1.5 mg = 6.27 x 10(8) bacilli) and 36 household contacts randomly receiving 1.5, 2.0, 2.5 mg vaccine intradermally. Duration of study was 18 weeks. Vaccination induced lepromin conversion in 100% of lepromatous leprosy cases and lepromin negative household contacts and augmentation of lepromin reactivity in 100% of lepromin positive household contacts, which was stable for the 15 weeks duration of follow-up. The maximum augmentation in lepromin reactivity was obtained with 1.5 mg of vaccine, which is probably the supramaximal dose. Overall, post-vaccination, those without prior BCG vaccination scars showed higher mean values of lepromin augmentation. Local vaccination site changes included induration, ulceration, itching, pain and uncomplicated regional lymphadenopathy, all of which remitted spontaneously by 15 weeks. Systemic side-effects noted were pyrexia, ENL and jaundice, and were seen with no greater frequency than that reported in other vaccine trials. Overall, systemic side-effects were easily controlled and were not accompanied by clinically detectable nerve or ocular damage. The safety profile investigations revealed an increase in the mean values of Hb%, RBC count and PCV in household contacts and of PCV in lepromatous patients, post-vaccination. Alterations in the liver function tests were also observed in patients of lepromatous leprosy. Thus, M. habana vaccine appears to be useful in stimulating specific CMI against M. leprae as evidenced by increased lepromin reactivity.
A retrospective review of seven patients with teratomas of the head and neck treated at out center over the past 5 years, which represented 2% of all teratomas (sacrococcygeal, ovarian, and retroperitoneal) seen over the past 20 years, was performed. After investigation to exclude associated anomalies, all but one of the children underwent surgery for removal of the tumour. All excised tumours were subjected to histopathological examination to confirm their teratomatous nature. The patients were followed up at regular intervals for up to 4 years. The patients' ages ranged from newborn to 2.5 years. There were three cervical, two oral, and two skull teratomas. The youngest patient had a cervical teratoma with respiratory compromise, requiring tracheal intubation. All but one patient (skull teratoma) had excision of the tumours with a satisfactory outcome. Histopathological examination of the excised tumours showed mature tissue from the three germinal layers in all specimens. Specific components included glandular epithelium, keratinising epithelium, and muscle fibres. Follow-up did not show any recurrence in the operated children. The three with cervical teratomas had normal levels of thyroid hormone postoperatively. Head and neck teratomas in children are mostly benign lesions amenable to curative excision. In cervical teratomas airway management takes priority.
Congenital lumbar hernia (CLH) is a rare anomaly with only 45 cases reported in the English-language literature. This paper describes nine patients with CLH treated in our unit. Unusual features included the relatively high incidence of inferior lumbar hernia, presentation at the age of 6 years in one case, and an association with hydrometrocolpos and anorectal malformation, which is hitherto unreported. In seven patients the hernia could be repaired successfully. One patients' parents refused surgery for the CLH after treatment of a hydrometrocolpos and another died of fulminant pneumonia before the operation. Early operation is the treatment of choice, and repair with local tissues is preferable. The need for prosthetic material arises when the size of the defect is large. A successful operation offers a good quality of life.
BACKGROUND/PURPOSE: The management of exomphalos in the authors' department over a 26-year period is reported together with a technique for delayed closure of the ventral hernia resulting from conservative treatment of exomphalos major. METHODS: Patients were classified into exomphalos minor and major. Exomphalos minor was treated by early surgical closure. Exomphalos major was treated preferentially conservatively with delayed repair of the ventral hernia. RESULTS: There were 104 patients (68 boys and 36 girls; exomphalos minor, 45; exomphalos major, 59). Forty-two patients with exomphalos minor underwent operation. Three patients died before surgery, and 9 others postoperatively of overwhelming sepsis. Fifteen babies with exomphalos major needed early operation (skin closure only in 3 and prolene mesh repair in 12), there were 2 preoperative and 4 postoperative deaths. Forty-two patients were treated conservatively, among these, 8 died of sepsis. Thirty-four children had closure of the ventral hernia (prolene mesh, 7 and native tissue, 27); there was no morbidity. Two children died after laparotomy for adhesive intestinal obstruction. CONCLUSION: Mortality rate was related to sepsis, complications of delayed presentation, and severe congenital anomalies. There were no ill effects attributable to mercury or iodine absorption. Delayed ventral hernia repair by double breasting of the fibrous tissue sheet underlying the skin was found to be a reliable technique with low morbidity.
BACKGROUND: H-type urethral duplication is the most complex form of this uncommon anomaly. METHODS: This report describes the treatment of four such patients, three of which had hypoplasia of the entire anterior urethra, and one had anterior fusiform megalourethra. RESULTS: Satisfactory results were obtained in all four by staged surgical management in which the anal opening was transposed to the perineum, preserving the continence mechanism. Subsequently, urethroplasty was performed. In one patient, the megalourethra was tailored and incorporated into the urethroplasty in a third-stage procedure. All patients were well on follow-up. CONCLUSION: With early treatment, good results can be achieved in this condition.
UNLABELLED: OBJECTIVES To correlate renal function with the site of the ectopic orifice in patients with a single ectopic ureter and to evaluate the role of ureteric reimplantation in the preservation of renal function. PATIENTS AND METHODS: Forty-four patients (41 female, age 1.5 months to 20 years) with a single ectopic ureter have been managed in our institution in the last 21 years. The classical symptom of continuous wetting with intermittent normal micturition was reported in most of the female patients. The investigative evaluation included intravenous urography (i.v.U), cysto-urethroscopy, vaginoscopy with retrograde ureteric catheterization, micturating cysto-urethrography (MCU) and ultrasonography. Diuretic renography was carried out in four patients after it became available in 1992. Renal function was assessed in relation to urinary tract anomalies and with outcome after ureteric re-implantation. RESULTS: Thirty-eight patients (two males) had a unilateral ectopic ureter; the ectopic orifice was vaginal in 12, vestibular in 11, urethral in nine, at the bladder neck in two, the seminal vesicle in one and undetermined in three. Twenty-one patients had renal and/or ureteric abnormalities, with reflux detected on MCU in three ureters. Associated anomalies included hypospadias (two, one female), skeletal anomalies (two), anorectal malformations (three), cryptorchidism (two), and unilateral cystic ovary (one). Two patients had preoperative hypertension. In 15 patients, renal function was considered sufficient to justify ureteric reimplantation, 14 of whom regained continence. One girl had suprapubic leakage from the bladder and died during secondary nephroureterectomy. Another girl had persistent incontinence; she was found to have contralateral duplex ureters with a vestibular ectopic orifice and was cured after upper polar heminephroureterectomy. i.v.U and renography carried out in two patients each within 4 weeks of surgery showed a moderate improvement in renal function. Eight patients reported for follow-up after ureteric reimplantation (mean duration 11 months); none had hypertension or urinary infection. Twenty-three patients with rudimentary kidneys underwent nephroureterectomy. Histopathological examination of the excised kidneys showed moderate to severe dysplasia with chronic pyelonephritis. Six patients (one male) had bilateral single ectopic ureters, with normal renal function in the five females. Unilateral reimplantation in the boy resolved the symptoms; one girl died before surgery and the other four underwent bilateral ureteric reimplantation, after which one was dry for up to 3 h while the other three were incontinent, one of whom subsequently underwent urinary diversion. CONCLUSIONS: There was no clear correlation of renal function with the site of the ectopic ureteric orifice, as most of the patients with a vaginal ectopic ureter had sufficient renal function to justify renal preservation. Ureteric reimplantation preserved renal function, although the improvement after surgery was determined by the degree of renal dysplasia.
Perineal canal (PC) is a rare anomaly constituting 4% of all anorectal malformations. Sixty patients (56 females and 4 males) with PC managed over the past 27 years are reported. The ages ranged from 2 days to 13 years. The chief symptom was passage of fecal matter through both the anus and the fistula. One girl had undergone previous, unsuccessful surgery. All our patients were treated by anterior sagittal anorectoplasty (ASARP), which allowed anatomic exposure and accurate repair of the anomaly. In 49 patients without any perineal inflammation primary ASARP was undertaken. Surgery was delayed in 11 patients with perineal excoriations and/or active inflammation. One patient died post-operatively due to unrelated causes and 1 developed a recurrence. Anal dilation was required in 7 cases. Fifty patients were seen at first follow-up 12 weeks after surgery. All were continent and had normal defecation without the use of laxatives. Thirty-four could be followed up to the age of 3 years; they were continent with normal bowel habits. There was no shift in the position of the anus and no instance of rectal dilation. Individualization of the management and operation by the anterior sagittal approach thus offers good results in this uncommon anorectal anomaly.
Congenital short colon (CSC) is a condition in which the colon is replaced, wholly or partially, by a dilated pouch together with anorectal malformation and a colourinary fistula. Most of the reported series have been from northern India where this malformation is frequent. This paper details the management of 108 patients with CSC over a period of 23 years. The patients were classified into two types: (1) Partial short colon, where a segment of normal colon is present between the ileum and the sac. These patients could be treated by excision of the pouch and pull-through of the segment of normal colon during either single- or two-stage surgery. (2) Complete short colon, where the ileum opened directly into the sac, and formation of a tube from this sac (coloplasty) was required in one or more stages to provide a length of normally functioning colon. Review of the results showed staged management to be superior. The follow-up has ranged between 3 and 7 years, with satisfactory long-term results in both groups of patients. We have evolved a protocol for the management of CSC that has improved the prognosis and quality of life of these patients.
Vestibular fistula and perineal ectopic anus are the most common anorectal malformations in female children. Anterior saggital anorectoplasty (ASARP) was used to treat 416 patients with these anomalies, as well as cases of perineal canal and third-degree perineal tear, during a 20 year period. Preliminary colostomy was performed in only four patients, who had a perineal tear. For ASARP, a midline incision was made and the rectum was separated from the vagina and placed in the center of the sphincteric muscle complex. The perineal body was reconstructed, and normal appearance of the perineum was achieved. Six patients had complications postoperatively (4 had secondary hemorrhage, 2 had wound infection). One patient with hemorrhage and one with infection required laying open of the wound and revision surgery after 12 weeks. Both recovered well. Of the 416 patients, 397 returned for follow-up 12 weeks after the surgery. Of these, 390 had a normal-looking perineum and normal defecation. Of the remaining seven patients, three had retraction of the rectum, one had recurrence of the fistula, and another had acquired perineal canal. These five children required revision ASARP and recovered successfully. The other two patients had mucosal prolapse, which reduced spontaneously. Three hundred twenty-six children of the 416 patients have had follow-up beyond the age of 3 years. Of these, 291 had normal bowel habits without the use of laxatives. Of the other 35, four had fecal impaction of unknown etiology, which was managed conservatively, 6 had anal stenosis (four underwent posterior Barrow's flap interposition and two responded to dilatation), and 25 had a posterior ledge that caused constipation and required cutback. Overall, eight patients (1.9%) required revision ASARP and 40 others (9.8%) had minor complications. This operation provides consistently good results, and the authors recommend it for the management of anorectal malformations and perineal trauma in female children.
Seventy-two patients with complete short colon (CSC) were treated at the authors' institution between 1971 and 1994. Initially, one-stage coloplasty with abdominoperineal pull-through was performed in 10 neonates, with a 50% survival rate. The subsequent 62 patients had staged management in which a window colostomy was performed at the time of presentation. Of the 50 survivors in this group, 20 had coloplasty with a abdominoperineal or sacroabdominoperineal pull-through by June 1994. A protective ileostomy was done at the time of coloplasty in 10 patients, and was closed 6 to 12 weeks later. Two of the 20 patients who had staged reconstruction died because of complications; the others recovered well. The overall survival rate after coloplasty was 76.7%. Eighteen of the 23 survivors have had follow-up for up to 8 years. They have shown normal growth and good continence and have been free of significant long-term complications. The other 30 patients are awaiting definitive surgery. A staged management plan is advocated for complete short colon because it provides consistent results and good quality of life.
The authors report on two patients who had fusiform megalourethra with unusual features. The first patient had left hydronephrosis with duplication of the ureter, oblique sagittal septum in the urinary bladder, and Y duplication of urethra with megalourethra of both channels. The second had features of the abdominal muscle deficiency syndrome, with anterior ectopic anus, rectourethral fistula, and fusiform megalourethra. Both patients were treated by preliminary urinary diversion. The first patient subsequently had staged reconstruction. The other patient died of urinary sepsis. The authors believe that preliminary urinary diversion followed by planned staged reconstruction offers hope for the reconstruction and rehabilitation of these cases.
Endoscopic retrograde cholangiopancreatography (ERCP) was performed in eight consecutively treated patients with choledochal cyst to assess its technical feasibility and complications. Satisfactory visualization of the complete anatomy of the biliary tract was possible in seven patients. Four patients had a type IV choledochal cyst and three had type I. In one patient, the procedure showed a congenital web in the lower end of the common bile duct; in another, there was complete membranous block of the lower end of the common bile duct. The findings of ERCP and intraoperative cholangiography were concordant for six patients. Only one patient had exacerbation of preexisting cholangitis. The authors conclude that ERCP is an essential investigative method in the management of choledochal cysts in children.
The authors report an unusual case of anal agenesis associated with rectal atresia. Such an anomaly has been reported only once before. Coincidentally, this patient also had a rarely encountered skeletal anomaly--unilateral cleft foot.
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