Cryptorchidism in boys with imperforate anus

In a retrospective study of the case reports of 136 boys who were operated on for an imperforate anus and who survived at least 18 months, cryptorchidism was the most common associated anomaly, involving 26 cases (19%). The higher the level of the anorectal malformations, the higher was the incidence of cryptorchidism. The incidence of renal and ureteric malformations and dysplasias showed a parallel tendency. The incidence of vertebral malformations and dysplasias in the T10-S5 area was low among patients with a covered anus or a perineal fistula. Cryptorchidism was found associated with urological and with T10-S5 vertebral malformations and dysplasias. Recognition of this association is probably new. The histopathological findings of testicular biopsy specimens and the location of the undescended testes in patients with an imperforate anus showed the same pattern as seen in undescended testes from patients with cryptorchidism only. These findings, together with the existing literature on the subject, indicate that further studies on the association of cryptorchidism, urological, and T10-S5 vertebral malformations and dysplasias may be very helpful toward a better understanding of cryptorchidism in general.     

Congenital megalourethra associated with urethral duplication and imperforate anus

A newborn with a prenatal diagnosis of right hydroureteronephrosis and enlarged penis is presented. At birth, the baby had an imperforate anus (IA) with a megalourethra; radiologic and ultrasonographic studies showed a left polycystic kidney and right hydroureteronephrosis, right vesicoureteral reflux, and an incomplete urethral duplication with dilatation of the posterior urethra. The IA was corrected on the 1st day of life and a vesicostomy was performed at 1 month. At 1 year of age the valve obstructing the ventral posterior urethra was resected and the vesicostomy was closed. At 14 months the baby underwent a urethroplasty with a vertical preputial tubularized island flap and excision of the penile urethral duplication. Exact knowledge of the malformation was essential in planning the appropriate surgical treatment.     

Cloaca, the most severe degree of imperforate anus: experience with 195 cases

OBJECTIVE: To provide a follow-up of 195 patients with cloacal malformations seen by the author from 1959 to 1998. SUMMARY BACKGROUND DATA: Cloaca, which occurs in approximately 1 of 50,000 births, is the most complex type of imperforate anus with confluence of the rectum, vagina, and bladder in a urogenital sinus. Functional results for the bowel, the genital tract, and the urinary tract were formerly poor. Cloacal exstrophy, which is an even more complex spectrum of malformations, was uniformly fatal until 1960. In addition to imperforate anus, these babies have an omphalocele, two exstrophic bladders, between which there is an open cecum, and a blindly ending colon hanging down in the pelvis from the cecum. Although both of these diagnoses contain the word "cloaca," which is Latin for sewer, they are really two separate entities in terms of surgical management. Cloaca and cloacal exstrophy in most cases are very different anatomic problems. However, there are variants that are like a hybrid, which is the rationale for reporting together an experience with both entities. METHODS: Records were reviewed of 154 patients with cloaca and 41 patients with cloacal exstrophy to assess anorectal function, urinary continence, and sexual function where available. RESULTS: Follow-up was available in 141 cloaca patients: 82 have spontaneous bowel movements and satisfactory control, 38 use enemas to evacuate, 9 have a colostomy, 7 have fecal soiling, and 5 are too recently operated to evaluate. Regarding urinary control, 83 void spontaneously, 40 catheterize to empty, 4 have urinary diversion, 1 has a continent diversion, 5 patients are wet, and 8 are too recently operated to judge. Twenty-four patients are now adults, 17 of who have experienced coitus and 7 have not. Seven have had babies, all except one by cesarean section. Results of surgery for cloacal exstrophy are not as good, but are encouraging nonetheless for an anomaly that was uniformly fatal before 1960. Of the 41 cloacal exstrophy patients being followed, 7 have not undergone surgery. Fifteen have a colostomy; 19 had pull-through of the colon, but 3 were subsequently reversed for fecal incontinence. Most depend on enemas to evacuate. Urinary dryness was attained in 30 patients, usually by intermittent catheterization of the bladder, which was augmented with small bowel or stomach or both. Only three void voluntarily. Fifteen of the completed long-term patients wear no bag. Only three of the completed patients wear two bags. The rest have one bag. CONCLUSIONS: Imperforate anus and associated malformations in cloaca and cloacal exstrophy are not hopeless problems. A reasonable lifestyle can be achieved for most of these children with comprehensive surgical planning.     

Bifid scrotum, perineal hamartoma and high imperforate anus: a case report

This is a case report of a newborn patient with imperforate anus, urethro-colonic fistula, perianal hamartoma, and bifid scrotum. Successful staged repair of these anomalies is described together with review of the embriology related to the case.     

Tethered cord and associated vertebral anomalies in children and infants with imperforate anus: evaluation with MR imaging and plain radiography

PURPOSE: To evaluate in children and infants with imperforate anus the prevalence and types of occult myelodysplasia that may result in tethered cord and the association of these lesions with vertebral anomalies. MATERIALS AND METHODS: Records and images were retrospectively reviewed in 86 patients who underwent spine magnetic resonance imaging and had either low-level imperforate anus (n = 30), intermediate-level imperforate anus (n = 15), or high-level imperforate anus (n = 41). RESULTS: Thirty-one of 86 patients (36%) had occult myelodysplasia suggestive of tethered cord (27% of all patients with low-, 33% of all patients with intermediate-, and 44% of all patients with high-level lesions). Of these 31 patients, 16(52%) were asymptomatic, 24, (77%) had a thickened fatty filum, 13 (42%) had normal conus position, and 23 (74%) had vertebral anomalies. Twenty-four of the 31 patients (77%) underwent surgical untethering. CONCLUSION: A substantial number of patients with all types of imperforate anus have occult myelodysplasia that may necessitate surgical intervention, including those patients with normal spine radiographs.     

Abdomino-anal resection of the colon in cancer of its lower ampullar section invading into the anus]

The special comprehensive multidisciplinary programme for tuberculosis control was used as a basis for planning. The developed special comprehensive interdepartmental programme for tuberculosis control in the region is aimed at involving all services and departments in whom tuberculosis control should be a national, official, professional duty. A multidisciplinary approach to organizing and making antituberculous measures is especially required as antituberculous institutions have the shortest funds now. The programme has approved by the regional administration.     

Arteriovenous malformation associated with moyamoya disease

The first case of a child with an arteriovenous malformation (AVM) associated with moyamoya disease is reported. The patient presented ischemic symptoms and underwent indirect bypass surgery on both sides when she was 5 years old. Four years later she suffered from headache, and a small AVM of the left frontal lobe associated with the moyamoya vessels was detected. Single photon emission computed tomography (SPECT) was performed at age 11 and demonstrated low local cerebral blood flow (CBF) in the left frontal lobe and right temporal lobe, although the revascularization after the bypass surgery seemed to be effective, as judged on pancerebral angiography. We feel that brain ischemia due to the moyamoya disease may have played a causative role in the development of the AVM.     

Anteriorly located anus: is constipation caused by abnormal location of the anus?

Anteriorly located anus (ALA) is frequently associated with severe constipation accompanied by defecation pain. Between 1988 and 1994, the authors treated 27 children (26 girls, 1 boy; age range, 0 to 11 years) to surgically correct ALA. The operation was performed according to a uniform protocol to longitudinally divide the internal sphincter muscle from the anal skin level to 2 cm above the dentate line on the posterior wall of the anorectum. For anal reconstruction, any of the conventional procedures was employed. Twenty-two of the 27 patients have had follow-up in our clinic for 12 months to 6 years (mean, 2.75 years). Eighteen are completely free of constipation and defecation pain and have regular spontaneous bowel movements. The other four require occasional use of enemas or laxatives. Anal incontinence did not occur in any patient. The results of this study suggest that abnormal function of the internal sphincter is the most likely cause of constipation or defecation pain in patients with ALA and that internal sphincterotomy is the cornerstone of surgical treatment.     

Imperforate anus: nutritional care

The authors analyze an experience with treatment of 2750 patients with different forms of nonlactational mastitis. Special attention is given to peculiarities of the clinical course of the disease, importance of early diagnosis of the inflammation in the mammary gland. Pathogenetical treatment of different forms of nonlactational mastitis is substantiated. Dissection of all pathogenetically altered tissues is thought to be necessary in operative treatment of patients with purulent forms with the following histological investigation.     

Pial arteriovenous malformation with massive perinidal edema

The dental, clinical, radiological, pedigree and dermatoglyphic findings of a patient showing hypoplasia of enamel and intrinsic staining of the teeth from erythroblastosis fetalis are presented.     

Factors associated with successful arteriovenous malformation radiosurgery

OBJECTIVE: To analyze the clinical and angiographic variables that affect the results of arteriovenous malformation (AVM) radiosurgery and to propose a new method of reporting patient outcomes after AVM radiosurgery. This method incorporates both the obliteration status of the AVMs and the postoperative neurological condition of the patient. METHODS: Patient outcomes were defined as excellent (nidus obliteration and no new deficits), good (nidus obliteration with a new minor deficit), fair (nidus obliteration with a new major deficit), unchanged (incomplete nidus obliteration without a new deficit), poor (incomplete nidus obliteration with any new deficit), and dead. Two hundred twenty patients who underwent AVM radiosurgery at our center before 1992 were subjected to a multivariate analysis with patient outcomes as the dependent variable. RESULTS: Multivariate analysis determined four factors associated with successful AVM radiosurgery: smaller AVM volume (P=0.003), number of draining veins (P=0.001), younger patient age (P=0.0003), and hemispheric AVM location (P=0.002). Preradiosurgical embolization was a negative predictor of successful AVM radiosurgery (P=0.02). CONCLUSION: AVM obliteration without new neurological deficits can be achieved in at least 80% of patients with small volume, hemispheric AVMs after single-session AVM radiosurgery. Future studies on AVM radiosurgery should report patient outcomes in a fashion that incorporates all the factors involved in successful AVM radiosurgery.