Case Snippets

frequent location. Gastrointestinal and retroperitoneal sites were each identified in 12 percent of cases.1 There is a bimodal age incidence, with a small cluster of cases occurring in childhood.2 A female-to-male preponderance of 4:1 is reported. The cardinal histologic finding is the presence of numerous histiocytes with pathognomonic intracellular and extracellular inclusions known as Michaelis-Gutmann bodies, which typically stain positively with periodic acid-Schiff, von Kossa, and Prussian blue stains.
An association between immunosuppression and malakoplakia has been documented.3 Chronic granulomatous disease (CGD) is a primary immunedeficient
state manifested by defective phagocytosis.
4 Celiac disease is also believed to be due to immune dysfunction.5
A 4-year-old boy was hospitalized for chronic diarrhea,
severe malnutrition and abdominal pain. He was a normal 3300-gram neonate at birth with normal growth and development during the first year of life. He had been hospitalized one year ago for chronic diarrhea. At that time anti-endomysial antibody test was positive twice and duodenal biopsy showed moderate duodenitis with moderate
villous atrophy. He was on gluten-free diet, but because
of no response over 9 months, his parents discontinued
the regimen.
His brother had had chronic intractable diarrhea and recurrent skin infections with no response to gluten-free diet. He died due to an unknown infection at age 6 years.

On examination he was a cachectic child with 9.5 Kg weight, 85 cm height and 49 cm head circumference. He had pale conjunctiva, brittle hair, distended abdomen and no organomegaly. His mental status was normal.
Stool examination was negative for parasites. Immunologic
assessment was near normal (35% T cells, 28% B cells, 74% CD4, 17% CD8) except for positive nitro-bluetetrazolium
test. Anti-HIV and anti-HTLV1 were also negative.
On colonoscopy he had erythematous mucosa in the rectosigmoid and descending colon; biopsy showed Michaelis-Gutmann bodies.
After starting ciprofloxacin followed by cotrimoxacole,
on regular diet, his diarrhea stopped. His weight increased by 2.5 Kg after one month but bloating and abdominal distension did not resolve. We reassessed him for celiac disease. Anti-tissue transglutaminase test was positive. Duodenal biopsy showed partial villous atrophy;
cryptosporidium infestation was also seen.
To our knowledge, this is the first case reported
of gastrointestinal malakoplakia in a child, occurring with CGD and celiac disease.
The etiology of malakoplakia is unclear. Current evidence points to a defect in macrophage killing activity. Nondigested micro-organisms are found within the lysosomes of macrophages in affected persons. Macrophages from these patients show a decrease

found to have a single colonic polyp, which was removed; biopsy revealed adenomatous polyp with no dysplasia.
Classically the polyps in Peutz-Jegher syndrome are hamartomatous, but adenomas and adenocarcinomas
are also reported. Hizawa et al2 described his experience with 75 GI polyps from 7 patients with PJS where 71 had hamartomatous polyps, 2 adenoma, 1 cancer in adenoma, and 1 pyogenic granuloma.
Small bowel polyps are treated with laparotomy with enteroscopy and polypectomy. Our patient had developed colonic and ovarian malignancies along with small bowel polyps; over just a two-year follow
up, all of these were succesfully treated. A hamartoma-adenoma-carcinoma sequence as been described
in PJS.2
References
1. Burdick D, Prior JT. Peutz-Jeghers syndrome. A clinicopathologic
study of a large family with a 27-year followup.
Cancer 1982;50:2139-46.
2. Hizawa K, Iida M, Matsumoto T, Kohrogi N, Yao T, Fujishima M. Neoplastic transformation arising in Peutz-Jeghers polyposis. Dis Colon Rectum 1993;36:953-7.



1: Asian J Surg. 2006 Jul;29(3):180-4.

Links

Urethral advancement and glanuloplasty with V flap of the glans in the repair of anterior hypospadias.

Roodsari SS, Mulaeian M, Hiradfar M.

Department of Pediatric Surgery, Mashhad University of Medical Sciences, Mashhad, Iran. sheikh-hos[at]mums.ac.ir

BACKGROUND: Hypospadias is a common urethral anomaly in boys. More than 65% of hypospadias cases are anterior (glanular, coronal and distal penile shaft). More than 200 original techniques have been applied to correct hypospadias. Each of these techniques has some complications, the most common of which are fistula and meatal stenosis. METHODS: A total of 74 boys with anterior hypospadias underwent the procedure of urethral advancement and glanuloplasty (UAGP) with V flap of the glans in our medical centres between March 1994 and March 2000. The procedure included degloving, correction of chordee, urethral mobilization and glans plasty. RESULTS: Cosmetic results were excellent in most patients. There was no fistula, and meatal stenosis was also not observed after applying V flap of the glans. In a 1-6-year follow-up (mean +/- SD, 3.15 +/- 1.79 years), the results, functionally and cosmetically, were satisfactory in all cases, with no long-term complication or chordee. CONCLUSION: Our findings suggest that UAGP is an excellent technique for repairing anterior hypospadias with satisfactory results and low complication rate.

PMID: 16877221 [PubMed - indexed for MEDLINE]

 



1: Asian J Surg. 2006 Jul;29(3):180-4.

Urethral advancement and glanuloplasty with V flap of the glans in the repair of anterior hypospadias.

Roodsari SS, Mulaeian M, Hiradfar M.

Department of Pediatric Surgery, Mashhad University of Medical Sciences, Mashhad, Iran. sheikh-hos[at]mums.ac.ir

BACKGROUND: Hypospadias is a common urethral anomaly in boys. More than 65% of hypospadias cases are anterior (glanular, coronal and distal penile shaft). More than 200 original techniques have been applied to correct hypospadias. Each of these techniques has some complications, the most common of which are fistula and meatal stenosis. METHODS: A total of 74 boys with anterior hypospadias underwent the procedure of urethral advancement and glanuloplasty (UAGP) with V flap of the glans in our medical centres between March 1994 and March 2000. The procedure included degloving, correction of chordee, urethral mobilization and glans plasty. RESULTS: Cosmetic results were excellent in most patients. There was no fistula, and meatal stenosis was also not observed after applying V flap of the glans. In a 1-6-year follow-up (mean +/- SD, 3.15 +/- 1.79 years), the results, functionally and cosmetically, were satisfactory in all cases, with no long-term complication or chordee. CONCLUSION: Our findings suggest that UAGP is an excellent technique for repairing anterior hypospadias with satisfactory results and low complication rate.

1 : THE JOURNAL OF QAZVIN UNIVERSITY OF MEDICAL SCIENCES AND HEALTH SERVICES Spring 2006; 10():133-136.

CONGENITAL POUCH COLON WITH IMPERFORATE ANUS; A REPORT OF 3 CASES

SADEGHIPOUR ROODSARY S.*,HIRADFAR M.,ZABOLINEJAT N.,ALAMDARAN S.A.

Congenital pouch colon (CPC) is an unusual condition in imperforate anus (IA), usually of high variety. This malformation is mostly reported from India, where it comprises about 7% of all anorectal malformations. In this anomaly, the normal colon is very short or absent and is replaced by a pouch like dilatation that usually communicates through a fistula with genitourinary tract or persistent cloaca. We are reporting on three cases of newborns (2 females, and 1 male) with this anomaly diagnosed during September 2001 to September 2004. These three cases comprise less than 3% of all cases of IA during this time period. The two girls had anorectal agenesia, short segment of colon and a pouch with vaginal fistula and persistent cloaca (Type II). Colon in the third case was replaced by a dilated pouch that communicated with a wide fistula to the bladder (Type I). This is very important for pediatric surgeons to be aware of the features of this condition to allow for proper diagnosis and surgical management.

Keyword: Pouch Colon, Imperforate Anus, Anorectal Malformation

 

Abstracts
International Conference on Childhood Cancer (ICCC-2006)
29-31 Oct. 2006
Tehran, Iran

Welcome Message
Childhood malignancies have emerged worldwide as a major health problem and rank after accidents as the second cause of mortality in developed countries. The less developed countries are facing similar problems in pace with improving social health services, increasing living standards and public awareness about childhood cancer ,its curability and expecte availability of newest diagnostic and treatment approaches. Since late
1960 we have been witnessing an enormous improvement in terms of
better understanding the natural history of childhood malignancies getting deeper insight into their genetic and molecular biology, their
epidemiology and impact of environmental factors on the diverse
features of the childhood cancers
The aim of the International Pediatric Oncology Conference in Iran is to build an international forum for researchers in all fields of pediatric oncology and pediatric oncologist to share and exchange their knowledge and experiences in daily practice. Of further importance of this conference will be to direct the attention of the health authorities toward childhood malignancies to make proper planning for training programs, building child friendly oncology departments allocating sufficient facilities and funds to give our patients the utmost benefits of new therapeutic modalities and control strategies. This conference will also address the needs of young physicians and nurses about the state- of -the art in pediatric oncology. In addition to the exciting scientific programs of the conference, a very rich Iranian cultural heritage is another unforgettable experience that the participants will enjoy.