Eui Ho Hwang

An unusual case of monozygotic epigastric heteropagus twinning

Epigastric heteropagus refers to unequal and asymmetric conjoined twins in which the dependent component (parasite) is smaller and usually attached to the epigastrium of the dominant component (autosite). Epigastric heteropagus is a very rare type of conjoined twins. It is generally assumed that conjoined twins represent a form of monozygotic twinning, but there has been no confirmation of this monozygotic hypothesis. Epigastric heteropagus twins differ in several ways from symmetrical conjoined twins. These include male preponderance, and no major connection of vessels, bowels, or bones. These significant characteristics suggest to the authors that the fusion of two fertilized ova and the atrophy of one fetus resulted in this interesting malformation. However, a DNA study indicated monozygotic origin in their case. The possible pathogenesis was ischemic atrophy of the body structure of the monozygotic conjoined twins at an early gestational age.

Clinical experience with conservative surgery for vaginal endodermal sinus tumor

Two cases of vaginal endodermal sinus tumor (EST), a rare pediatric malignancy, were managed with conservative surgery followed by adjuvant chemotherapy. The first case involved a 9-month-old girl with EST of the vagina, who was treated with a partial vaginectomy and VAC regimen (vincristine, actinomycin D, cyclophosphamide) during a 2-year period. The serum alpha-fetoprotein (AFP) level returned to normal after excision of the tumor, and it remained normal throughout the treatment period. There was no evidence of disease 30 months after diagnosis. The second case involved an 8-month-old girl with EST of the vagina, who was treated with local excision of the mass through a vaginotomy. The VAC regimen was administered, but the serum AFP level remained elevated. A follow-up abdominopelvic computed tomography scan, taken 4 months after the operation, showed local recurrence of the tumor. The VAC regimen was then changed to a BEP regimen (bleomycin, etoposide, cisplatin). The serum AFP level returned to normal after 2 courses of the new regimen, and no tumor was visible on the follow-up magnetic resonance imaging study. For vaginal EST, primary conservative surgery and adjuvant chemotherapy are attractive measures to preserve both reproductive and sexual function. The extent of conservative surgery requires at least a partial vaginectomy. Simple tumor excision may not be adequate to achieve cure or to prevent local recurrence, even with adjuvant chemotherapy. The serum AFP level is useful for diagnosing and monitoring vaginal EST in the infant.

Acquired choledochal cyst from anomalous pancreatobiliary duct union

The high incidence of anomalous pancreatobiliary duct union (APBDU) in children who have a choledochal cyst has been well documented. Additionally, cylindrical dilatation of the bile duct has been reproduced in animal models by anastomosing the pancreatic duct to the bile duct. In recent years, APBDU has been considered a possible etiologic factor in the formation of a choledochal cyst. The authors observed a progressive cylindrical dilatation of the common bile duct in a 6-year-old boy over an 18-month period. An operative cholangiogram showed a type Ic choledochal cyst and a type B APBDU (Todani classification). This clinical experience suggests that a normal common bile duct in children can be progressively dilated and become an acquired choledochal cyst arising as a complication of the preexisting APBDU.

Multiple intestinal ulcerations and perforations secondary to methicillin-resistant Staphylococcus aureus enteritis in infants

PURPOSE The aim of this study was to define a distinctive clinical entity of multiple intestinal ulcerations and perforations in infants. METHODS Two infants underwent abdominal exploration for surgical abdomen and were noted to have multiple intestinal ulcerations and perforations. A peculiar and unique surgical finding, numerous transverse linear ulcerations scattered along the entire small intestine, prompted us to search for similar instances. Five similar cases were additionally identified by members of the Korean Association of Pediatric Surgeons. The clinical courses, the surgical findings, and the results of bacterial cultures were reviewed. As well, the tissues of resected intestines were examined histopathologically. RESULTS The characteristics of this entity are as follows. (1) It usually occurs in infants who have been treated with broad-spectrum antibiotics. (2) Despite broad-spectrum antibiotic treatment, diarrhea and abdominal distension developed progressively and deteriorated. (3) Histological evaluation showed mucosal ulcers with neutrophil infiltration, submucosal microabscesses, and colonies of Gram-positive cocci. (4) Methicillin-resistant Staphylococcus aureus (MRSA) was the predominant organism cultured from the body fluid. (5) Only two cases, the completely resected one and the one immediately treated postoperatively with vancomycin, survived. CONCLUSIONS This entity is caused by multiple intestinal ulcerations and perforations secondary to MRSA enteritis in infants. It has a high mortality rate because of its difficult diagnosis. However, early recognition of this entity can lead to successful treatment.

Colon perforation in hyperimmunoglobulin E syndrome

Colon perforation from hyperimmunoglobulin E syndrome is very rare, and only one case has been reported in the English-language literature. Herein, the authors report another case of colon perforation experienced in hyperimmunoglobulin E syndrome. The patient was an 8-year-old girl with frequent infection, eczematoid dermatitis, and an increased serum level of immunoglobulin E. During admission, panperitonitis developed caused by colon perforation. Treatment was resection of the perforated segment of the colon and a double-barrel colostomy. The patient has been doing well 18 months after treatment.

Laparoscopic Total Intracorporeal Correction of Choledochal Cyst in Pediatric Population

BACKGROUND The laparoscopic correction of the choledochal cyst is an attractive treatment option. However, even the skilled surgeons do not prefer the intracorporeal jejunojejunal anastomosis due to the technical difficulties. In this article, we present the feasibility of laparoscopic total intracorporeal correction of the choledochal cyst, including the retrocolic Roux-en-Y hepaticojejunostomy and jejunojejunostomy. METHODS A prospective review of 6 cases of consecutive laparoscopic surgery for choledochal cyst in the pediatric population from March 2007 to April 2008 was performed. All patients underwent laparoscopic excision of a choledochal cyst and total intracorporeal Roux-en-Y reconstructions. The intracoporeal jejunojejunostomy was made by introducing an endoscopic gastrointestinal anastomosis device (Endo-GIA; US Surgical, Norwalk, CT) through the umbilical port with hand-sewn reinforcement. We evaluated the patients age at the time of operation, time taken for total operation, time taken for jejunojejunostomy, and intra- and postoperative events. RESULTS All 6 cases were girls (age ranging from 4 months to 7 years). All had type I choledochal cyst. Five ports were utilized: one telescopic port at the umbilicus, one left subcostal port for liver retraction, two operating ports on the right flank and left side of the umbilicus, and one right-lower quadrant port for the assistant. The mean time for total operation was 275 + or - 58 minutes (range, 210-360). Total intracoroporeal jejunojejunostomy took 38 + or - 10 minutes (range, 25-55). All patients were symptom free during the median follow up of 3.5 months. CONCLUSION In the pediatric population with choledochal cyst, total intracorporeal Roux-en-Y hepaticojejunostomy and jejunojejunostomy during laparoscopic surgery is feasible without the need for exteriorization of the bowel.

How important is the role of the internal anal sphincter in fecal continence? An experimental study in dogs

It is a generalized concept that the internal anal sphincter (IAS) plays a significant role in fecal continence by generating high pressure in the anal canal at rest and relaxation during rectal distention. Agreement also exists on the importance of internal sphincter-saving anoplasty on anorectal malformations in establishing anal continence. Twelve dogs were divided into four groups. Group 1, a control group, was subjected to a perirectal dissection only. Group 2 underwent the same perirectal dissection plus a 2-cm resection of the anal canal. Group 3 underwent the perirectal dissection plus a 4-cm resection of the anal canal, and group 4 underwent perirectal dissection and transposition of the anus to the posterolateral portion of the voluntary muscle mass. Clinical continence was evaluated, and manometric results were compared with preoperative measurements. All dogs in groups 1, 2, and 3 were clinically continent without soiling except one in group 3, and also manometric results showed minimal change between preoperative and postoperative anal pressure profiles. The transposed anus of group 4 showed continuous fecal soiling. The anal resting pressure (ARP) was also decreased but still existed in this group. This experimental study showed that the IAS contributes to the anal resting tone. However, resection of the IAS did not completely interfere with fecal continence. The smooth muscle of pulled-through rectum seemed to partly take over the function of the IAS.