The onset of hypertrophic pyloric stenosis in the postoperative course of esophageal atresia with tracheoesophageal fistula is rarely reported. The diagnosis could be delayed due to its mimicking symptoms of other postoperative complications including gastroesophageal reflux or anastomotic stricture. We present an infant who had surgery for esophageal atresia with tracheoesophageal fistula. He had never fed since birth. The infant presented with an increased amount of orogastric tube drainage and consistently distended gastric air on simple abdominal X-ray. Abdominal ultrasonography showed hypertrophic thick pyloric muscle. The diagnosis of pyloric stenosis was confirmed d is rarely reported. The diagnosis could be delayed due to its mimicking symptoms of other postoperative complications including gastroesophageal reflux or anastomotic stricture. We present an infant who had surgery for esophageal atresia with tracheoesophageal fistula. He had never fed. The infant presented with uring surgery. After pyloromyotomy, the patient's condition improved.
This study was aimed to evaluate associated congenital anomalies in the patients with esophageal atresia with tracheoesophageal fistula (EA/TEF). Forty-two neonates with the diagnosis of EA/TEF treated over a 10 year period in a single institution were included in this study. The demography of EA/TEF was analyzed. Major associated anomalies including vertebral, anal, cardiac, renal, limb, neurologic and chromosome were reviewed and categorized. Males were slightly more dominant than females (1.47:1) and all patients had Gross type C EA/TEF. Only 19% of the patients had solitary EA/TEF without associated anomalies. Cardiac anomalies were the most common associated congenital anomaly in patients with EA/TEF (73.8%). But 47.6% were cured spontaneously or did not affect patients' life. Atrial septal defect (ASD) was the most common cardiac anomaly followed by patent ductus arteriosus (PDA) and ventricular septal defect (VSD). Among gastrointestinal anomalies (23.8%), anorectal malformations were the most frequent, 70% Vertebral and limb abnormalities accounted for 11.9% and urogenital malformations 9.5% of the anomalies in patients with EA/TEF. VACTERL associated anomalies were 23.8% and 4.8% had full VACTERL. Almost 12% of EA/TEF had neurologic anomalies. Patients with EA/TEF require preoperative evaluation including neurologic evaluation to detect anomalies not related to VACTERL. Though associated cardiac anomaly occurred in 73.8% of patients in our study, only 21.42% needed surgical correction. The authors suggesrs further studies with large numbers of patients with EA/TEF.
Tracheal injury is a rare complication of endo-tracheal intubation. However in neonates, the rates of morbidity and mortality are high. Recommendations for treatment are based on the several reports of this injury and are individualized. Conservative management can be effective in some cases. We describe the case of a neonate who presented with subcutaneous emphysema after intubation in a neonatal intensive care unit. This patient suffered full VACTERL syndrome and had 1.7mm diameter subglottic stenosis. Conservative management resulted in no further increase in subcutaneous emphysema and after 10 days the patient was stable.
Labial adhesion in prepubertal girls is a common gynecologic problems. The labia minora are fused by thin transparent or thick fibrous membrane in the midline from the clitoris to posterior fourchet. The prevalence of labial adhesion may be even greater because many children with labial fusions are asymptomatic and these cases remain unreported. They are often unrecognized by physician and parents because most of symptomatic children complained urinary symptoms. The authors experienced 2 cases of labial adhesion in girls; one asymptomatic partial fusion and the other symptomatic complete fusion. These lesions were treated successfully by mechanical separation of labial adhesion and petroleum ointment (Vaseline) application without recurrence in follow-ups.
Extraosseous Ewing's sarcoma is a rare primary malignant soft tissue tumor which is histologically identical to Ewing's sarcoma. This tumor tends to involve the soft tissue of the lower extremity and paravertebral region of adolescents and young adults but particularly rare in infants. We recently experienced a case of extraosseous Ewing's sarcoma which presented in the left arm of 4 months infant.
Abdominal cystic lymphangiomas arising from the mesentery, omentum and retroperitoneum are rare and occasionally confused with other cause of acute abdomen. Sixteen children who underwent surgery for abdominal cystic lymphangioma between 1984 and 2005 at the Division of Pediatric Surgery, Keimyung University Dongsan Medical Center were evaluated retrospectively. There were 9 boys and 7 girls. Age ranged from 12 days to 13 years (mean age : 4.7years). The cysts were located in the omentum (4 cases),mesentery (9 cases : jejunum : 3, colon : 6). retroperitoneum (2 cases) and pelvic cavity (one case). The cyst content was hemorrhagic (8 cases), serous (5 cases), and inflammatory (2 cases), and chyle (one). Prenatal diagnosis was made in 3. The clinical features were variable, but abdominal pain, mass, and abdominal distention in order. There were 3 emergency operations in patients with complicated cyst, who were suspected of having panperitonitis and volvulus preoperatively. Complete excision was accomplished in all cases. There was one mortalityin a newborn with E.coli sepsis. Intestinal obstruction developed in 2 cases in long-term follow-up. No recurrence was observed. Although intraabdominal lymphangioma arising from mesentery, omentum and retroperitoneum are rare, it should be considered as a possible cause of acute abdomen.
Inguinal hernia is the most common disease treated by the pediatric surgeon. There are several controversial aspects of management 1)the optimal timing of surgical repair, especially for preterm babies, 2)contralateral groin exploration during repair of a clinically unilateral hernia, 3)use of laparoscope in contralateral groin exploration, 4)timing of surgical repair of cord hydrocele, 5)perioperative pain control, 6)perioperative management of anemia. In this survey, we attempted to determine the approach of members of KAPS to these aspects of hernia treatment. A questionnaire by e-mail or FAX was sent to all members. The content of the questionnaire were adapted from the “
Chronic duodenal obstruction related to a congenital web is a rare anomaly that is sometimes difficult to diagnose preoperatively. A case of partial duodenal obstruction along with a foreign body by a congenital duodenal web in a 10-year-old girl is presented. She has had one year history of intermittent epigastric discomfort without nausea, vomiting and growth retardation. The studies including UGI series and gastroduodenoscopy disclosed a perforated web in the 2nd portion of the duodenum and a dark go stone just proximal to the web. Via a longitudinal duodenotomy across the web, the web was partly excised with preservation of ampulla of Vater and the duodenum was closed in transverse fashion. The aperture of ampulla of Vater was located at 7 o'clock direction of posterior surface of the duodenal web. This particular case reminds clinicians to consider a duodenal web in a youngster presenting with a longstanding foreign body in the duodenum as one of the underlying pathologies.
Anal canal duplications occurring in a pair of 4 month-old healthy female twins are presented. The openings were located in the posterior midline of the anus since birth without a history of perianal abscess or swelling. Excision of the duplicated anal canals was performed using posterior sagittal approach. Although the anal canal duplication occurs predominantly in female, to our knowledge, this is the first case of anal duplication in a monozygotic female twins reported.
The authors, over the last 6 months, have treated 2 patients with perforated typhlitis complicating acute lymphocytic leukemia (ALL) with good outcome. The first patient was a 13-year-old male who developed intermittent high fever, abdominal pain, abdominal distention and diarrhea during the course of maintenance chemotherapy. The peripheral leukocyte ranged from 230-470/mm3. Serial ultra sonograms and CT scans demonstrated irregular thickening of the cecal and ascending colonic walls and subsequent ragged perforation of the posterior wall of the cecum. He survived after treatment by right hemicolectomy and aggressive supportive measures. The patient case was a 3 year-old female who developed intermittent high fever , right lower abdominal pain, a mass, and watery diarrhea during the course of maintenance chemotherapy. Serial ultra sonograms and CT scans demonstrated irregular thickening of the cecal wall (6-15mm in thickness) and subsequent small perforation of the posterior wall of the cecum with thick-walled localized abscess. She has recovered completely after aggressive medical management. We learned two lessons from our experience treating these patients: 1) early diagnosis provided by a high index of suspicion and the use of ultra sonogram or CT scan is essential. And 2) although perforation is one of the surgical indications for the treatment of typhlitis, it is possible to manage the perforation nonoperatively in selected cases with localized abscess.
A case of primary hyperparathyroidism due to parathyroid adenoma is presented. A 14-y-o male came in with voiding difficulty. The intravenous pyelogram (IVP) demonstrated a stone at the proximal one third of the left ureter and marked hydronephrosis of the left kidney, and the Tc-99m sestamibi nuclear scan demonstrated an hot spot below the lower pole of the left lobe of the thyroid. Laboratory study demonstrated hypercalcemia (12.4mg/dL) and elevated parathyroid hormone (143.67pg/mL). The excised parathyroid gland which was located below the lower pole of the left lobe of the thyroid, turned out to be parathyroid adenoma, consisting of mainly chief cells. Postoperatively he complained of transient hypocalcemic symptoms, which resolved by administration of calcium preparation and vitamin D.
This paper includes our 9-year experience of 34 infants with biliary atresia with introduction of a new non-invasive diagnostic method, that is, ultrasonographic "triangular cord" (TC) sign. TC sign was defined as visualization of a triangular or a band-like echogenicity just cranial to the portal vein. Ultrasonographic TC sign seemed to be a simple, non-invasive, time-saving and useful tool in the diagnosis of biliary atresia, representing 84% sensitivity. Active bile excretion was restored in 90% of the patients who were treated between 31-60days, 78% of those between 61-90 days, and 33% of those being 91days or older. The incidence of postoperative cholangitis was 36%, and construction of antireflux valve in the Roux-en -Y loop did not affect the incidence of postoperative cholangitis (P=0.18). As for the surgical outcome, of 34 infants with biliary atresia, 23 (68%) are alive for 2-102 months period, and 12 of them are alive for more than 5 years . Five-year estimate survival by Kaplan-Meier method was 66 %.
A 6(1/2)-year-old girl developed recurrent cholangitis following hepatic portoenterostomy for biliary atresia. Computed tomogram showed an ovoid cyst (4.5 × 4.0 cm in size) in the left hepatic lobe and another tubular dilatation (2.0 × 0.8 cm in size) in the right hepatic lobe. Percutaneous transhepatic cholangio-drainage (PTCD) with cystogram showed an ovoid cyst in the left hepatic lobe (Tsuchida type A), measuring 6.6 × 5.0 cmin size. She became afebrile and anicteric with aid of PTCD and parenteral antibiotics. However she continued to drain 45-150 cc of bile per day via the tube for over 2 weeks. Then she successfully underwent intrahepatic cystojejunostomy with guidance of intraoperative ultrasonography. This case illustrates relapsing cholangitis caused by Tsuchida type A intrahepatic cyst, which was successfully managed with PTCD followed by internal drainage procedure.
To differentiate biliary atresia from intraheaptic cholestasis, Tc-99m DISIDA hepatobiliary scintigraphies and percutaneous needle biopsies of 60 consecutive infants were evaluated retrospectively. Twenty three patients had biliary atresia and 37 patients intraheaptic cholestasis(neonatal hepatitis 34, TPN induced jaundice 2 and Dubin-Johnson syndrome i). All sixty patients underwent Tc-99m DISIDA hepatobiliary scintigraphy with phenobarbital pretreatment. Of 23 patients with biliary atresia, 22 were correctly interpreted showing 96% sensitivity while of 37 patients with intraheaptic cholestasis, only 12 had intestinal excretion of radionudide showing 32% specificity. Among the forty needle biopsies, 17 of biliary atresia and 23 of intrahepatic cholestasis, 37 were correctly interpreted as either having biliary atresia or intrahepatic cholestasis. The overall diagnostic accuracy was 93%. Of 3 misdiagnosed cases, the histologic findings of two patients with biliary atresia(aged 43 days and 54 days at the first needle biopsy) were essentially the same as those of neonatal hepatitis. Follow-up biopsies, however, showed findings consistent with biliary atresia. The third one(VLBW premie with history of 8 weeks of TPN) showed mild ductal proliferation and portal fibrosis. This was interpreted as suspicious for biliary atresia. Jaundice resolved gradually. In summary, patients who have intestinal excretion of radionudide on Tc-99m DISIDA hepatobiliary scintigraphy, biliary atresia can be ruled out. But the patients who do not have intestinal excretion of radionudide should have further investigation by needle biopsy. Judicious use of Tc-99m DISIDA hepatobiliary scintigraphy and percutaneous needle biopsy can give a diagnostic accuracy of 95% or more in cases of infantile cholestasis.
Segmental dilatation of the colon is a very rare disease entity of unknown etiology and may mimic Hirschsprung's disease. It is characterized by dilatation of a segment of the colon of variable length with obstruction due to lack of peristalsis in a normally innervated intestine. Recently authors experienced a case of segmental dilatation of the sigmoid colon in a 6 month-old male, who presented with severe constipation, abdominal distention, and abdominal mass since 2 months of age. Down's syndrome and congenital nystagmus were associated. Barium enema demonstrated focal dilatation of the sigmoid colon, but the rectum and descending colon proximal to the affected colon were of normal caliber. Rectal suction biopsy with acetylcholinesterase staining was normal and anorectal manometry showed normal rectosphincteric reflex. At operation, there was a massively dilated and hypertrophied sigmoid colon with increased tortuous serosal vessels, measuring 15 cm in length and 10 cm in width. Teniae coli were identifiable in the affected segment. Frozen section biopsies at the proximal, affected, and distal colon showed ganglion cells. Descending loop colostomy was constructed initially and segmental resection and end to end colocolostomy were carried out 3 months later. Final histologic examination showed 1) normal colonic mucosa with ganglion cells, 2) prominent submucosal fibrosis and marked muscular hypertrophy, 3) unremarkable acetylcholinesterase activity and immunohistochemical findings against S-100 protein. On 8 months follow-up, he has been doing well and moves bowels 1-2 times daily.
The acetylcholinesterase(ACHE) activity in 37 biopsies from the patients with Hirschsprung's disease was analyzed for histochemical patterns according to age, and its evolutional behavior was also assessed. The histochemical criterion used for the diagnosis of Hirschsprung's disease was that of Chow, i.e., “the presence of many coarse discrete cholinergic fibers in the muscularis mucosae and in the immediately subjacent submucosa regardless of an infiltration of cholinergic fibers in the lamina propria.” The acetylcholinesterase activity in Hirschsprung's disease was further classified into 3 patterns, advocated by de Brito and Maksoud: Pattern I-many thick fibers exclusively in the muscularis mucosae and submucosa(newborn pattern). Pattern II-many thin fibers in the muscularis mucosae and submucosa with a clear infiltraiton of cholinergic fibers in the lamina propria(classical pattern). Pattern III-an intermediate pattern showing morphological characteristics of the two patterns with predominance of one or the other. Of 37 biopsies, Pattern 1 was seen excusively in 16 biopsies taken from the neonates. Pattern III was 11 of 37 biopsies and it was mainly seen in children between 2 to 12 months of age. Pattern II was seen in all 3 biopsies of older than 1 year of age and in 6 ones of 3 to 12 months of age. In summary, this study reinforces the impression that there is an evolutional character of the distribution and morphology of the cholinergic ACHE positive fiber with age. In other words, ACHE activity in Hirschsprung's disease appears to evolve with age from the newborn pattern to the intermediate pc.ttern finally to the classical pattern.
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