• Advances in pediatric surgery
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• v.20 no.2
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• pp.38-42
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• 2014

Purpose: Gastroschisis and omphalocele are major anterior abdominal wall defects. The purpose of this study was to analyze the clinical differences and mortalities of gastroschisis and omphalocele in Asan Medical Center. Methods: A retrospective review of the medical records was conducted of 103 cases of gastroschisis and omphalocele from September 1989 to February 2013 in Asan Medical Center in Korea. Results: There were 43 cases (41.7%) of gastroschisis and 60 cases (58.3%) of omphalocele. There was a female predominance in both gastroschisis (60.5%) and omphalocele (58.3%). The average gestational age at delivery was $36.7{\pm}0.4$ weeks for both groups. The mean birth weights were $2,381.9{\pm}80.6g$ for gastroschisis and $2,779.4{\pm}82.8g$ for omphalocele (p=0.001). Mean maternal ages in the gastroschisis and omphalocele groups were $27.5{\pm}0.7$ years and $30.5{\pm}0.7$ years, respectively (p=0.002). Associated malformations were documented in 13 infants (30.2%) with gastroschisis and 46 infants (76.7%) with omphalocele (p<0.001). All of gastroschisis patients except one underwent surgery including 31 primary repairs and 11 staged repairs. Fifty-two infants with omphalocele underwent surgery-primary repair in 41 infants and staged repair in 11 infants. Among 103 cases, 19 cases (18.4%) expired. Mortality rates of gastroschisis and omphalocele were 23.3% (10/43 cases) and 15.0% (9/60 cases), respectively (p=0.287). The main causes of death were abdominal compartment syndrome (6/10 cases) in gastroschisis, respiratory failure (4/9 cases) and discharge against medical advice (4/9 cases) in omphalocele. Conclusion: Gastroschisisis was associated with younger maternal age and lower birth weight than omphalocele. Associated malformations were more common in omphalocele. The mortality rates did not make a statistical significance. This might be the improvement of treatment of cardiac anomalies, because no patient died from cardiac dysfunction in our study. Furthermore, abdominal compartment syndrome might be the main cause of death in gastroschisis.

• Clinical and Experimental Reproductive Medicine
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• v.49 no.3
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• pp.215-218
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• 2022

Herein, we report an exceptionally rare case of a 25-year-old woman with cloacal exstrophy/omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) syndrome achieving a viable pregnancy despite many gastrointestinal and genitourinary malformations and multiple respective corrective operations. The patient was born with two vaginas, two uteruses, four ovaries, an imperforate anus, a large omphalocele including bowel and bladder exstrophy, and diaphysis of the pubic rami. This patient is the only documented OEIS patient not to have tethered spinal cord as an anomaly, perhaps contributing to her successful pregnancy. After experiencing preeclampsia with severe features at 35 weeks, the baby was born via cesarean section.

• Advances in pediatric surgery
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• v.4 no.1
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• pp.16-26
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• 1998

Parenteral nutrition has been an essential part of postoperative care of neonates requiring major surgery who are unable to tolerate enteral feeding for long periods during the postoperative period. However, TPN via central venous catheters(central TPN), used in increasing trend, still presents significant morbidity. To find out whether TPN via peripheral veins(peripheral TPN) could be used as a viable alternative for postoperative parenteral nutrition in neonates, a clinical study was carried out by a retrospective analysis of 53 neonates subjected to peripheral TPN for more than 7 days after surgery. Operations consisted of procedures for esophageal atresia with tracheoesophageal fistula, gastroschisis and omphalocele. Surgery was performed at the Division of Pediatric Surgery, Department of Surgery, Hanyang University Hospitall, from 1983 to 1994. The mean total duration of TPN was 13.3 days (range; 7-58 days), the average daily total fluid intake was 117.6 ml/kg during TPN and 158.6 ml/kg during subsequent oral feeding. The average daily total calorie intake was 57.7 kcal/kg during full strength TPN and 101.3 kcal/kg during subsequent oral feeding. The mean urine output was maintained at 3.5 ml/kg/ hour during TPN and at 3.6 ml/kg/hour during subsequent oral feeding. The increment of body weight observed during TPN was 132 g in TEF, 53 g in gastroschisis and 3 g in omphalocele patients, while loss of body weight was not observed. The mortality rate was 5.7 %(3/53) and was related to the underlying congenital anomalies, not the TPN. The most common complication of peripheral TPN observed was laboratory findings suggestive of liver dysfunction in 23 cases(43.4 %) with no significant clinical symptom or signs in any case, transient pulmonary edema in one case, and generalized edema in one case. None of the major complications usually expected associated with central TPN were observed. The result of this study suggest that peripheral TPN can be used for adeguate postoperative nutritional support in neonates requiring 2 to 3 weeks of TPN.

• Journal of Veterinary Clinics
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• v.26 no.5
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• pp.467-471
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• 2009

This report describes Neural Tube Defects (NTDs) with Abdominal Wall Defects (AWDs) on the sibling of Yorkshire terriers. The NTDs and AWDs are rare serious congenital defects. The NTDs are neurulation abnormality that results from to failed transformation of the neurual tube by the incomplete closure of the embryonic neural plate. These dysraphic states range form mild to severe according to developmental malformation that include fusion defects of skull (crania bifida; CB) and fusion defects of vertebrae (spina bifida; SB). The AWDs are genetic defects that results from to failed formation of abdominal wall and cavity. These dysraphic states are omphalocele and gastroschisis. The 12-month dam was delivered by caesarian section and 4 littermate had obvious malformations. One male dead stillbirth fetus (L1) was revealed the extruded abdominal viscera, omphalocele. One female fetus (L2) was died within 1 hour after birth with defects of abdominal muscle upper umbilicus, gastroschisis. 3rd fetus (L3) was died within 36 hours after parturition and revealed a copious dermal and vertebral defects on the midline thorax, upper SB asperta. 4th fetus (L4) is still growing well now at 6 months but at the 2 week age, appears hairy nevus on the frontal cranium and dorsal thoracic portion. The radiograph of L1 and L2 are shown decrease bony density of calvarium and L3 was shown defect of spinose processes of the T9-T13. On our knowledge, this is first report of the SB and CB in Yorkshire terrier. And also sibling of NTDs with AWDs that has not previously been reported in the dog.

• Advances in pediatric surgery
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• v.13 no.1
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• pp.37-44
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• 2007

A vitelline duct (VD) anomaly is a relatively common congenital abnormality of the umbilical area. The anomalies include patent vitelline duct (PVD), cyst, fistula or sinus. The incidence is approximately 2% of the populations, but development of symptoms is rare. Recently, we experienced two cases; PVD accompanied by a small omphalocele and intestinal volvulus due to mesenteric band between Meckel's diverticulum and the mesentery. Thereafter,we evaluated the data of vitelline duct anomalies for 27 years. From 1980 to 2006, 18 cases of VD anomalies were reviewed based on the hospital records retrospectively. There were 15 boys and 3 girls and age ranged from 2 days to 15 years. Among the 18 cases, 15 cases were symptomatic and consisted of Meckel's diverticulum (10 cases), PVD (4 cases) and umbilical polyp (1 case). Three asymptomatic cases of Meckel's diverticulum were found incidentally were and were observed without resection. Ten cases of Meckel's diverticulum were presented with intestinal bleedings (4 cases), intestinal obstructions (5 cases) and perforation (1 case). Wedge resections and segmental resections of ileum were performed in 8 patients and 2 patients, respectively. Postoperative complications were adhesive ileus (1 case) and wound seroma (1 case). Small omphaloceles were accompanied in two of 4 PVD patients. There was 1 small omphalocele case which was accompanied by a prolapse of ileum. In summary, VD anomalies were more common in male and more than half of them were found in patients less than 1 year of age. PVD was diagnosed most frequently in neonates. Meckel's diverticulum presented with intestinal obstruction more frequently than bleeding.

• Journal of Dental Anesthesia and Pain Medicine
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• v.16 no.4
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• pp.313-316
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• 2016

Airway obstruction in pediatric patients always poses a challenge for anesthesiologists. Beckwith-Wiedemann syndrome causes various abnormalities such as macroglossia and omphalocele. Patients with these abnormalities often need corrective surgeries. Management of difficult airway caused by conditions such as macroglossia in patients with this syndrome could be challenging. We encountered a case of difficult airway in an infant with Beckwith-Wiedemann syndrome. It was predicted that macroglossia might cause difficult ventilation, intubation, and extubation. Preoperative assessment and preparations for difficult airway should be considered.

• Advances in pediatric surgery
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• v.6 no.2
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• pp.160-165
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• 2000

Beckwith-Wiedemann syndrome presents with multisystemic patterns of congenital anomalies and macrosomia. This syndrome was independently described by Beckwith in 1963 and by Wiedemann in 1964. There is wide spectrum of clinical manifestations, including prenatal or postnatal overgrowth, neonatal hypoglycemia, macroglossia, visceromegaly, omphalocele, hemihypertrophy and a predisposition for embryonal tumors, most frequently Wilms' tumor. We managed a case of Beckwith-Wiedemann syndrome with left adrenal cortical neoplasm of undetermined malignancy.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.27 no.6
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• pp.559-564
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• 2005

Beckwith-Wiedemann syndrome is an autosomal dominant growth excess disorder, which occurs with a reported incidence of 1 in 13,700 to 1 in 17,000 live births. It constitutes a discrete clinicopathologic entity characterized by macroglossia, abdominal wall defects (omphalocele), visceromegaly, gigantism, hemihypertrophy, hypoglycemia, and the increased risk of solid tumor development from multiple cell lines. A macroglossia is a key component of the syndrome, and can lead to cosmetic, functional and psychologic disorder. This report shows a 5-year-old patient with Beckwith-Wiedemann syndrome, who had macroglossia and received reduction glossectomy.

• Archives of Reconstructive Microsurgery
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• v.10 no.2
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• pp.163-168
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• 2001

The defects of the abdominal wall could be brought about either congenitally, for instances in such cases as omphalocele or gastroschisis, or by various acquired causes-trauma, excision of tumors, excision of burn scar, tissue necrosis caused by infection, hematoma after abdominal surgery, tissue necrosis after radiation therapy and so on. As for the techniques of the reconstruction of the abdominal wall defects, many authors have developed and reported diverse methods. To summarize, primary closure, skin graft, local skin flaps, various myocutaneous flaps, free flap, fascia graft, artificial mesh, tissue expansion, etc could be used in the reconstruction of the abdominal wall defects. The periumbilical perforator-based island skin flap has a many advantages such as no significant sacrifice of the rectus abdominis muscle, wide rotation arc, reliable blood flow of the perforator, short elevation time for flap, and for middle-aged, obese patients, the donor site may be the best from the cosmetic point of view. We used perforator-based island skin flap in 5 cases with reasonable result from March 1999 to May 2001. There were no significant complications and donor sites could be repaired primarily.

• Clinical and Experimental Pediatrics
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• v.52 no.10
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• pp.1171-1174
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• 2009

Edwards syndrome, also called trisomy 18, is one of the most common autosomal anomalies. The survival rate of patients with Edwards syndrome is very low and its characteristic findings include cardiac malformations, mental retardation, growth retardation, specific craniofacial anomalies, clenched hands, rocker-bottom feet, and omphalocele. Compared with the classic Edwards syndrome, the symptom of partial duplication of chromosome 18 is relatively mild with a good prognosis. We report the case of a baby with partial duplication 18q11.2-q12. The characteristic phenotype features of Edwards syndrome were observed in the patient. However, the symptom was milder than the typical Edwards syndrome. At present, we can expect better prognosis for this patient.