Congenital absence/hypoplasia/fusion of ribs are very rare anomalies and presentation varies from asymptomatic to life-threatening. Visibly evident cases are straightforward to diagnose but this is not always the case. Familiarity and awareness of these anomalies can help to diagnose cases with subtle signs and symptoms. Proper radiological investigations are vital for anatomical delineation. Absence or hypoplasia of the inferior ribs along with its attached muscles can cause ‘lung hernia’ that produces an unstable chest leading to paradoxical respiratory movements. Here, we present a case of hypoplastic and fused ribs in an infant, who presented with respiratory distress and created a diagnostic dilemma.

Hernia of the umbilical cord associated with segmental dilatation of the ileum (SDI) is a rare entity with only 17 cases reported in literature. We hypothesize that the prominent mesenteric vessels or folds that splay out towards the ends of the SDI signify the presence of an antecedent duplication cyst which eventually got resorbed to form the dilated segment.

This is a survey on congenital posterolateral diaphragmatic hernia, conducted by Korean Association of Pediatric Surgeons (KAPS). A registration form for each patient during the 5-year period between 2010 and 2014 and a questionnaire were sent to each member. Twenty-six members in 16 institutions returned completed forms. Total patients were 219. Prenatal diagnoses were done in 181 cases (82.6%). Preoperative mortality was 11.4%. Minimal invasive surgery was done in 61 cases (31.8%). Risk factors related with death were Apgar score, oxygenation index, preoperative pH and bicarbonate, O₂ saturation, the presence of hernia sac, and the size of defect. The neonatal survival and 1-year survival of total patients were 77.6% and 75.3%.

Although Meckel's diverticulum is the most common vitellointestinal duct (VID) anomaly, patent vitellointestinal duct (PVID) is the most common symptomatic embryological defect. Patient may present with the anomaly itself or due to complications like intestinal obstruction secondary to volvulus, intussusception or adhesions. Prolapse occurs if the diverticulum is wide-mouthed enough to allow bowel to come out or due to increased intra-abdominal pressure like cry or cough. Bowel prolapse through PVID is rare and double prolapse of proximal as well as distal loop in a newborn is extremely rare. Omphalocele with prolapsing bowel through PVID as found in our index case is even rarer in literature. The pediatric surgeon should be familiar with these varied manifestations in the newborn because the prolapsed bowel can progress to gangrene and complications if not identified and operated upon early.

Palpable inguinal mass in children should be differentiated from inguinal hernia, hydrocele, lymph node, and tumor. Though using ultrasonography, fatty tumor would be misdiagnosed as incarcerated inguinal hernia containing fatty component. We experienced the huge inguinal lipoblastoma in 5-year-old girl mimicking recurrent incarcerated hernia. Laparoscopic exploration revealed it was not incarcerated hernia but well demarcated bulging mass from abdominal wall. Mass was about 10×4×3 cm and extended from internal inguinal ring to saphenous opening. It was near total excised because of right external iliac vein injury. Pathologically, it was proven as lipoblastoma containing mature adipocyte with lipoblast and fibrous septa. Postoperatively, we noticed a segmental thrombotic occlusion of external iliac vein. After 1 year, she has no symptom related to occluded vessel. The remained lipoblastoma showed no interval change. Even lipoblastoma has a good prognosis with low recurrence rate, we need careful follow-up.

The present study compared the postoperative analgesic effects of ilioinguinal and iliohypogastric nerve block with infiltration of local anesthetics (bupivacaine) into the wound in children after inguinal hernia repair. Ninety children below 7 years old who were scheduled elective inguinal hernia repair were randomly allocated into one of three groups. The patients in nerve block (NB) group, ilioinguinal and iliohypogastric nerve block was done with 0.5 mL/kg of 0.25% bupivacaine. The patients in infiltration of local anesthetics (LI) group, 0.5 mL/kg of 0.25% bupivacaine was infiltrated into the wound after surgery. The patients in control group were allocated as a Control group. Postoperative pain was assessed at 1, 3, 5, and 24 hours after operation with FLACC scale and additional analgesic consumption were counted. The three groups were not significantly different in age, sex, body weight, and duration of operation. Pain scores at 1 hour and 3 hours after operation were significantly higher in Control group than in NB group and LI group (p<0.01), whereas there were no difference between NB group and LI group. The rescue analgesics administration was significantly higher in Control group (n=11) than in NB group (n=6) and LI group (n=7) (p<0.05). There were 2 cases of transient femoral nerve palsy in NB group. Both of ilioinguinal and iliohypogastric nerve block and infiltration of local anesthetics into the wound provided effective postoperative analgesia in early postoperative period following inguinal hernia repair in children. But no difference between the two methods. Technically, infiltration of local anesthetics into the wound was easier and safer than ilioinguinal and iliohypogastric nerve block.

Ketamine is a safe and effective drug for pediatric anesthesia, sedation and analgesia. We hoped to identify that surgeons could operate a pediatric hernia with the ketamine anesthesia without general anesthesia. The study was a consecutive case series of 2230 inguinal hernia patients aged 1 months to 17 years in a Joo's day-surgical clinic during 11-year period. The patients had pediatric inguinal hernia surgery without general anesthesia under the day-surgery system. We retrospectively analyzed the medical record of patients who were registered with the Diagnosis Related Group (DRG) system. All patients received ketamine (5mg/kg) and atropine (0.01mg/kg) intramuscularly before surgery. After anesthesia, we injected 1~2% lidocaine (Less than 5ml) subcutaneously at the site of incision and started operation. The surgical method was the high ligation method of the hernia sac.) In total 2230 patients, male were 1756 and female were 474. 2076 patients were a unilateral inguinal hernia at the time of surgery and 154 were bilateral hernia patients. Less than three months, depending on the age of the patients was 391, and less than 12 months the patient was 592 people (26.5%). After surgery, there were no accidents or long term complications associated with ketamine anesthesia. We think the surgeon can safely do the pediatric inguinal hernia surgery using ketamine and lidocaine without anesthesiologist through 11 years of our surgical experiences.

Experimental tracheal ligation (TL) has been shown to reverse the pulmonary hypoplasia associated with congenital diaphragmatic hernia (CDH) and to normalize gas exchange. The purpose of this study was to determine whether the TL would correct the surfactant deficiency present in the fetal rabbit model of CDH by using lamellar body count. Lamellar bodies are synthesized and secreted by the type II pneumocytes of fetal lung. The phospholipids present in these bodies constitute the major component of pulmonary surfactant. Twenty-one pregnant New Zealand rabbits underwent hysterotomy and fetal surgery on gestational day 24. Two fetuses of each pregnant rabbit were operated. In the fetus of one end of bicornuate uterus, left DH was created by excision of fetal diaphragm through open thoracotomy (DH Group). In the fetus of the other end of bicornuate uterus, left DH and TL were created (TL Group). The fetuses were delivered by Cesarean section on gestational day 31. Fourteen in control group, 12 in the DH group and 13 in TL group were born alive. En bloc excision of lungs, bronchi and trachea was done in all newborn rabbits. A five Fr catheter was inserted through trachea and repeated irrigations with 10 cc normal saline were done. The irrigated fluid was centrifuged at 280 xg for 5 minutes and the lamellar bodies were counted with the upper level fluid in platelet channel of electronic cell counter. The average lamellar body counts were 37.1 ± 14.2 × 10³/µL in control group, 11.5 ± 4.4 × 10³/#x00B5;L in DH group, and 6.5± 0.9 × 10³/#x00B5;L in TL group. Lamellar body count in DH group was lower than in control group and did not increase after TL. This study shows TL has no therapeutic effect on decreased surfactant level of CDH and the pregnant rabbit is appropriate for the animal model of CDH.

Hiatal hernia is a very rare disease in the pediatric population. However information from our esophageal atresia postoperative follow-up program has hypotheses; “Hiatal hernia may more frequently occur in postoperative esophageal atresia patients (EA group) than in the general pediatric population (GP group)” and “The tension on the esophagus after esophageal anastomosis may be an important etiologic factor of hiatal hernia in EA group”. To prove the first hypotheses, we compared the incidence of hiatal hernia in the GP group with the incidence in the EA group. The Incidence in the GP group was obtained from national statistic data from Statistics Korea and Health Insurance Review and Assessment Service of Korea. The incidence in the EA group was obtained from the medical record and the imaging studies of our esophageal atresia postoperative follow-up program. To prove the second hypothesis, the presumptive risk factors for the development of hiatal hernia in EA group, such as the type of esophageal atresia, degree of esophageal gap, the stage operation and the redo-operation with resection and re-anastomosis of esophagus were analyzed statistically. The total number of patients in the EA group was ninety-nine and there were 5 hiatus hernias. The incidence of EA group (5 %) is significantly higher than incidence of GP group (0.024 %). (p=0.0001) The statistical analysis of the presumptive risk factors for hiatal hernia development in EA group failed to show any evidence of correlation between postoperative esophageal tension and the hiatal hernia. This study shows that the postoperative patients with esophageal atresia have high occurrence of hiatal hernia and should be followed up carefully to detect hiatal hernia.

Extracorporeal membrane oxygenation (ECMO) has been utilized in congenital diaphragmatic hernia (CDH) patients with severe respiratory failure unresponsive to conventional medical treatment. We retrospectively reviewed 12 CDH patients who were treated using ECMO in our center between April 2008 and February 2011. The pre ECMO and on ECMO variables analyzed included gestational age, sex, birth weight, age at the time of ECMO cannulation, arterial blood gas analysis results, CDH location, timing of CDH repair operation, complications and survival. There were 9 boys and 3 girls. All patients were prenatally diagnosed. Mean gestational age was 38.8 ± 1.7 weeks and mean birth weight was 3031 ± 499 gram. Mean age at the time of ECMO cannulation was 29.9 ± 28.9 hours. There were 4 patients who survived. Survivors showed higher 5 min Apgar scores (8.25 ± 0.96 vs. 7.00 ± 1.20, p=0.109), higher pre ECMO mean pH (7.258 ± 0.830 vs. 7.159 ± 0.986, p=0.073) and lower pre ECMO PaCO2 (48.2 ± 7.9 vs. 64.8 ± 16.1, p=0.109) without statistical significance. The hernia was located on the left side in 10 patients and the right side in 2 patients. The time interval from ECMO placement to operative repair was about 3~4 days in 5 early cases and around 24 in the remaining cases. There were 3 cases of post operative bleeding requiring re operation and 2 cases of abdominal compartment syndrome requiring abdominal fascia reopening. ECMO catheter reposition was required in 4 cases. Three cases of arterial or venous thrombosis were detected and improved with follow up. Our data suggests that ECMO therapy could save the lives of some neonates with CDH who can not be maintained on other treatment modalities. Protocolized management and accumulation of case experience might be valuable in improving outcomes for neonates with CDH treated with ECMO.

Minimally invasive techniques for pediatric inguinal hernia repair have been evolving in recent years. We applied the laparoscopic method to repair pediatric inguinal hernia using the techniques of sac transection and intra-corporeal ligation. Between November 2008 and August 2010, 67 pediatric patients (47 boys and 20 girls) with inguinal hernias were included in this study. Postoperative activities, pain, and complication were checked prospectively at regular follow-up. One patient presented with clinically bilateral hernia, and three patients had metachronous hernias. Thirty-two cases out of 63 patients with unilateral hernias had a patent processus vaginalis on the contralateral side. Mean operation time was 35±11.4 minutes for unilateral hernias and 43±11 minutes for bilateral hernias. There were no intra-operative complications. One patient had a small hematoma on the groin postoperatively, which subsided spontaneously in a week. Recurrence and metachronous hernia were not found at follow up. In summary, laparoscopic inguinal repair in children is safe, easy to perform and has an additional advantage of contralateral exploration. Further studies should include long term follow-up.

Congenital diaphragmatic disease is one of the common major congenital anomalies, and its mortality remained still high despite recent medical advances. The aim of this study is to examine the clinical characteristics of congenital diaphragmatic diseases. A total of 39 patients with congenital diaphragmatic disease that underwent surgery from January, 1997 to December, 2009 at Pusan National University Hospital were included in this study. Medical records were retrospectively reviewed. The male to female ratio was 30:9. Six out of 39 cases died (NS) before surgery, 17 patients had Bochdalek's hernia (BH), 11 patients hiatus hernia (HH), 4 diaphragmatic eventration (DE), and 1 Morgagni hernia (MH). There were no differences in mean birth weight and mean gestational age. NS (83.3%). BH (35.3%) was diagnosed more frequently than other diseases in the prenatal period. Three patients (17.6%) of BH expired due to pulmonary hypoplasia and 1 patient had co-existing congenital heart disease. BH was diagnosed more frequently in the prenatal stage and had a higher motality rate than other conditions. Therefore, BH needs to be concentrated more than other anomalies.

Contralateral groin exploration (CGE) in children with unilateral inguinal hernia remains controversial. Between January 2002 and December 2007, 1967 pediatric patients with inguinal hernia were treated by two surgeons with different criteria of CGE (group A; boys younger than 2 years, older boys prematurely delivered, and all girls, B; birth weight lower than 2kg with inguinal hernia presentation within 6 months after birth, and suspicious physical findings) at Samsung medical center. Patient's age, sex, body weight, diagnosis, and metachronous contralateral inguinal hernia (MIH) incidence were analyzed retrospectively. Among 895 patients in group A, CGE was performed in 460 patients (66.4%) and MIH incidence was 1.7%. In group B, 31 patients (3.5%) had CGE among 1072 patients, and MIH incidence was 4.2%. The average hospital costs of group A and B were 763,956 won and 500,708 won, respectively. The CGE criteria of group B had advantage in total hospital cost. The primary site and the age at presentation had a signiticant effect on the incidence of MIH. But MIH incidence was low and the more contralateral explorations lead to increase of total costs. Therefore, routine contralateral groin exploration and surgery for a patent processus vaginalis could not be justified.

Peritoneal dialysis (PD) has been utilized for the children with end stage renal disease. Nevertheless, it is thought to promote inguinal hernia by increasing intraabdominal pressure. To investigate the clinical characteristics of inguinal hernia in children on PD, 155 cases of PD in children between January 1996 and June 2007 at Seoul National University Children's Hospital were reviewed retrospectively. Inguinal hernia developed in 16 cases (10.3%, M:F=8:8). Hernia occurrence was not correlated to age. Eleven cases (69%) of inguinal hernia developed in first 6 months after initiation of PD. All inguinal hernias were surgically repaired. No complications occurred related to inguinal hernia or surgery. Recurrent hernia developed in 1 patient (6.3%) of 2 cases who had PD postoperatively on the day of surgery.

In conclusion, inguinal hernia developed more frequently with children on PD than general population (3.5~5%). The rate of hernia development was highest within the first 6 months following initiation of PD. After repair of hernia, we recommend to discontinue PD immediate postoperatively to prevent recurrence.

This study is a retrospective analysis of 1244 cases of the inguinal hernia in children under the age of fifteen years who were operated at the department of pediatric surgery, Inje University Busan Paik Hospital from March, 1997 to February, 2007. The ratio of male to female was 3.6:1. The type of hernia was indirect in all of the cases. The hernia was on the right side in 656 cases (53.9 %), left side in 467 cases (37.5 %), and bilateral in 121 cases (9.7 %). The hernia presented most frequently in infants under age 12 months; 364 cases (29.2 %). Fifty-nine cases (21.7 %) were in female and 305 cases (31.3 %) in male. There were 428 cases (33.6 %) in 1-3 years age group, 295 cases (23.7 %) in 4-6 years, 112 cases (9.0 %) in 7-9 years, 39 cases (3.1 %) in 10-12 years and 16 (1.2 %) in 13-15 years. The content of hernia sac was small bowel (59 %), omentum (31 %) in males and the ovary and tube (54 %) and small bowel (26 %) in female. The incidence of combined operation at the time was 3.2 %, and consisting of orchiopexy (67.5 %), frenulotomy (12.5 %), appendectomy (10 %), circumcision (5 %), and fistulotomy (5%). The incidence of combined disease was 2.8 % and consisting of undescended testis, Hirschsprung's disease, idiopathic hypertrophic pyloric stenosis, imperforate anus, and congenital heart disease. After unilateral inguinal hernia repairs, contralateral hernias developed in 34 patients. The laterality of the primary site of hernias were left in 19 cases (55.8 %), and right 15 cases (44.1 %). The 936 cases (75.2 %) were operated under general anesthesia; Mask bagging 663 cases (53.2 %), endotracheal intubation 257 cases (20.6 %), and laryngeal mask 16 cases (1.2 %). The remainder 308 cases (24.7 %) were operated under regional caudal anesthesia.

The term “Littre's hernia” was originally defined by Reinke in 1841 as “the presence of a Meckel's diverticulum in any hernia sac.” Littre's hernia is a very rare disease, which accounts for less than 1% of all Meckel's diverticula. We report a case of Littre's hernia experienced in a 45 day-old infant.

Prophylactic contralateral exploration in unilateral inguinal hernia repair is still controversial. The purpose of this study is to analyze the contralateral incidence of hernia and to verify the necessity of the simultaneous contralateral exploration. Infants and children operated on for inguinal hernia or hydrocele at the Department of Pediatric Surgery of Asan Medical Center from January 1996 to December 2005 were analyzed retrospectively. A total of 383 patients (9.8 %) out of 3,925 patients underwent a simultaneous bilateral operation. A total of 222 patients (6.2 %) out of 3,542 patients underwent a secondary metachronous contralateral operation after primary unilateral inguinal hernia or hydrocele repair. Because simultaneous bilateral operation cases included true bilateral inguinal hernia or hydrocele, and unilateral hernia and simultaneous contralateral exploration, bilateral incidence of inguinal hernia and hydrocele could be maximally considered as 15.4% (605 patients). Therefore, the prophylactic contralateral exploration in unilateral inguinal hernia or hydrocele should be determined carefully in considering history and physical examination of the patients, and postoperative complications.

It is known that pediatric inguinal hernia is caused by the incomplete closure of processus vaginalis (PV). In the case of unilateral hernia, possibile contralateral patent PV should be considered because of its delayed appearance as well as its risk of incarceration. Direct visualization of patent PV could be done by contralateral exploration or by indirect exploration through the ipsilateral opening site of the affected hernia assisted with laparoscope. A patient group (321 persons) to whom laparoscopy was not performed from March 2000 to March 2003 was analyzed and compared with a patient group (280 persons) to whom laparoscopy was performed from April 2003 to September 2005. With all 601 patients, the sex ratio (male/female) of patients was 3.8:1. The side distribution was 57.7% in the right, 32.1% in the left and 10.1% in bilateral. There was no difference of sex and side distribution between before and after laparosopy adoption. We did not find an age correlation in natural closure of the residual PV of the peritoneum. Contralateral hernia developed in 14 persons (2.5%) after the operation of unilateral inguinal hernia before laparoscope adoption. But no contralateral hernia developed after April 2003 with laparoscopy. We think that if we use laparoscopy, being a safe and accurate method, to check whether the contralateral residual PV is opened or closed, possible future contralateral operation can be avoided.

Contralateral exploration in children with unilateral inguinal hernia is controversial. This study was done to identify risk factors for the development of contralateral inguinal hernia in patients with unilateral inguinal hernia. The clinical experience of 4,206 inguinal hernias repaired by one pediatric surgeon on 3,358 children at HanYang University Hospital from September 1979 to December 2002 was analyzed. 1,868 (55.6%) hernias occurred on right side, 1,190 (35.4%) on left side, and 300 (8.9%) were bilateral. 2,702 children were boys and 656 were girls (M:F=4.1:1). 170 children of 3,058 children with unilateral hernias (5.6%) developed contralateral inguinal hernia at 1 day to 95 months after herniotomy. 146 children were boys and 24 were girls (M:F=6.1:1). The patients who had had herniotomy before 1 year of age developed contralateral hernia in 17.4%, compared with 5.6% overall average. The earlier the first herniotomy was performed, the more frequently contralateral hernia developed. The occurrence of contralateral inguinal hernia was more frequent in boys (146 of 2,460, 5.9%) than girls (24 of 598, 4.0%) and more frequent after left herniotomy (80 of 1,190, 6.7%) than after right herniotomy (90 of 1,868, 4.8%), but statistically not significant. 52.9% of contralateral inguinal hernia developed within 1 year after hernia repair, and 87.6% developed within 3 years. Routine exploration on contralateral side in children is not necessary.

A 3-year-old boy with purulent otitis media received a chest radiograph as the part of a routine work up. The patient was normal appearing, in no acute distress. The patient's lung and heart sounds were clear and normal. The patient's abdomen was soft, non-distended, and non-tender. An anterior cardiophrenic mass was incidentally identified on the lateral chest radiograph. A computed tomography scan demonstrated a diaphragmatic hernia with bowel loops in the retrosternal space. An exploratory operation revealed a diaphragmatic defect (4 cm in diameter) on the left side of the falciform ligament, through which transverse colon was protruded. There was no hernia sac, and the defect was closed with interrupted No. 2 silk sutures. The child was discharged on the 8th postoperative day without any complications. During 6 months of follow-up period, recurrence was not noticed.

There are considerable controversies in the management of congenital diaphragmatic hernia. By 1997, early operation, routine chest tube on the ipsilateral side and maintainingrespiratory alkalosis by hyperventilation were our principles (period I). With a transition period from 1998 to 1999, delayed operation with sufficient resuscitation, without routine chest tube, and permissive hypercapnia were adopted as our practice. High frequency oscillatory ventilation (HFOV) and nitric oxide (NO) were applied, if necessary, since year 2000(period II). Sixty-seven cases of neonatal Bochdalek hernia from 1989 to 2005 were reviewed retrospectively. There were 33 and 34 cases in period I and II, respectively. The neonatal survival rates were 60.6 % and 73.5 %, respectively, but the difference was not significant. In period I, prematurity, low birth weight, prenatal diagnosis, inborn, and associated anomalies were considered as the significant poor prognostic factors, all of which were converted to nonsignificant in period II. In summary, improved survival was not observed in later period. The factors considered to be significant for poor prognosis were converted to be nonsignificant after change of the management principle. Therefore, we recommend delayed operation after sufficient period of stabilization and the avoidance of the routine insertion of chest tube. The validity of NO and HFOV needs further investigation.

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 “American Academy of Pediatrics (AAP) Section on Surgery hernia survey revisited (J Pediatr Surg 40, 1009-1014, 2005)”. For full-term male baby, most surgeons (85.7 %) perform an elective operation as soon as diagnosis was made. For reducible hernia found in ex-preterm infants already discharged from the neonatal intensive care unit (NICU), 76.2 % of surgeons performed an elective repair under general anesthesia (85.8 %). 42.9 % of the surgeons performed the repair just before discharge. For same-day surgery for the ex-premature baby, the opinion was evenly divided. For an inguinal hernia with a contralateral undescended testis in a preterm baby, 61.9 % of surgeons choose to ‘wait and see’ until 12 month of age. The most important consideration in deciding the timing of surgery of inguinal hernia in preterm baby was the existence of bronchopulmonary dysplasia (82.4 %), episode of apnea/bradycardia on home monitoring (70.6 %). Most surgeons do not explore the contralateral groin during unilateral hernia repair. Laparoscope has not been tried. Most surgeons do not give perioperative analgesics or blood transfusion.

This is a survey on congenital posterolateral diaphragmatic hernia, conducted by the Korean Association of Pediatric Surgeons(KAPS). A registration form for each patient during the 5-year-period between 1998 and 2002 and a questionaire were sent to each member. Twenty-ninemembers in 22 institutions returned completed forms. The average number of patients per surgeon was 1.4 cases a year. The male to female ratio was 1.64: 1, and annual incidencewas 1/14,522 live births. In this review, factors influencing survival in congenital posterolateral diaphragmatic hernia were age at admission, birth weight, time of antenatal diagnosis, birth place, Apgar score, onset time of symptoms and signs, preoperative cardiopulmonary resuscitation, associated anomalies of themusculoskeletal system, central nervous system, or chromosomes, preoperative stabilization, levels of preoperative FiO₂, pH, and AaDO₂, perioperative complications, bilaterality of defect, size of the defect, and presence or absence of hernia sac.

Testicular feminization syndrome (TFS) is a genetic disorder due to androgen insensitivity of the target organs. The most common clinical presentation of complete TFS is inguinal hernia in the infant or primary amenorrhea in the adolescence. A 7-year old phenotypically female patient was seen with a complaint of a right inguinal mass. Under the diagnosis of right inguinal hernia, high ligation was performed. Six months later, the patient showed a left inguinal mass. On operation, the mass looked like a testis. The external genitalia were normal female, but a uterus and ovary were not identified. Chromosome study showed a 46, XY karyotype and the levels of serum testosterone and dihydrotestosterone were increased after HCG stimulation. The patient was diagnosed as complete TFS and underwent bilateral gonadectomy 6 months later.

Inguinal hernia is one of the most common surgical diseases in pediatric patients. But the management of the side opposite the clinically apparent inguinal hernia, the “silent side” is controversial. Four hundred fifty-eight cases of pediatric inguinal hernias, operated by one pediatric surgeon at the Divisionof Pediatric Surgery, Department of Surgery, Chonbuk National University Hospital from January 1998 to December 2002, were reviewed retrospectively to determine the characteristics and significances of the silk-glove test on the side opposite the clinically apparent inguinal hernia. Males were preponderant as 2.8:1. Allhernias were of the indirect type and were repaired by high ligation of the sac. There were 238 (52.0%) right sided hernias, 160 (35.0%) were on the left and 60 (13.0%) were bilateral. The number of patients with a positive silk-glove sign on the contralateral side was 158(39.7%), and 133 of these had a contralateral patent processus vaginalis. Positive predictive value of Silk-glove test was 84.2% (133/158). The silk-glove test is a simple, safe, and relatively accurate method for contralateral exploration in the unilateral inguinal hernias in children.

performed at the time of discharge. There was only one recurrence of adirect inguinal hernia. Necrotizing enterocolitis developed in 17 patients, 11 were operated upon, two had peritoneal drainages, and 9 had enterostomies. Five of 11 surgical infants died after operation and three of the nonsurgical infants died of various complications. Although micropremies have potentially high risks of serious complications and death, the outcome can improve with careful surgical observation and judgment.

The purpose of this retrospective study was to evaluate the effects of diagnostic sonography in pediatric patients with inguinal hernias. The patients were classified into two groups. Group A included the patients who had been operated upon for inguinal hernia in 1980's, when diagnostic sonography was not available. Group B included the patients, operated upon for inguinal hernia from 2001 to 2002, when inguinal sonography was employed to detect potential bilateral hernias. The age distribution, sex ratio, laterality, bilaterality, and concomitant symptoms were compared between group A and group B. There were 296 cases in group A and 377 cases in group B. The prevalent age group was from 1 to 5 years. There was no difference in age group distribution between both groups. The male to female ratio was 5.3:1 in group A and 3.5:1 in group B. The ratio of unilateral to bilateral hernia was 5:1 in group A and 3:1 in group B. In cases with a unilateral hernia, the ratio of right to left was 1.5:1 in group A and 1.8:1 in group B. In cases with bilateral hernia, the simultaneous bilateral hernia was 33 cases (67.4 %) in group A and 75 cases (80.6 %) in group B. The sequential bilateral hernia was 16 cases (32.7 %) in group A and 18 cases (19.4 %) in group B. Although the ratio of bilateral hernia was increased in group B, the portion of the sequential bilateral hernia was significantly decreased in group B. In conclusion, there were no differences in the age distribution and the laterality between group A and B. The ratio of female patients and the incidence of bilateral hernia were increased in group B even though the portion of the sequential bilateral hernia was decreased. This result shows that the preoperative inguinal sonography in unilateral hernia with potential bilateral hernia is useful in early detection of the sequential contralateral hernia.

A lipomatous lesion of the cord is an accidentally encountered structure during the operative repair of inguinal hernia. This lesion has been reported as a lipoma of the cord in adults. However, there is only a limited number of reports in the pediatric age group. To evaluate the prevalence of this lesion in children and in order to review the surgical significancies, 600 hernia operations in 411 children during a period of 4 years from January, 2000 to December, 2003 in the Division of Pediatric Surgery, Department of Surgery, the Catholic University of Korea, were included in this study. There was a total of 31 (5.2%) lipomatous lesions in 25 (6.1%) cases; 3 cases in infants, 17 between 1 to 4 years, and 5 above 5 years of age. Male was more prevalent (male to female ratio 14:11). The laterality of clinical hernia with the lesions was 10 in the right, 13 in the left and 2 in both sides. The patients with ipsilateral lesions to the hernia were 14, contralateral in 5 and bilateral in 6 cases. Excluding 1 case of bilateral lesions in bilateral hernia, 10 lesions were contralateral to the clinical hernias. In 1 case, lipomatous lesion was the sole finding with nonsignificant patent processus vaginalis. Every lesion was suture ligated and resected with gentle traction of the dissected hernia sac. It has not been clearly defined whether the lesion is a stopper or a provocator of the hernia development. However, removal is highly recommended to make a differential diagnosis from the recurrent inguinal hernia in future. The term “lipomatous lesion” seems to be pathologically accurate and must be differentiate from the true lipomas.

Inguinal hernia is the most frequent problem requiring surgery in children. Moreover, subsequent contralateral occurrence after repair of the symptomatic unilateral inguinal hernia(UIH) is not rare. This study is to evaluate the diagnostic value of inguinal ultrasonography (IUS) for potential bilateral inguinal hernia(BIH). A prospective study was performed for preschool children less than 6 years of age who were diagnosed as UIH from July 1999 to December 2000. We selected 58 cases with potential BIH, based on the past history, such as prematurity, ventriculo-peritoneal shunt, family history of BIH, hernia on the left side (LIH), age below 2, female, and contralateral positive silk glove sign on the physical examination. Screening with IUS and bilateral surgical exploration were applied on these cases. Forty-seven cases were males (81.0%) and 11 cases were females(19.0%). Thirty-four were infants. Symptomatic right inguinal hernia (RIH) were 28 (48.3%), and LIH were 30 cases (51.7%). Six cases had no evidence of contralateral patent process vaginalis (PPV) by IUS but showed contralateral PPV by operation, Two cases were suspicious to contralateral PPV under IUS, but operative findings were negative. Fifty cases showed contralateral PPV by IUS as well as operation. The detection rate of contralateral PPV under IUS was 86.2%. The preoperative IUS may reduce contralateral exploration.

Inguinal hernia is a major surgical disease in pediatric surgery, occurring in 3.5% to 5% of all mature newborns and 9% to 11% of all premature babies. The
objective
of this study is to analyze the predisposing factors in association with recurrences of inguinal hernias in infants and children. In the period from January 1995 to September 2001, 1,575 infants and children who had primary inguinal hernias and recurrent inguinal hernias operated on at the Department of Pediatric Surgery at Seoul National University Hospital were evaluated retrospectively. We evaluated the data by medical records and by telephone interview. The sex, age, location of hernia, comorbidity, prematurity, incarceration, interval to operation after incarceration, postoperative complications were analyzed as predisposing factors in associated with hernia recurrence. Operative findings of recurrent inguinal hernia were reviewed. The data were statistically analyzed with Pearson Chi-Square test and Fisher-exact test. A total of eighteen (1.14%) out of 1,575 patients underwent an operation due to recurrent inguinal hernia. In 5 (27.8%) out of 18 recurred patients, institution of the primary herniorrhaphy was our hospital and in the other 13 (72.2%) was outside hospital. No impact on the development of recurrences was seen for sex, age, interval to operation after incarceration, and postoperative complications. The significant predisposing factors of recurrent inguinal hernias were left inguinal hernias (p=0.002), comorbidity (p=0.002), prematurity (p=0.006), incarceration (p=0.017) and technical error of first herniorrhaphy. We expect that knowledge for predisposing factors of recurrent inguinal hernias and experienced skill of pediatric surgeons will decrease recurrence rate in primary inguinal hernia.

Between March 1999 and January 2000, 82 boys with the diagnosis of inguinal hernias (12 bilateral and 70 unilateral hernias), underwent Ultrasound (US) examination of both sides of the groin, a total of 164 inguinal imaging prior to surgery. The patients ages ranged from 3 days to 12 years with a mean of 32.6 months. Ninty four examinations were on the clinically symptomatic side and 70 were on the asymptomatic side. The US criteria for the diagnosis of an inguinal hernia were as follows: 1) visceral hernia, the presence of bowel loops, or omentum in the inguinal canal, 2) communicating hydrocele, the presence of fluid in the processus vaginalis, 3) widening of patent processus vaginalis at the level of nternal inguinal ring. The width of patent processus vaginalis at the level of internal inguinal ring over 4 mm is onsidered an occult hernia. Among the 94 symptomatic groins, US findings showed 31 (33 %) visceral hernias, 18 (19 %) communicating hydroceles, and 38 (41 %) widening of the internal inguinal ring, and 7 (7 %) groins without abnormalities. In 70 asymptomatic groins, there were 4 (6 %) visceral hernias, 5 (7 %) communicating hydroceles, 11 (16 %) widening of the internal inguinal ring, and 50 (70 %) groins without abnormalities. Among the 70 asymptomatic groins there were US abnormalities in 20 (28 %). One hundred and seven groins with positive US findings were surgically explored. Among 107 operated sites, the operative findings were compatible with the US diagnosis in 104, a sensitivity for US of 97.2 %. In patients with US findings of widening of internal inguinal ring (>4 mm), there was patent processus vaginalis in 36 out of 38 symptomatic groins and 10 of 11 asymptomatic groins. The sensitivity of US to the operative findings in widening of internal inguinal ring was 93.8 %. For visceral hernia and communicating hydrocele, the sensitivity of positive US findings was 100 %. Ultrasonography for inguinal hernias appears to be a rapid, reliable, and noninvasive screening diagnostic tool with high positive specificity. Therefore, we recommend the use of US as a routine diagnostic tool in pediatric patients with inguinal hernias and hydroceles.

Congenital diaphragmatic hernia (CDH) in the past was considered a surgical emergency requiring immediate operation. Several groups now advocate preoperative stabilization and delayed surgery. The treatment strategy for CDH in this institution is delayed surgery after preoperative stabilization. The aim of this study was to evaluate the results of delayed surgery. A retrospective review of 16 neonates with CDH was performed. Surfactant, conventional mechanical ventilation, high frequency oscillation, and nitric oxide were utilized for preoperative stabilization as necessary. The difference in outcome between two groups differentiated by the duration of the preoperative stabilization periods with mechanical ventilation (≤ 8 hours and >8 hours) was determined. Chi-square test was used to analyze the data. There were 7 right-sided hernias and 9 left. The average duration of stabilization was 32.4 hours. Hepatic herniation through the defect was found in 6 cases and all died. The most common postoperative complication was pneumothorax. The mortality rate of the right side hernia was higher than the left (85.7% vs. 33.3%, p=0.036). Mortality rate of the group (N=8) whose preoperative stabilization period was 8 hours or less was better than that (N=6) whose preoperative stabilization period was more than 8 hours (25.0% vs. 83.3%, p=0.031). The overall mortality rate was 56.3%. The better prognosis was noticed in left side hernia, no liver herniation, or shorter preoperative stabilization period.

Ventriculoperitoneal shunt (VP shunt) for hydrocephalus is thought to inhibit the closure of processus vaginalis by increasing intraabdominal pressure, thus it promotes the inguinal hernia. We reviewed the incidence and characteristics of the inguinal hernia in VP shunted patients, and tried to estimate the patency rate of processus vaginalis in early childhood. A reprospective review of patients undergone insertion of VP shunt between January 1980 and May 1998 at Seoul National University Children Hospital was done. 262 patients were included in this study. Among them, 28 patients developed inguinal hernia (10.7%). Six patients developed inguinal hernia before the insertion of VP shunt. According to the age of VP shunt, the inguinal hernia developed in 16.2% (12/74) of patients who had undergone VP shunt before 6 months old, 12.4% (11/89) between 6 months and 2 years old and 5.1% (5/99) after 2 years old. Among 22 patients excluding 6 patients who developed hernia before VP shunt, the incidence of inguinal hernia after VP shunt was 8.6% (22/256) with male predominance (M:F=18:4). 8 patients developed inguinal hernia bilaterally (36.4%). It is suggested that at least 14% of processus vaginalis is patent until 2 years old.

Femoral hernias are very uncommon in children and very easily misdiagnosed. During a period of three years, three children of femoral hernia were treated by one pediatric surgeon at Severance Hospital. Only one case was diagnosed correctly before surgery; the other two were thought to be either an indirect inguinal hernia or groin mass. Only one patient had curative hernioplasty (McVay hernioplasty) at the first operation; the other two did not have curative hernioplasty at the first operation. Femoral hernia in childhood is a challenging clinical problem because of its rarity and similar clinical presentation as indirect inguinal hernia. The frequency with which an incidental indirect inguinal hernia sac or patent processus vaginalis can be found at surgery can perpetuate a misdiagnosis. The absence of an expected indirect inguinal hernia sac or an apparent recurrence of an indirect inguinal hernia should lead to consideration of possible femoral hernia.

Despite of advances in perinatal management and treatment modalities congenital diaphragmatic hernia(CDH) remains a frustrating problem. Although the sheep has proven to be a reliable experimental model for the production of intrauterine CDH, the rabbit may have some advantages. These include lower cost, smaller body size, year-round availability, high number of fetuses per pregnancy, and short gestational period. To evaluate the feasibility of the rabbit model of CDH, twenty-seven pregnant New Zealand rabbits were utilized. Hysterotomy and an operative procedure for creating a diaphragmatic defect on gestational day 24 or 25, in two fetuses of each pregnant rabbit were performed. In one fetus of one cornu of the uterus, the left fetal diaphragm was excised through an open thoracotomy(DH group). In another fetus in the other cornu, CDH was created and the trachea clipped(Surgiclip, USSC, Norwalk, Conn., USA) (TL group). Delivery was by Cesarean section on 30 days of gestation. Among twenty- seven pregnant rabbits, 12 in the DH group and eight in the TL group were born alive. The most common herniated organ was the left lobe of the liver. In thee DH group, the lungs were hypoplastic with decreased lung weightlbody weight ratio, reduced numbers of alveoli, thicker media of the pulmonary arteries, and immature alveoli. In TL group, the alveoli were more mature and did not differ from the control animals. In conclusion, (1) pulmonary hypoplasia develops in the fetal rabbit diaphragmatic hernia model and (2) simultaneous tracheal ligation prevents pulmonary hypoplasia.

A total of 335 pediatric inguinal hernias were analyzed at the Department of General Surgery, St. Benedict Hospital, for last 10 years (1986 to 1995). Male patients were predominant(2.25 : 1), and 78.2 % were under 2 years of age. Right side was 1.63 times more frequent than the left. Among the 19 cases of incarcerated hernias, 84 % could be safely reduced preoperatively. Twelve cases(3.6 %) were repaired by the Bassini procedure and 21 cases(6.3 %) by internal ring repair. The majority (90.1 %) however did not require a posterior wall reconstruction. Bilaterality was found in 25 cases(7.5 %), and in 8 cases(2.4 %) subsequent contralateral hernia developed after primary ipsilateral repair. Postoperative complication occurred in 15 cases(4.5 %); scrotal seroma and/ or hematoma(3%), wound infection(0.6 %), and pneumonia(0.9 %). In order to reduce the incidents of complications, children with inguinal hernias should be treated by a pediatric surgeon.

Transmesenteric hernia, a type of internal hernias, is a rare cause of intestinal obstruction. This intraperitoneal hernia has no sac and is formed by protrusion of a loop of bowel through an aperture in the mesentery. Incarceration leads to intestinal obstruction and subsequently, strangulation and gangrene of varing lengths of intestine. This is a case report of 4-year-old girl with transmesenteric herniation of the terminal ileum through a defect in its own mesentery. Strangulation of the affected bowel necessitates resection and primary anastomosis with repair of mesenteric defect. The postoperative course was uneventful. Acute intestinal obstruction in the absence of an external hernia and with no history of a previous surgical procedure suggests the possibility of an internal hernia, especially if the patient has a history of chronic intermittent abdominal distress.

This is a case report of a sliding hiatal hernia with severe gastroesophageal reflux (GER) after repair of congenital diaphragmatic hernia(CDH). It was not possible to determine whether the hiatal hernia is a de novo lesion which was missed at the original operation or a consequence of overzealous repair of the Bochdalek defect at the expense of weakening of the diaphragmatic crura. This case demonstrates that a sliding hiatal hernia can be a cause of severe gastroesophageal reflux that should be managed surgically.

Three hundred and twenty-seven patients of 2,046 inguinal hernia cases primarily repaired at Hanyang University Hospital had the history of incarceration or presented as incarcerated inguinal hernia on admission. Incidence of incarceration of all male hernias was 14.2%(234 patients) and 22.7%(93 patients) of all female cases. Incarceration occurred in 17.3% of all right hernia cases and in 13.7% of all left hernia cases.

The incarceration occurred 52.6% of the hernia patients in the first month of life, 27.3% in the first year, 26.7% in the second year and 7.8% after 2 years of age. Strangulated inguinal hernia occurred in 8 patients: five patients had ovaries involved, two patients intestines, and one patient omentum. Emergency operations were performed on 66 patients(20.2%) because incarcerated hernia could not be reduced by taxis. At the time of operation, the hernia sacs were empty in 140 of 327 patients and the remainders contained omentum(50), small intestine (44), appendix and/or cecum(28), sigmoid colon(2), ovary and/or tube(66), and omental cyst(l).

An elective hernia repair should be performed promptly after presentation of the hernia, especially before 2 years of life because of high incidence of incarceration. In this study, of 327 incarcerated hernia, 187 patients(57.2%) did not have prior history of incarceration and incarceration developed more than 7 days after hernia onset in 95.6%. If the hernia repairs had been performed within 7 days after hernia onset, about half of the incarceration might have been prevented.

The clinical experience of 2,340 inguinal hernia hernia repaired by one pediatic surgeon om 2,079 children at Hanyang University Hospital from September 1979 to December 1993 was analyzed. Of 2,046 patients who had primary hernia repairs at Hanyang University Hospital, 1,636 were male and 410 female, and 55.5% of hernias occurred on the right side, 36.0 % on the left, and 8.6% were bilateral. The patients presented hernia under the age of 12 months were 45.3% and those performed herniotomy under the age of 12 months were 25.5%. Birth weight was less than 2.5kg in III patients(8.7%) of 1,279 data available patients. Ninety(6.6%) of 1,354 data available patients were premature (<37wks gestation). The proportions of bilateral inguinal hernia and the onset age under 12 months of life in low birth weight babies and premature babies were higher than in full - term babies. Incarcerated inguinal hernia occurred in 327 patients(16.0%) of whom 8 patients were strangulated hernias. The occurrence of incarceration inversely related with age of patients. The subsequent contralateral inguinal l1ernia following unilateral hernia repairs occurred in 80 patients(4.3%) among which 72 were male and 8 were female. The incidence of contralateral inguinal hernia was more frequent in boys(4.8%) than girls (2.2%) and in cases after left herniotomy(6.4%) than after right herniotomy(2.9%). Sixty percent of contralateral inguinal hernia developed within 1 year after primary hernia repair. The recurrence of inguinal hernia occurred in 6 patients(0.27%) treated at our hospital primarily. There were one or more associated congenital anomalies in 83 patients of which congenital heart diseases were the most common. Sliding hernia occurred in 25 patients consisted of 5 boys and 20 girls. Family history was noted in 35 patients and there were 28 sets of monozygotic and 3 sets of dizygotic twins.

For the prevention of later contralateral hernia as well as unnecessary contralateral exploration in pediatric patients with unilateral inguinal hernias, a reasionable indication of contralateral exploration is required. To examine the contralateral positivity, a prospective selective contralateral exploration has been performed by the author from Sept. 1985 to Dec. 1993, at Pediatric Surgical Section of the Department of Surgery, Kangnam St. Mary's Hospital, Catholic University Medical College. Among the total 1200 cases of pediatric inguinal hernias, 580 cases of contralateral side were explored at hernia operations, by the indications as; 1)male with infant onset, 2)female of all age, 3)prematurity, 4)profuse ascites due to cirrhosis, nephrotic syndrome, and ventriculoperitoneal shunt, and 5)remarkable silk sign. Overall positive rate was 71.4%, and positive rates of each indication were 80.7%, 70.4%, 73.1 %, 66.7%, and 72.0%, respectively. Right side hernia showed 67.0%, left side 75.7%, and positive familial history 71.8% of contralateral positivities. In male,getting older revealed lower positive rates and the rate suddenly dropped after 12 years of age. Birth order, mother's age at delivery, postmaturity did not show any significant differences between the rates. Recurrence was seen in 3(0.5%) ipsilateral and 2(0.3%) contralateral, both of which were negative esplorations on previons operations. Overall complication rate was 3.8%, including 1 infection, 14 fluid or blood accumulation, 5 edemas, 3 temporary testicular edemas, 2 persisting fevers, 2 enuresis and one delayed recovery from anesthesia. Among 38 cases with contralateral hernias developed after unilateral surgery by authors(6 cases) or surgeons in other institutions, 14 were males with infant onset, 4 were prematurities and 9 were females. Therefore, 27(71.7%) cases were originaly under the contralateral exploration indications. The primary site of the hermia was right in 25 and left in 13. With above results, the following indications for contralateral exploration could be suggested; 1)under one year of age, both sex, 2)prematurity, 3)remarkable silk sign, 4)in the double checked suspicions among males with infant onset, all age females, ascites, left hernia and familial history. After 12 years of age, exploration is not required. Considering complications, contralateral explorations could be considered only in the following situations ; 1)expert, experienced pediatric surgeon, 2)experienced pediatric anesthesiologist, 3)operations could be done smoothly in an hour, 4)good general condition of the patient.