Anesthetic management of a neonate with congenital diaphragmatic hernia under high-frequency oscillatory ventilation.

Perioperative management of a neonate with congenital diaphragmatic hernia (CDH) is challenging because of pulmonary hypoplasia, pulmonary hypertension, and respiratory insufficiency. In this report, we present our intra-operative experience in a 4-days old and 3070 grams CDH neonate. He was admitted to neonatal intensive care unit and intubated due to severe respiratory insufficiency. He showed signs of severe pulmonary hypoplasia and his echocardiography revealed a cardiac dextroversion. The patient was relatively stabilized after four days under combined high-frequency oscillatory ventilation (HFOV) and inhaled nitric oxide (iNO). A corrective surgical intervention was sustained with dopamine, dobutamine, fentanyl and midazolam infusions. Ventilator settings were: 9 cmH2O MAP; 15-Hz frequency; 30 cmH2O amplitude and 55% FiO2. Venous-blood gas analysis indicated pH:7.38 pO2:36.2, pCO2:39.2 with SpO2:98%. We believe that HFOV and iNO combination is an effective alternative for the anesthetic management of CDH cases as it provides better gas exchange and less volutrauma.

Comparison of Single-Port Percutaneous Extraperitoneal Repair and Three-Port Mini-Laparoscopic Repair for Pediatric Inguinal Hernia.

AIM: Laparoscopy has been widely used in surgical practice in pediatric age, and many techniques for laparoscopic hernia repair have been described till now. In this study, we compared two laparoscopic techniques performed by two surgeons; each surgeon practicing only one of the two techniques. MATERIALS AND METHODS: A retrospective analysis was performed on the surgical charts, enrolling 71 patients with uncomplicated inguinal hernia. Patients were divided into two groups according to the type of surgery: (Group A, 24 patients aged 2 months-8 years) laparoscopic percutaneous internal ring suturing technique and (Group B, 47 patients aged 35 days-12 years) three-port mini-laparoscopic technique. The hernia sac was ligated at the level of internal ring, using nonabsorbable 4/0-3/0 suture. Any unexpected contralateral opening was repaired in the same manner for both groups. Follow-up period was 4 months-2 years and 9 months-8 years, respectively. Operative time and complications were analyzed. RESULTS: Operation time (19.58 ± 7.06 minutes versus 35.87 ± 10.34 minutes, P < .001) was shorter in the percutaneous repair group. However, when subdivided by unilateral and bilateral presentation, only unilateral operative time was shorter compared to three-port group. There were no recurrences in Group A, while two recurrences occurred in Group B during the learning curve period. A contralateral opening accompanied the presenting unilateral hernia in 3 cases for Group A and 16 for Group B. One patient had to be converted open resulting from epigastric vessel injury, and postop hydrocele formation was seen in another in Group A. No intraoperative complications were seen in Group B. CONCLUSION: The overall experience shows that laparoscopic repair is a reliable approach regardless of the chosen technique. Percutaneous repair seems to be a less invasive method with shorter operative time, but it is not free of complications according to this series.

Inherited IL-12Rß1 Deficiency in a Child With BCG Adenitis and Oral Candidiasis: A Case Report.

Tuberculosis is a major worldwide problem, and protection from it is achieved mainly by live attenuated bacille Calmette-Guérin vaccine, which is capable of causing disease in immunocompromised host. Oral thrush is abnormal in healthy children, which suggests an underlying immunodeficiency. Mendelian susceptibility to mycobacterial disease is a rare primary immunodeficiency characterized by a selective predisposition to weakly virulent Mycobacteria and Salmonella and also predisposition to chronic mucocutaneous candidiasis. Interleukin 12 receptor ß1 (IL-12Rß1) deficiency is the most common disease of Mendelian susceptibility to mycobacterial disease, and to date only 50 IL-12Rß1 deficient patients with clinical signs of chronic mucocutaneous candidiasis have been reported. We report a 2.5-year-old daughter of consanguineous parents with both regional bacille Calmette-Guérin lymphadenitis and recurrent oral candidiasis carrying biallelic R175W mutation in the IL12RB1 gene, resulting in complete loss of expression of IL-12Rß1. To our knowledge, this is the first report of bacille Calmette-Guérin lymphadenitis with concurrent oral candidiasis displaying such a mutation. New mutations and wide clinical diversities are the indisputable fact of populations with a high rate of consanguineous marriages.

Balloon extraction of a retained rectal foreign body under fluoroscopy, case report and review.

Rectal foreign body impaction in infancy is extremely rare, and the literature does not describe a standard treatment procedure. Extraction by fiberoptic rectosigmoidoscope is advocated as the treatment of choice but is not free of possible complications. It should be also taken into consideration that the type of foreign body has an impact on the timing of treatment. We report a 50-day-old male presenting with a retained broken tip of a mercury thermometer in the rectum. A novel method of diagnostic approach and treatment is discussed.

Management of ovarian cysts during infancy: autoamputation presenting as a possible pitfall.

The widespread use of routine antenatal ultrasound has relatively increased the frequency of intrauterine diagnosis of ovarian cysts. In utero adnexal torsion may present with subsequent autoamputation in some of these lesions. Prenatal and postnatal ultrasonographic findings, however, may not always be relevant in making the correct diagnosis. The authors report on two cases with prenatally-diagnosed hypoechogenic cystic masses. The cysts failed to resolve after a period of conservative management. A laparoscopic approach revealed ovarian autoamputation presenting as cystic mass. Preoperative ultrasound, abdominal CT and MRI failed to detect the presence of autoamputation. The present report discusses the possibility of an otherwise silent ovarian autoamputation, which may necessitate laparoscopic intervention for correct diagnosis, in neonates presenting with persistent ovarian cysts.

Thoracoscopic assessment of mediastinal tuberculous lymphadenitis in a 4-month-old child.

Isolated mediastinal tuberculous lymphadenitis is a relatively common entity in children, second in frequency after cervical localization. In the absence of an accompanying parenchymal lesion, mediastinal tuberculous lymphadenitis may pose a diagnostic dilemma on admission and must be distinguished from other causes of mediastinal masses. Bronchoscopy is suggested as a diagnostic tool where tuberculosis cannot be excluded by radiology or specific skin tests. Thoracotomy and excision is reported as necessary to treat the obstructive symptoms. In this report, the diagnostic and therapeutic feasibility of thoracoscopic mediastinal node biopsy in a 4-month-old presenting mediastinal tuberculous lymphadenitis is reported.

Minimal access surgical repair of Morgagni hernia: the fate of the unresected hernia sac.

Treatment of retrosternal diaphragmatic (Morgagni) hernia is composed of a simple surgical closure of the retrosternal opening either conventionally by open abdominal or thoracic approaches, or more recently, by using minimal access surgery (MAS). Clinical experience using the latter approach is very limited in children. Removal of the hernia sac is a controversial issue, since the sac is said to carry the risk of cyst formation or show spontaneous resolution. This issue represents a 7-year-old boy with Morgagni hernia that was successfully repaired by the MAS approach. The hernia sac was not resected because of adhesion to the adjacent tissues. The patient was readmitted with fluid accumulation within the remaining sac 2 months after the operation. A complete spontaneous resolution was observed within 2 months under conservative follow-up. To our knowledge, the temporary complication described in this paper has not been published in the literature.

Esophageal muscle cell interaction with biopolymers.

BACKGROUND: The in vitro interactions of esophageal smooth muscle cells (SMCs) with synthetic absorbable polymers were tested and artificial muscle tissues harvested from subcutaneous implantation were examined. MATERIAL/METHODS: Esophageal tissue samples from adult and fetal (25-day gestational age) rabbits were cut into small pieces and cultured in Dulbecco's Modified Eagle Medium supplemented with 10% fetal bovine serum. Growing cells were identified as SMCs by immunostaining for anti-actin and anti-myosin antibodies. Equal volumes of agar gel and medium were mixed and used for 3-D culture. 5x10(5) cells and 1 mg polyglycolic acid (PGA) and poly-lactide-co-glycolide acid (PLGA) fibers were seeded in six-well tissue culture plates. On days 2 and 7 growing cells were counted by a hemocytometer and cell-polymer interactions were evaluated with light microscopy. Adult and fetal SMCs were seeded onto the PGA and PLGA scaffolds, cultivated for two weeks, and implanted subcutaneously on the backs of the rabbits. Cell-polymer implants were retrieved after four weeks and muscle formation was evaluated histologically and immunohistochemically. RESULTS: Growing cells stained positive for actin and myosin proteins. Cell-polymer interactions were poor after 24 hours, whereas intensive attachment to the fibers was detected 48 hours following cultivation. Both fiber materials supported cell proliferation. PLGA scaffolds improved muscle formation more efficiently than PGA, and fetal and adult SMCs showed similar mass quality. CONCLUSIONS: Scaffolds are important as cell-carrying vehicles, and material-cell interactions should be tested before application. A 3-D culture prepared with agar gel and medium is practical for testing material toxicity.

Incomplete duodenal obstruction in a newborn.

A case of newborn with incomplete duodenal obstruction caused by superior mesenteric artery syndrome has been presented with this report. A full term, 1-day-old baby girl was referred to our hospital because of recurrent bilious vomiting since birth and upper gastrointestinal barium study revealed the incomplete obstruction at the 3rd part of the duodenum with a vertical abrupt cutoff. The diagnosis of superior mesenteric artery syndrome was made with ultrasonography and duodenojejunostomy was carried out. Although it is extremely rare, superior mesenteric artery syndrome should also be considered as one of the rare cause of incomplete duodenal obstruction in newborn period.

Bronchobiliary fistula detected with hepatobiliary scintigraphy.

Bile leakage into the thoracic cavity is a rare complication of invasive cancer. A 12-year-old boy was diagnosed with undifferentiated sarcoma of the right lobe of the liver invading the diaphragm. An extended right hepatectomy and total resection of the mass was performed, leaving a patchy tumoral invasion at the anterior diaphragmatic surface. Surgery was followed with a combined chemotherapy regimen. In the sixth postoperative month, he was readmitted with bilious expectoration. Tc-99m mebrofenin hepatobiliary scintigraphy revealed radiotracer accumulation in the right hemithorax. Bile leakage into the right thoracic cavity was diagnosed based on the hepatobiliary scintigraphic findings. For this patient; hepatobiliary scintigraphy, which is routinely used to visualize the liver and biliary tree, provided a noninvasive mean for the precise diagnosis of a bronchobiliary fistula. The fistula was then confirmed and corrected with surgery. The patient recovered uneventfully.

Peritoneal tuberculosis with elevated serum CA 125 level mimicking advanced ovarian carcinoma in an adolescent.

Differential diagnosis between tuberculous peritonitis and peritonitis carcinomatosis is extremely difficult in patients with ascites, peritoneal implants and elevated CA 125 level. A 16-year-old girl presented with abdominal distention, intermittent fever and weight loss. Physical examination and radiologic studies revealed massive ascites, generalized peritoneal thickening and slightly enlarged right ovary with a cystic mass and left pleural effusion. Serum CA 125 was 939 U/L (normal range: 0-35 U/L) and other tumor markers including alpha fetoprotein (AFP) and beta-human chorionic gonadotropin (HCG) were within normal range. Acid-fast stain and culture were negative for Mycobacterium tuberculosis. Diagnostic laparoscopy and biopsy were performed with the presumptive diagnosis of peritonitis carcinomatosis, and histologic examination revealed multiple granulomas with epithelioid cells and caseification necrosis which confirmed tuberculosis. Quadruple anti-tuberculosis treatment was administered and the patient's clinical findings and serum CA 125 level returned to normal. In conclusion, tuberculous peritonitis should be considered in the differential diagnosis of patients with ascites and elevated serum CA 125. This marker may be useful in monitoring treatment response.

Lumbar teratoma presenting intradural and extramedullary extension in a neonate.

BACKGROUND CONTEXT: The association of teratomas and spinal malformations such as spina bifida, partial sacral agenesis, hemivertebrae, and diastematomyelia has been described in the literature. Reported cases, however, are mainly presacral or sacrococcygeal with an extremely rare presentation of intradural extension. PURPOSE: A case of lumbar teratoma with an intradural extension and extramedullary component and the clinical outcome following surgical treatment are reported. STUDY DESIGN/SETTING: To our knowledge, among the reported teratoma cases with an intradural extension and extramedullary component, our case has a distinguishing feature regarding the involvement of the lumbar spine. It is also the first case, showing no neurological deficit during the postoperative period. METHODS: A full-term, female infant presented with a 30 x 30 x 10 mm lumbar mass covered with normal skin. The mass contained an irregular, bone-like, hard and mobile material accompanying cystic components. Magnetic resonance imaging revealed a total closure defect of the first and second lumbar laminae and a subcutaneous mass with intradural extension. The lesion was found to penetrate the dural sac through an extended exposure from T12 to L3. There was no firm attachment of the intradural, extramedullary component of the lesion. Total removal of the tumor was achieved. RESULTS: The patient was discharged on day 7 without any neurological deficit or sign of hydrocephalus. The pathological examination showed a benign teratoma containing mature cartilage, muscle, adipose tissue, and glandular tissue. Follow-up at 2 years showed no recurrence or neurological deficit and a normal sphincter tone. Urodynamic evaluation was within normal limits. CONCLUSION: Accompanying a spinal dysraphic state, the mature teratoma in our case may support the idea of a tumor actually arising from a dysraphism and growing outward to produce the mass.

A continuous debate on contralateral processus vaginalis: evaluation technique and approach to patency.

We present our experience with the transinguinal diagnostic laparoscopy and discuss its efficacy in evaluating the contralateral side in unilateral inguinal hernias. The possible influence of the initial side of hernia, age and gender on recognized bilateralism were also evaluated in two study groups (diagnostic laparoscopy vs clinical diagnosis). In this retrospective study, we evaluated 36 bilateral, 158 left-sided and 303 right-sided consecutive inguinal hernia cases. A total of 211 out of 461 unilateral cases underwent hernia repair and transinguinal laparoscopic evaluation of the contralateral side. Complications and difficulties of the technique, the mean duration of laparoscopy and operative times were additionally analyzed from operation charts. In this study, bilateralism was determined by transinguinal laparoscopy in Group 1 (children with a contralateral patent processus vaginalis) and clinically in Group 2 those who had a metachronous hernia. The patients were also analyzed according to the side of the inguinal hernia, age and gender in both groups. We found an overall positive contralateral patency in 41 cases (19.4%). We failed to perform a successful diagnostic laparoscopy in six cases (2.7%). No anesthetic or surgical complications were noted. Transinguinal laparoscopy did not add any considerable time to the mean operation time. The incidence of bilateralism in Group 1 was higher in girls than boys. Clinically detected bilateralism was significantly higher in 0-6 month age group and contralateral patency detected via laparoscopy was high in all other age groups. We may conclude that transinguinal diagnostic laparoscopy is a feasible technique in children. We advocate its use as a tool with minimal complication risk even in the hands of novice.

Diffuse neonatal abdominal lymphangiomatosis: management by limited surgical excision and sclerotherapy.

Complete excision of diffuse abdominal lymphangiomatosis in the newborn is next to impossible. A 3-day-old female infant was found to have diffuse abdominal lymphangiomatosis predominantly in the left mesocolon and retroperitoneum. Initial management was by marsupialization, which was complicated by chylous ascites requiring periodic paracentesis and nutritional support. At the age of 45 days, left hemicolectomy and partial excision of the retroperitoneal cysts were performed together with intracystic injection of OK-432 into the residual cysts. The patient's progress after the second operation was satisfactory. Initial marsupialization followed by delayed partial resection together with injection of OK-432 into the residual cysts is an effective method of managing diffuse abdominal lymphangiomatosis in the newborn.

Advantages of video-assisted approach in detecting epididymal anomalies and treatment of nonpalpable testis.

In children with a nonpalpable abdominal testis, preoperative localization is very helpful prior to surgical investigation both to reduce the time required and to plan the correct surgical procedure. It is no doubt that laparoscopy is useful in both diagnosis and management of nonpalpable testis. In this study, 15 patients with 17 nonpalpable testes admitted to our clinic in 2 years period were evaluated retrospectively. Ultrasound study was performed in all patients after detailed physical examination. Testicular location, size, and configuration of epididymis were determined by laparoscopy and compared with ultrasound findings. Laparoscopy, in conjunction with ultrasound, permits not only a better localization and anatomy of the testis but is also superior in planning the best choice of management. We believe that the video-assisted inguinal approach is a feasible method in the treatment of nonpalpable testis when compared to pure open surgery.

Early enteral feeding in newborn surgical patients.

OBJECTIVE: We report the results of a multicenter prospective trial of early enteral trophic feeding in a group of 56 neonates who required abdominal surgery for a variety of congenital anomalies. METHODS: In this clinical study, 33 neonates were fed in the early postoperative period (early enteral nutrition [EEN] group), and the remaining 23 (control [C] group) were fasted until resolution of postoperative ileus. Patients in the EEN group (Kocaeli feeding protocol) received 3 to 5 mL of breast milk every hour through a nasogastric feeding tube, starting a mean of 12 h (8 to 20 h) after surgery. The nasogastric tube was clamped for 40 min after each infusion and then opened for drainage. Groups were further divided into two subgroups according to whether an intestinal anastomosis or laparotomy was performed. The change in daily gastric drainage, time to first stool, day of toleration to full oral feeding, and length of hospital stay were compared. Blood bilirubin levels, white blood cell count, and C-reactive protein levels were monitored. RESULTS: The time to first stool and day of toleration to full oral feeding occurred significantly sooner, whereas nasogastric tube drainage duration and hospital stay were significantly shorter in the EEN-anastomosis group than in the C-anastomosis group. Time to first stool occurred significantly sooner in the EEN-laparotomy group than in the C-laparotomy group, although other parameters did not differ. Neither anastomotic leakage nor dehiscence was observed in any group. There were two cases of wound infection and two of exitus among patients in the C group. CONCLUSION: Postoperative, early intragastric, small-volume breast milk feeding is well tolerated by newborns. It is a reliable and feasible approach in neonates even in the presence of an intestinal anastomosis after abdominal surgery.

First report of overactive detrusor in association with hypospadias detected by urodynamic screening.

AIMS: The purpose of this study was to determine urodynamic features in hypospadic patients. METHODS: Thirty-seven patients with hypospadias (distal: 31, proximal: 6) underwent preoperative urodynamic study according to International Continence Society (ICS) recommendations. Statistical analysis were done for comparison between urethral obstruction and non-obstruction in patients with detrusor overactivity (DO) plus the relationship of DO with localization of hipospadias. RESULTS: Nearly 45.9% of the patients showed overactive detrusor. Urethral obstruction was found in 60.8% of the patients. The rate of DO was higher in proximal hipospadias, and urethral obstruction than distal type, and non-obstructed patients (P > 0.005). The means overall cystometric capacity, maximum voiding detrusor pressure and maximal urinary flow measured were 132.6 +/- 111.14 ml (range 21-610), 72 +/- 53 cmH2O (range 12-181), and 7.9 +/- 7.1 ml/sec (range 2-30 ml/sec), respectively. CONCLUSIONS: To the best of our knowledge, this is the first study ever to show that overactive detrusor is an accompanying entity in the hypospadic patients.

A child with undifferentiated sarcoma of the liver complicated with bronchobiliary fistula and detected by hepatobiliary scintigraphy.

Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant mesenchymal tumor principally affecting patients of pediatric age. Bronchobiliary fistula is a very rare complication in patients with liver tumor. To the authors' knowledge, this is the first report of a bronchobiliary fistula resulting from tumor invasion in a child with liver sarcoma. A 12-year-old boy was diagnosed to have USL of the right liver lobe, invading the diaphragm. An extended right hepatectomy and total resection of the mass was performed, leaving patchy tumoral invasion of the anterior diaphragmatic surface followed by combined chemotherapy regimen. Six months after the operation, the presence of bilious sputum suggested a bronchobiliary fistula, which was confirmed by hepatobiliary scintigraphy. The patient underwent a right thoracotomy and fistula division. Although bronchobiliary fistula is a very rare complication in patients with hepatic tumors, suspicion in the appropriate clinical setting is necessary to recognize this problem. Hepatobiliary scintigraphy is the useful diagnostic procedure to define bronchobiliary fistula in children with liver tumor and clinical suspicion of bronchobiliary fistula.

Transient antiphospholipid syndrome in an infant with segmental small bowel infarction.

The clinical picture of venous or arterial thrombosis in the presence of circulating antiphospholipid antibodies is referred to as the antiphospholipid syndrome. A 5-month-old baby girl who was quite healthy so far was referred to our clinic with irritability, vomiting, and abdominal distension for 30 hours. Surgical exploration exposed a gangrenous ileal segment about 15 cm long. The postoperative period was unremarkable. Investigation to identify the risk factors for mesenteric thrombosis found anticardiolipin antibodies (isotype Ig G) and decreased protein C level. Protein S and antithrombin III were within normal levels. Hb electrophoresis results showed no HbS, and neither Factor V Leiden nor prothrombin 20210 mutations were detected. Eight months postoperatively, anticardiolipin antibodies were found within normal levels. Lupus anticoagulant, ds DNA, and ss DNA were negative. Direct coombs test and protein C, C3, and C4 were also within normal levels. She had no thrombotic episode in the 24 months postoperatively, although no anticoagulant medication was administered. To the authors' knowledge this case is the first report of segmental intestinal infarction in transient antiphospholipid syndrome in the pediatric population.