Umbilical hernia following gastroschisis closure: a common event?

PURPOSE: To assess incidence and natural history of umbilical hernia following sutured and sutureless gastroschisis closure. METHODS: With audit approval, we undertook a retrospective clinical record review of all gastroschisis closures in our institution (2007-2013). Patient demographics, gastroschisis closure method and umbilical hernia occurrence were recorded. Data, presented as median (range), underwent appropriate statistical analysis. RESULTS: Fifty-three patients were identified, gestation 36 weeks (31-38), birth weight 2.39 kg (1-3.52) and 23 (43 %) were male. Fourteen patients (26 %) underwent sutureless closure: 12 primary, 2 staged; and 39 (74 %) sutured closure: 19 primary, 20 staged. Sutured closure was interrupted sutures in 24 patients, 11 pursestring and 4 not specified. Fifty patients were followed-up over 53 months (10-101) and 22 (44 %) developed umbilical hernias. There was a significantly greater hernia incidence following sutureless closure (p = 0.0002). In sutured closure, pursestring technique had the highest hernia rate (64 %). Seven patients underwent operative hernia closure; three secondary to another procedure. Seven patients had their hernias resolve. One patient was lost to follow-up and seven remain under observation with no reported complications. CONCLUSIONS: There is a significant umbilical hernia incidence following sutureless and pursestring sutured gastroschisis closure. This has not led to complications and the majority have not undergone repair.

Esophageal atresia and malrotation: what association?

INTRODUCTION: Esophageal atresia/tracheo-esophageal fistula (EA/TEF) has an incidence of approximately 1:3,500. The incidence of malrotation is thought to be 1:200-500. We attempted to define the incidence of a combination and discuss the implications. METHODS: This was a retrospective review of all patients admitted to a single institution with a diagnosis of EA or EA/TEF or TEF between April 1981 and January 2013. Patients were included if the position of the duodeno-jejunal flexure (DJF) was determined by upper GI contrast study (UGIS), surgery or post-mortem. RESULTS: Case notes were reviewed for 235 patients. In the EA type A group, 3/28 (11 %; 95 % CI 3.7-27.2 %) had malrotation, significantly higher than the reported incidence of malrotation in the general population (p = 0.0008). All three patients in this group were symptomatic with one patient found to have a volvulus at emergency surgery. In the type C group, 6/196 (3 %, 95 % CI 1.4-6.5 %) had malrotation, significantly higher than the incidence reported for the general population (p = 0.0033) but not significantly different to that of the type A group (p = 0.0878). There were no patients with malrotation identified in any other EA/TEF type. In total, 9/235 (3.8 %; 95 % CI 2.0-7.2 %) patients with EA had malrotation, significantly higher than the 5/1,050 (0.48 %) reported for the general population (p = 0.0002). CONCLUSION: There is a high incidence of malrotation in patients with pure EA. In the type A group an attempt to identify the DJF position at gastrostomy siting and/or performance of UGIS in the neonatal period should be undertaken. There should also be a low threshold for UGIS in all EA/TEF patients.

The role of parenteral nutrition following surgery for duodenal atresia or stenosis.

PURPOSE: In our institution, some children routinely receive parenteral nutrition (PN) following surgery for duodenal atresia/stenosis, while others do not. Our aim was to compare growth and infection rate between these two treatment strategies. METHODS: This was a retrospective study of all children undergoing surgery for duodenal atresia/stenosis over 7 years. RESULTS: Of the 54 children, 19 commenced PN soon after surgery (the 'Initial PN' group). Of the remaining 35 children, 13 (37 %) subsequently required PN (the 'Delayed PN' group). The remaining 22 never received PN (the 'Never PN' group). The proportion of patients experiencing clinically suspected sepsis was higher in those receiving PN ('Initial' plus 'Delayed'; 41 %) compared with those who never received PN (14 %; p = 0.04). The 'Initial PN' and 'Never PN' groups did not show a significant change in weight Z score over time. However, the 'Delayed PN' group showed a significant decrease in weight Z scores from the time of operation to the time of achieving full enteral feeds, and failed to catch up by the time of last follow-up. CONCLUSION: Children with duodenal atresia/stenosis can be managed without PN. However, a third of these children subsequently require PN, lose weight centiles, and have a high rate of sepsis.

Morbidity after ganglioneuroma excision: is surgery necessary?

INTRODUCTION: Ganglioneuroma (GN), the benign form of peripheral neuroblastic tumour, is often asymptomatic and the diagnosis can be incidental. Our aim was to evaluate the incidence of complications after surgical treatment following diagnosis of this tumour. MATERIAL AND METHODS: 24 consecutive children were diagnosed with GN in our centre between January 1989 and December 2009. All patients had negative urinary catecholamines and/or biopsy confirming the diagnosis of GN. Data are reported as mean ± SD. RESULTS: Age at diagnosis was 73 ± 43 months. The most common presentation was respiratory symptoms and/or abdominal pain; 9 (38%) patients were asymptomatic. Tumour location was in the chest (n=14), abdomen (n=7), or pelvis (n=3). 23 children (9 asymptomatic) were operated on; 1 child with a thoracic mass did not undergo surgery because of severe neurological impairments from birth unrelated to GN. 13 children (4 asymptomatic) had a thoracotomy, 8 children (4 asymptomatic) had laparotomy, and 2 (1 asymptomatic) underwent perineal resection. A macroscopically complete surgical excision was performed in 17 cases (74%) and a macroscopically near-complete excision in 6 (26%). At histological examination, resection margins contained tumour in 10 patients (43%) and were free of tumour in the remaining 13 (57%). 7 children (30%) had complications after surgery including 3 patients with Horner's syndrome (which persisted in 2), 1 with chylothorax, 1 with pneumothorax, 1 with pain in the arm, and 1 who developed adhesive intestinal obstruction. 2 children received adjuvant chemotherapy. We re-evaluated the histology specimens according to the International Neuroblastoma Pathology Classification and found that the diagnosis of GN was confirmed in 20 cases (83%), while intermixed ganglioneuroblastoma (iGNB) was diagnosed in 4 patients (17%). At 33.5 ± 40 months (range 1-137) follow-up, all 24 patients, including the child not operated on and the children with incomplete resection or iGNB, are alive with no tumour progression or recurrence. CONCLUSIONS: GN excision is associated with postoperative complications which can be persistent and may affect the quality of life of survivors. In our series we did not observe tumour progression in spite of incomplete excision. The rationale for GN excision should be revisited.

Surgical repair of incarcerated inguinal hernia in children: laparoscopic or open?

PURPOSE: The management of Incarcerated Inguinal Hernia (IIH) in children is challenging and may be associated with complications. We aimed to compare the outcomes of laparoscopic vs. open repair of IIH. METHODS: With institutional ethical approval (09SG13), we reviewed the notes of 63 consecutive children who were admitted to a single hospital with the diagnosis of IIH between 2000 and 2008. Data are reported as median (range). Groups were compared by chi-squared or t-tests as appropriate. RESULTS: · Open repair (n=35): There were 21 children with right and 14 with left IIH. 2 patients also had contralateral reducible inguinal hernia. Small bowel resection was required in 2 children. · Laparoscopic repair (n=28): All children had unilateral IIH (19 right sided, 9 left sided). 15 children (54%) with no clinical evidence of contralateral hernia, had contralateral patent processus vaginalis at laparoscopy, which was also repaired. The groups were similar with regard to gender, age at surgery, history of prematurity, interval between admission and surgery, and proportion of patients with successful preoperative manual reduction. However, the duration of operation was longer in the laparoscopy group (p=0.01). Time to full feeds and length of hospital stay were similar in both groups. Postoperative follow-up was 3.5 months (1-36), which was similar in both groups. 5 patients in the group undergoing open repair had serious complications: 1 vas transaction, 1 acquired undescended testis, 2 testicular atrophy and 1 recurrence. The laparoscopic group had a single recurrence. CONCLUSION: Open repair of incarcerated inguinal hernia is associated with serious complications. The laparoscopic technique appears safe, avoids the difficult dissection of an oedematous sac in the groin, allows inspection of the reduced hernia content and permits the repair of a contralateral patent processus vaginalis if present.

Umbilical polyp in infants and children.

AIM OF THE STUDY: The umbilical polyp is a rare congenital lesion resulting from the persistence of omphalomesenteric duct (OMD) enteric mucosa at the umbilicus. Exploration of the abdomen to exclude the presence of associated OMD remnants is controversial. The aim of this study was to evaluate the need for peritoneal cavity exploration in children with umbilical polyp. METHODS: All umbilical lesions (n = 53) excised between 1995 and 2005 in a single institution were reviewed to identify patients with umbilical polyp (n = 13). This is characterised histologically by the presence of gastrointestinal mucosa. A follow-up study of patients with umbilical polyp was performed. Data are reported as median (range). RESULTS AND CONCLUSIONS: All 13 patients underwent excision of an umbilical polyp at a median age of 15.1 months (3.1 - 80.5). All presented with a discharging polyp (associated with bleeding in 9) which did not respond to topical silver nitrate. Median diameter of the lesions was 0.5 cm (0.2 - 1). Histology revealed the presence of small bowel mucosa in 11 (associated with pancreatic tissue in 1 and gastric mucosa in 1) and large bowel mucosa in 2. All patients underwent inspection and probing of the base of the polyp after its excision. In 6 patients an associated OMD anomaly was suspected and exploration of the peritoneal cavity was performed (mini-laparotomy in 5 and laparoscopy in 1). No OMD anomaly was found. The 7 children who did not undergo exploration of the abdominal cavity remain asymptomatic after 5.8 years (0.9 - 13.7) follow-up. An umbilical polyp can be present in the absence of other OMD anomalies. Exploration of the peritoneal cavity in children with an umbilical polyp does not seem to be necessary.

Laparoscopic surgery in children is associated with an intraoperative hypermetabolic response.

BACKGROUND: Laparoscopic surgery is thought to be associated with a reduced metabolic response compared to open surgery. Oxygen consumption (VO2) and energy metabolism during laparoscopic surgery have not been characterized in children. METHODS: We measured respiratory gas exchange intraoperatively in children undergoing 19 open and 20 laparoscopic procedures. Premature infants and patients with metabolic, renal, and cardiac abnormalities were excluded. Anesthesia was standardized. Unheated carbon dioxide was used for insufflation. VO2 was measured by indirect calorimetry. Core temperature was measured using an esophageal temperature probe. RESULTS: We found a steady increase in VO2 during laparoscopy. The increase in VO2 was more marked in younger children and was associated with a significant rise in core temperature. Open surgery was not associated with significant changes in core temperature or VO2. CONCLUSIONS: Laparoscopy in children is associated with an intraoperative hypermetabolic response characterized by increased oxygen consumption and core temperature. These changes are more marked in younger children.

Echocardiographic assessment of conjoined twins.

OBJECTIVE: To determine the accuracy of prenatal and postnatal echocardiography in delineating the degree of cardiac fusion, intracardiac anatomy (ICA), and ventricular function of 23 sets of conjoined twins with thoracic level fusion presenting to a single centre over a 20 year period. METHODS: 13 thoracopagus, 5 thoraco-omphalopagus, and 5 parapagus pairs presenting to the authors' institution between 1985 and 2004 inclusive were assessed. Echocardiographic data were analysed together with operative intervention and outcome. Twins were classified according to the degree of cardiac fusion: separate hearts and pericardium (group A, n = 5), separate hearts and common pericardium (group B, n = 7), fused atria and separate ventricles (group C, n = 2), and fused atria and ventricles (group D, n = 9). RESULTS: The degree of cardiac fusion was correctly diagnosed in all but one set. ICA was correctly diagnosed in all cases, although the antenatal diagnosis was revised postnatally in three cases. Abnormal ICA was found in one twin only in two group A pairs, one group B pair, and both group C pairs. All group D twins had abnormal anatomy. Ventricular function was good in all twins scanned prenatally, and postnatally function correlated well with clinical condition. Thirteen sets of twins in groups A-C were surgically separated; 16 of 26 survived. None from groups C or D survived. CONCLUSIONS: Prenatal and postnatal echocardiography accurately delineates cardiac fusion, ICA, and ventricular function in the majority of twins with thoracic level fusion. It is integral in assessing feasibility of separation. The outcome in twins with fused hearts remains dismal.

Resection and primary anastomosis is a valid surgical option for infants with necrotizing enterocolitis who weigh less than 1000 g.

HYPOTHESIS: Primary anastomosis following intestinal resection is a valid surgical option in the treatment of infants with necrotizing enterocolitis (NEC) who weigh less than 1000 g. DESIGN: Retrospective case series. SETTING: Tertiary neonatal surgery referral center. PATIENTS: All infants with confirmed NEC weighing less than 1000 g admitted to our intensive care unit over 4 years. INTERVENTION: For infants requiring laparotomy and intestinal resection, primary anastomosis was performed whenever the clinical condition permitted. MAIN OUTCOME MEASURES: Short- and long-term survival, length of intensive care unit stay, and complications. RESULTS: Fifty-one infants with NEC who weighed less than 1000 g were admitted during the study period. Twelve infants underwent intestinal resection and primary anastomosis (median weight at surgery, 0.83 kg; range, 0.6-0.96 kg). One infant developed recurrent NEC, requiring further surgery, but there were no anastomotic leakages and no strictures. The median postoperative stay on our intensive care unit was 14 days (range, 2-49 days). All 12 infants survived their episode of acute NEC, and 8 are alive, with a median follow-up of 34.2 months (range, 4.7-48.4 months). Only 1 death was related to NEC. During the same period, 14 infants underwent intestinal resection and stoma formation. Ten survived the acute episode, and 6 are alive at a median follow-up of 24.0 months (range, 13.1-33.9 months). The median postoperative intensive care unit stay was 18 days (range, 2-74 days). Necrotizing enterocolitis-related complications occurred in 8 of these infants. CONCLUSIONS: The outcome of infants with NEC who weigh less than 1000 g and undergo primary anastomosis is comparable to that of infants treated using alternative techniques. It is, therefore, a valid surgical option in selected infants. The mortality in this group of infants is high.

Peritoneovenous shunting is an effective treatment for intractable ascites.

AIM AND METHODS: A retrospective review was carried out of children undergoing peritoneovenous shunting for intractable ascites. RESULTS: 11 children, aged 3 months to 12 years (median 31 months) underwent peritoneovenous shunting over the past 17 years. The duration of ascites ranged from one month to 2.5 years (median two months). The primary pathology consisted of previous surgery in eight (three neuroblastoma, one renal carcinoma, one hepatoblastoma, one adrenal teratoma, one renal artery stenosis, and one diaphragmatic hernia), and cytomegalovirus hepatitis, lymphatic hypoplasia, and lymphohistiocytosis in one patient each. All patients had failed to respond to previous treatment including peritoneal drainage in six patients, diuretics in five, and parenteral nutrition in five. There were no intraoperative problems. Postoperative complications included pulmonary oedema in three patients, shunt occlusion in three, infection in two, and wound leakage in one. Ascites resolved after shunting in 10 patients. Five shunts were removed one to three years after insertion without recurrence of ascites. Three others are free of ascites with shunts in place for less than one year postoperatively. Three children died from their underlying disease: two after resolution of ascites (neuroblastoma) and one in whom the ascites failed to resolve (lymphohisticytosis). CONCLUSIONS: Peritoneovenous shunting is an effective treatment for symptomatic intractable ascites in children (10 of 11 successful cases in this series). Elective removal of the shunt after one year is recommended.

Retrospective comparison of open versus laparoscopic pyloromyotomy.

BACKGROUND: Laparoscopic pyloromyotomy is gaining popularity in the management of pyloric stenosis. However, there is no unequivocal evidence in favour of the laparoscopic over the open approach. This paper reports an experience with laparoscopic pyloromyotomy and an attempt to identify any benefit over the open procedure. METHODS: This was a retrospective review of all 87 pyloromyotomies performed at this institution for pyloric stenosis over the 39 months since the first laparoscopic pyloromyotomy was performed. RESULTS: Data for 39 infants who underwent laparoscopic pyloromyotomy were compared with those for 38 infants who underwent pyloromyotomy via a periumbilical incision. Patient demographics were similar between the two groups. The duration of operation was longer for laparoscopic pyloromyotomy than for the open procedure (median 50 versus 30 min; P = 0.001). There were no differences in recovery time, postoperative length of hospital stay, complication rates and postoperative analgesia requirements between the two groups. CONCLUSION: Laparoscopic pyloromyotomy has been incorporated successfully into the authors' standard working practice. Complication rates recovery times were similar to those achievable with the open procedure. There was no clear benefit of one approach over the other.

Reduction in visceral slide is a good sign of underlying postoperative viscero-parietal adhesions in children.

BACKGROUND/PURPOSE: Viscera stuck to the anterior abdominal wall from previous surgery risk injury during laparoscopic surgery. A prospective study was conducted to determine if these adhesions are detectable on ultrasound scan by showing a reduction in the normal visceral slide. METHODS: Patients undergoing laparoscopic procedure after a previous laparotomy underwent preoperative real-time ultrasound scan to observe if viscera slides freely under the abdominal wall. A reduction in slide was considered a positive sign of underlying adhesions. These findings were correlated with the operative findings. RESULTS: Anterior abdominal wall scans were performed on 17 children. Reduced visceral slide was seen in 10. Viscero-parietal adhesions were found in 9 of 10 patients. Visceral slide was reduced in a very localized area in 6 patients, and, in these, a loop of bowel (n = 3), liver and bowel (n = 2), or liver (n = 1) was adherent. In 4, reduced visceral slide was seen over a wide area. Extensive adhesions were found in 3 of 4. One renal transplant patient with peritonitis had a false-positive ultrasound scan. At laparotomy there were no adhesions. The peritonitis is thought to have prevented an adequate examination. Seven patients had normal visceral slide. Of these, 4 had no adhesions, but 3 children had flimsy omental adhesions. The sensitivity and specificity of visceral slide in predicting adhesions were 75% and 80%, respectively. CONCLUSIONS: Reduction in visceral slide is a good sign of underlying postoperative viscero-parietal adhesions. Ultrasonographic mapping of the abdominal wall may be useful in selecting an adhesion-free site for trocar insertion in children with previous operations requiring laparoscopic procedures.

Experience in the management of conjoined twins.

BACKGROUND: Conjoined twins occur in approximately one in 200 000 live births. Their management is often extremely complex and experience with large numbers is restricted to a few centres worldwide. METHODS: An analysis of 17 sets of symmetrical conjoined twins managed by a single team of surgeons over a 16-year period was undertaken. RESULTS: Management could be divided into three groups. Those in group 1 (n = 5) had non-operative management, owing to cardiac fusion or gross anatomical union, and all died. Those in group 2 (n = 7) underwent emergency separation, when one twin was dead or threatening the survival of the sibling or where there was a correctable life-threatening associated anomaly (four of 14 survived). Twins in group 3 (n = 5) underwent elective separation, allowing time for detailed preoperative investigation and planning (eight of ten survived). CONCLUSION: Accurate preoperative investigation, a team approach, previous experience, and meticulous operative and postoperative management contributed to the success rate achieved in this series.

T cryptantigen activation is associated with advanced necrotizing enterocolitis.

BACKGROUND/PURPOSE: Thomsen-Friedenreich cryptantigen activation (TCA) exposes neonates with necrotizing enterocolitis NEC to the risk of hemolysis after transfusion of blood products. The authors aimed to determine the prevalence of TCA in neonates with NEC and to correlate TCA with severity of disease and outcome. METHODS: One hundred four neonates with NEC were tested for TCA on admission. Patients with TCA requiring transfusion were given packed red cells, low-titer anti-T fresh frozen plasma, and washed platelets to avoid hemolysis. RESULTS: Twenty-three infants had TCA, and 96% of these had stage III disease. The incidence of TCA was significantly higher in infants with stage III disease compared with those with stage II (30% v 4%; P <.01). A total of 91% of infants with TCA required laparotomy compared with 81% of those with no activation. At laparotomy, widespread disease was more common in the TCA group (71% v 55%). TCA did not significantly increase mortality rate (TCA, 39% v no TCA, 28%); this may reflect the transfusion policy of our unit. CONCLUSIONS: Twenty-two percent of neonates with NEC referred to our unit had TCA. There is an association between TCA and advanced NEC. Screening of neonates with advanced NEC for TCA is advised to identify those at risk of hematologic complications.

Oesophageal atresia: a simplified approach to early management.

Management following the repair of oesophageal atresia (OA) with tracheooesophageal fistula (TOF) in the past included the routine use of an intercostal chest drain, a gastrostomy, or a transanastomotic tube (TAT) for enteral nutrition and a routine contrast swallow (CS) before oral feeds. There has been a trend towards simplification of the management, but this is not universal. The aim of this study was to evaluate the safety of a simplified management regime in infants undergoing primary repair of OA in a retrospective case note review of infants undergoing surgery for OA with TOF under the care of one consultant over a 12-year period. Intercostal chest drains, TATs, and CSs were not routinely used. Early enteral feeding was initiated and oral feeding was allowed in babies of adequate birth weight (BW) and gestation. A CS was only performed when there were specific anastomotic concerns. Parameters recorded included demographic details, time to first enteral feed by tube or mouth, time to full oral feeds, and complications. Forty patients were studied; 17 were managed without (group 1) and 23 with (group 2) a TAT. Sex distribution, gestational age, and BW were comparable in the two groups. In group 1, the time to the establishment of full oral feeds was 2-8 days (average 3.9). Four infants developed strictures; 2 were managed with dilatation alone and 2 required surgery. In group 2, the time to the establishment of full enteral feeds was 2-12 days (average 5.9). Four patients developed strictures; 2 underwent an anti-reflux procedure and a 3rd resection of a cartilaginous remnant. There was 1 death in a patient with intractable cardiac failure. The majority of infants with OA and TOF can thus be safely managed without routine chest drainage or CS. A sizeable minority do not require a TAT. Early introduction of oral feeds in the non-TAT group is not associated with an increased complication rate.