Left-sided gastroschisis with caecal agenesis: A rare case report

Gastroschisis is a congenital anomaly characterised by a defect in the anterior abdominal wall through which the intestinal contents freely protrude. Defect is located almost always to right of umbilicus. To our knowledge very few cases of left-sided gastroschisis have occurred and presented in literature. We report case of left-sided gastroschisis with caecal agenesis, short gut, and malrotation of intestine.     

Left-sided gastroschisis and pseudoexstrophy: a rare combination of anomalies

Pseudoexstrophy is a rare variant of the exstrophy/epispadias complex, comprised of the musculoskeletal defects of classic exstrophy but with an intact bladder and urethra. We present a case of a neonate with left-sided gastroschisis and pseudoexstrophy, the first reported combination of these two anomalies. The anomalies in this case were unusual but highlight the importance of careful clinical evaluation before and during surgery so as to carefully define the anatomy of the abnormalities, which will assist in planning surgery and further postoperative management. Identifying and reporting these unusual cases is essential to further expand our understanding of these conditions and their potential associations.     

Two cases of left-sided gastroschisis: review of the literature

In gastroschisis, the opening is almost always to the right of the umbilicus, although eight cases of left-sided gastroschisis have been reported in the literature. We encountered two additional cases of left-sided gastroschisis. One was treated successfully, but the another child died. We report the present two these two cases and a review of the literature.     

Cisapride and Caesarean section: Their role in babies with gastroschisis

OBJECTIVE: The objective of this study was to compare the neonatal postoperative course and morbidity for patients with gastroschisis who received cisapride with those who did not receive cisapride. STUDY DESIGN: Data were obtained by review of the medical records of all the patients with gastroschisis who were admitted to Sydney Children's Hospital between January 1984 and December 1995. Data were compared between 15 babies who received cisapride with 27 who did not. The mode of delivery and outcome of babies in whom gastroschisis was diagnosed antenatally was compared with those who were diagnosed at birth. RESULTS: Duration to the commencement of feeds, attainment of full feeds and the length of hospital stay were not statistically different between these two groups, with or without cisapride (p = > or = 0.1). There were more elective Caesarean sections in the antenatally diagnosed group compared to those detected at birth and the outcome of these two groups showed no statistically significant difference. CONCLUSIONS: Our study identified no benefit from cisapride therapy in babies with gastroschisis and also there was no benefit from elective Caesarean section for babies with antenatal diagnosis of gastroschisis.     

Rare case of fetal gastroschisis with aplasia of the foot and external genital organs

Gastroschisis is a rare anomaly and is usually not associated with any other congenital anomalies. The embryology of gastroschisis and omphalocele remains a matter of speculation. Incidences of gastroschisis are particularly high among pregnancies in very young women. The present case is reported because of its rare association with the condition of gastroschisis, disrupted omphalocele, with aplasia of the foot and external genital organs, as well as imperforate anus with distal rectal atresia.     

Neonatal outcome of gastroschisis and exomphalos: a 10-year review

OBJECTIVE: To study neonatal outcomes associated with gastroschisis and exomphalos in a regional neonatal unit. METHODS: A retrospective (1988-97) data analysis to study the effect of the type of defect/surgery, mode/place of delivery and associated anomalies on time to start and reach full feeds, duration of total parental nutrition (TPN) support and total hospital stay. Exact bivariate test procedures were used for data analysis. RESULTS: Twenty-one cases of gastroschisis (17 inborn) and five cases (four inborn) of exomphalos were identified. Of these, 23.8% cases of gastroschisis and 60% of cases of exomphalos had associated gut anomalies. The survival rates for gastroschisis and exomphalos were 91 and 100%, respectively. The median time to start and reach full enteral feeds in outborn neonates was longer than in inborn neonates (9 vs 25 days, respectively, P = 0.01; and 16 vs 49 days, respectively, P = 0.01), as was the duration of TPN support (14 vs 42 days, respectively; P = 0.02). Neonates with gastroschisis had significant delays in starting and reaching full feeds compared with neonates with exomphalos (median 13 vs 4.5 days, respectively, P = 0.03; and 24 vs 8, respectively, P = 0.02) and they required prolonged support with TPN (median 23 vs 6 days, respectively; P= 0.01). Antenatal detection was significantly more frequent in inborn compared with outborn neonates (100 vs 67%, respectively; P = 0.03). The severity of associated gut anomalies and the delivery to surgery interval did not differ significantly to explain the increased morbidity in outborn neonates. Outcome was not significantly different after analysis by type of surgery and mode of delivery. CONCLUSIONS: Increased morbidity in outborn neonates may be related to factors such as temperature, care, hydration status, care of the defect and vascular compromise of prolapsed gut during prolonged transportation.     

Perinatal demography of gastroschisis in North Queensland

Aim: To review the demography of gastroschisis in North Queensland. Methods: A retrospective chart review of live born cases of gastroschisis originating in North Queensland from 1988 to 2007. Results: Fifty‐nine cases were identified, giving an overall rate of 3.2 per 10 000 live births. In mothers <20 years old compared with 20–24 and 25–29 it was 12.1, 6.3 and 1.7. Overall, the rate was higher in Indigenous mothers (6.6 vs. 2.6, OR 2.5; 95% CI 1.4–4.5, P= 0.0018). Overall, the rate increased significantly from 0.7 per 10 000 live births in the first 5 years of the study to 4.8 in the last (trend test P= 0.0015), but it increased particularly in mothers <20 years old, from 2.40 in the first 5 years to 19.3 in the last (trend test P= 0.0177). It also rose from 0 to 3.6 in those aged 25–29 (P= 0.0337) but remained stable in other age groups. There was no difference in the outcomes of babies born to Indigenous or non‐Indigenous mothers, or in outcomes of babies delivered vaginally or by Caesarean section, or in outcomes of babies from any particular location in North Queensland. There was no significant difference in the average age of Indigenous and non‐Indigenous mothers. Conclusion: Gastroschisis is increasing in North Queensland, especially in mothers <20. Overall, the rate in Indigenous mothers is two to three times higher.     

Ward reduction of gastroschisis in a single stage without general anaesthesia may increase the risk of short-term morbidities: Results of a retrospective audit

Background:   Ward reduction of gastroschisis in a single stage without the need for general inhalational anaesthesia (ward reduction) has been reported by some authors to be effective and safe. We introduced this practice to our neonatal unit 2 years ago.
Aim:   To compare the short-term outcomes of this new practice with the standard procedure of reduction under general anaesthesia (GA).
Methods:   Retrospective case series of all infants with gastroschisis between January 2004 and January 2008.
Results:   Twenty-seven infants were managed with the traditional approach and 11 infants underwent ward reduction without GA. Infants in the ward reduction group had an increased frequency for all the three major adverse events (ischemic necrosis of bowel: 27.3% vs. 3.7%, odds ratio (OR) 10.72, 95% confidence interval (CI): 0.72, 159.6; need for total parenteral nutrition (TPN) more than 60 days: 18% vs. 3.7%, OR 4.13, 95% CI: 0.28, 61.55; and unplanned return to theatre: 27.3% vs. 7.4%, OR 3.88, 95% CI: 0.44, 34.08), although none of these events reached statistical significance. There were no significant differences between the groups for the outcomes of time to reach full feeds, duration of hospital stay and number of days on antibiotics.
Conclusions:   These results raise concerns over the role of ward reduction of gastroschisis in a single sitting without the use of GA. Randomised trials with appropriate design and sample size are needed before embracing this method as a standard practice.     

Epidemiology,management and outcome of gastroschisis in Sub-Saharan Africa: Results of an international survey

Background:

The aim was to compare gastroschisis (GS) epidemiology, management and outcome in low-income countries (LIC) in Sub-Saharan Africa (SSA) with middle- (MIC) and high-income countries (HIC).

Materials and Methods:

A 10-question survey was administered at the 2012 Pan-African Paediatric Surgery Association Congress. Results are presented as median (range); differences were analysed using contingency tests.

Results:

A total of 82 delegates (28 countries [66 institutions]) were divided into LIC (n = 11), MIC (n = 6) and HIC (n = 11). In LIC, there were fewer surgeons and more patients. LIC reported 22 cases (1-184) GS/institution/year, compared to 12 cases (3-23)/institution/year in MICs and 15 cases (1-100)/institution/year in HICs. Antenatal screening was less readily available in LIC. Access to parenteral nutrition and neonatal intensive care in LIC was 36% and 19%, compared to 100% in HIC. Primary closure rates were similar in LIC and HIC at 58% and 54%, respectively; however, the majority of staged closure utilised custom silos in LIC and preformed silos in HIC. In LIC, mortality was reported as >75% by 61% delegates and 50-75% by 33%, compared to <25% by 100% of HIC delegates (P < 0.0001).

Conclusions:

Gastroschisis is a problem encountered by surgeons in SSA. Mortality is high and resources in many centres inadequate. We propose the implementation of a combined epidemiological research, service delivery training and resource provision programme to help improve our understanding of GS in SSA whilst attempting to improve outcome.     

Surgical management of gastroschisis in North Queensland from 1988 to 2007

Aim:   To review outcomes of gastroschises originating in North Queensland and repaired in the neonatal centre in Townsville from 1988–2007, and compare these outcomes with published data from other centres.
Methods:   A retrospective chart review of outcomes after primary operative repair (POR) with wound closure in fascial layers in the theatre, primary non-operative repair (PNOR) with apposition of the umbilical cord and adhesive dressing in the ward, secondary repair (SR) after a silo, and complex repair (CR) of cases with obstruction, perforation or atresiae. Epidemiological data of babies originating in North Queensland but managed in Brisbane were secured from Mater Mothers' Hospital and the Royal Hospital for Children.
Results:   Fifty cases were treated in Townsville: 16 by PNOR, 22 by POR, 6 by SR and 6 by CR. Outcomes of uncomplicated cases treated by PNOR, POR and SR were similar. Outcomes of complicated cases after CR were significantly worse. No significant differences were found between PNOR in Townsvillle and published outcomes after SR from four overseas units. No differences were detected in outcomes of inborn and outborn babies, those delivered by vaginal or caesarean birth or between indigenous and non-indigenous babies. Three (6%) died. Gastroschisis is increasing in North Queensland, particularly in younger mothers. The incidence is 2–3 times higher in indigenous mothers.
Conclusion:   No significant differences were found between PNOR and other techniques for uncomplicated cases. PNOR avoids the use of theatre and staff, reducing delay and cost, and the need to transport a sick baby.     

Structural birth defects associated with omphalocele and gastroschisis, Hawaii, 1986–2001

ABSTRACT   There is limited information on the specific structural birth defects associated with the abdominal wall defects (AWD) omphalocele and gastroschisis, particularly which defects occur with the AWD at greater than expected rates (rates among all infants and fetuses with birth defects other than the AWD). Using data from a population-based birth defects registry in Hawaii, this study calculated the rates for 48 specific structural birth defects among the AWD and compared these rates to the expected rates. There were 60 cases of omphalocele, 96 cases of gastroschisis, and 12 161 infants and fetuses with structural birth defects excluding the AWD among deliveries during 1986–2001. For omphalocele, higher than expected rates were found for 23 (47.9%) of the defects. These involved defects of a variety of organ systems. For gastroschisis, higher than expected rates were found for 8 (16.7%) of the defects, mainly neural tube defects (NTD) and specific defects of the orofacial and gastrointestinal system and the genital and urinary system. Both omphalocele and gastroschisis had elevated rates for NTD, intestinal atresia/stenosis, malrotation of intestines, obstructive genitourinary defects and limb reduction deformities. Certain specific structural birth defects occurred more often than expected with the AWD. The associated birth defects tended to vary between omphalocele and gastroschisis, although there were a few similarities. Due to the small number of cases, further research involving larger amounts of data are warranted.     

Intestinal atresia in association with gastroschisis: a 26-year review

Purpose  

We reviewed our experience with gastroschisis (GS) complicated by intestinal atresia over the last 26 years. Our aim was to determine the effect of different management strategies employed and the morbidity associated with this condition in our unit.     

Pre-term and particularly pre-labor cesarean section to avoid complications of gastroschisis

The marked advantages and merit of pre-term and particularly pre-labor (PTPL) cesarean section (C-section) in the avoidance, and indeed, virtual elimination of severely disabling gastroschisis (GS) complications in infants diagnosed prior to birth by ultrasound has unfortunately remained controversial in the 10 to 12 years since it was first reported and strongly recommended by numerous authors. During this period, GS has remained one of the four major causes of the short-gut syndrome (SGS) in infancy and childhood and a major cause of prolonged, costly, complicated, and hazardous neonatal intensive care unit stays with requirements for total parenteral nutrition (TPN). The most serious and frequent complications of GS in infants born without PTPL C-section are the occurrence of the “peel”, which greatly enlarges and rigidifies the eviscerated gut, and of “complicated GS” (intestinal atresia/s, stenosis, necrosis, perforations) (CGS). The “peel” occurs in 100% of these cases and CGS in approximately 20%. “Peel” enlargement and rigidification of eviscerated intestine in the presence of a reduced peritoneal cavity causes great difficulty in covering the eviscerated, enlarged, and rigidified gut with abdominal wall, skin, a prosthesis, etc., and frequently produces gut ischemia from excessive pressure, which may lead to necrotizing enterocolitis (NEC) and SGS as well as prolonged hospital stays. The presence of a “peel” greatly complicates the hazards of dealing with cases of CGS, as resection and anastomosis are virtually impossible in the presence of a “peel.” The authors report personal experience with 77 cases of GS dating as far back as 1951; 44 of the infants were born after the onset of labor by vaginal or C-section delivery and all had some degree of “peel” formation. Of 320 cases from the literature (including some of the cases reported here), 61 (19.1%) involved CGS. Of the 33 cases born PT, and especially PL, there were no cases of “peel” and only 1 case of CGS (3.0%). This infant had a single atresia associated with a very small (1 cm) defect in the abdominal wall and no labor-induced “peel,” which was easily and successfully repaired by resection and anastomosis. The 6.4-fold reduction in the occurrence of CGS by PTPL C-section (3.0% vs 19.1%) was statistically significant by the chi-square test (P < 0.05), as was the 100% elimination of the disabling “peel.” If the single case of CGS associated with a very small defect and no labor or labor-associated “peel” is eliminated, the incidence of CGS in the remaining PTPL group of 32 cases falls to 0 (0% versus 19.1%, P < 0.007). PT and especially PL C-section may be expected to virtually eliminate “peel” formation and CGS and to remove GS as one of the four major causes of SGS. The findings of this report that PT labor prior to PT C-section may result in both “peel” formation and CGS further solidifies the role of labor in the production of both the “peel” and the equally disabling CGS. Failure to appreciate the central role of labor in GS complications has doubtless contributed to the persistent controversy concerning the value and importance of PTPL C-section for gastroschisis diagnosed in utero. The pediatric surgeon has an important responsibility with the obstetrician to monitor the possible occurrence of occult labor in the waning weeks of pregnancy and be prepared to do a prompt C-section if it occurs and there is adequate lung maturity. The achievement of “peel”- and CGS-free gut would greatly facilitate the use of the new Bianchi technique of gut reduction without anesthesia. The combination of the use of epidural anesthesia for the elective PTPL C-section with the Bianchi approach would spare both mother and baby any untoward effects of general anesthesia and present the potential for massive reductions in hospital costs with minimal patient manipulation and disturbance. For infants born with labor-associated “peel,” re-evaluation of the suitability and effectiveness of surgical “peel” decortication from involved gut is strongly urged. Accepted: 13 July 1998     

Pancreatitis with panniculitis and arthritis: a rare association

Abstract A case of pancreatitis with polyarthritis and panniculitis is reported because of its rarity and the severe nature of the disease.     

Fraser-Cryptophthalmos syndrome with cardiovascular malformations: a rare association

Fraser-Cryptophthalmos syndrome is a multiple malformation disorder associated variably with cryptophthalmos (hidden eye), anomalies of the head, nose and ears; syndactyly, renal and genital malformations. In this report, we describe a case of Fraser syndrome with cardiovascular malformations: coarctation of aorta, an association not previously described.