Subcostal Margin Research Articles

A phase 3, randomized, double-blind, placebo-controlled study of ruxolitinib plus parsaclisib in patients with JAK- and PI3K-inhibitor treatment-naïve myelofibrosis.

TPS7058 Background: Ruxolitinib (JAK1/JAK2 inhibitor) significantly improves outcomes in patients with myelofibrosis (MF); however, a subset of patients may experience a suboptimal response. Recent phase 2 data showed that addition of PI3Kδ inhibitor parsaclisib to ruxolitinib monotherapy resulted in additional alleviation of MF symptoms and splenomegaly in patients with MF (Yacoub. EHA2020. S216). This phase 3, randomized, double-blind study (INCB 50465-313; NCT04551066), evaluates the combination of ruxolitinib and parsaclisib in patients with MF who are naïve to Janus kinase (JAK) and PI3K inhibitor therapies. Methods: Eligible patients are aged ≥18 years with a diagnosis of primary MF, post-polycythemia vera MF, or post-essential thrombocythemia MF, have a Dynamic International Prognostic Scoring System (DIPSS; Passamonti. Blood. 2010;115:1703-1708) risk category of at least Intermediate (INT)-1, palpable spleen ≥5 cm below left subcostal margin; total symptom score ≥10 at screening, ECOG PS 0–2, and life expectancy ≥24 weeks. Patients will be excluded if they previously received therapy with any JAK inhibitor, any PI3K inhibitor, any experimental or standard drug therapy for MF ≤3 months of first study dose and/or lack of recovery from all toxicities related to previous therapies to grade ≤1, have recent history of inadequate bone marrow reserve (eg, platelet count = 50×109/L) or have inadequate liver or renal function at screening. Approximately 440 patients will be randomized (1:1) to ruxolitinib plus parsaclisib 5 mg QD or ruxolitinib plus matching placebo, with stratification at randomization by DIPSS risk category (high vs INT-2 vs INT-1) and platelet count (≥100×109/L vs 50 to = 100×109/L inclusive). Treatment will begin on Day 1, with starting ruxolitinib dose level determined by baseline platelet count, and will continue as long as treatment is tolerated and discontinuation criteria are not met. When the last enrolled patient has completed 24 weeks of treatment, the study will be unblinded and patients randomized to ruxolitinib plus placebo who have adequate hematology parameters will be able to crossover to receive parsaclisib together with continued ruxolitinib. The primary objective is the evaluation and comparison of spleen volume at Week 24 for patients who received ruxolitinib plus parsaclisib versus ruxolitinib plus placebo. Secondary objectives include evaluation and comparison of patient-reported MF symptoms, overall survival, time to onset and duration of response in spleen volume, and safety and tolerability for ruxolitinib plus parsaclisib versus ruxolitinib plus placebo. Sites are opening across the United States, Europe, Asia, and New Zealand. Clinical trial information: NCT04551066.

Differences of the midline-crossing venous drainage pattern in supraumbilical and infraumbilical regions: Angiographic study using fresh cadavers.

Current clinical and anatomical studies show that the venous problem associated with the deep inferior epigastric perforator flap results from poor midline-crossing. We examined the venous anatomy of the infraumbilical midline area and the dynamic venous flow of the deep inferior epigastric perforator flap in nine fresh cadavers. All nine abdominal specimens were harvested between the subcostal margin and the groin crease. Two specimens were used to analyze the abdominal venous anatomy, one of which was divided into two hemi-abdominal specimens. The remaining seven specimens were harvested as deep inferior epigastric perforator flaps with one major paraumbilical perforator. Venous cannulation and serial angiographic agent injection were performed in several conditions. Each specimen was radiographed using a soft X-ray system. For additional information, computed tomography (CT) angiography-visualized superficial inferior epigastric veins (SIEVs) and the supraumbilical branch were analyzed. We noted that the venous drainage between the bilateral SIEVs was easier to configure in the supraumbilical area than in the infraumbilical area. Only one to two short polygonal venous networks connect the bilateral superficial inferior epigastric veins in the supraumbilical area; however, long and multiple polygonal venous networks connect the bilateral superficial inferior epigastric veins in the infraumbilical area, which could be a predisposing factor for venous congestion. The mean distance from the umbilicus upper border to evident supraumbilical midline crossover was 18.39±4.03 mm (range: 10.10–28.49) in CT angiograms. In cadaver specimens, the mean distance was 10.87±4.85 mm (range: 4.6–18.9). Supraumbilical midline crossover was more favorable than infraumbilical midline crossover in venous flow.

Splenomegaly in Children with Sickle Cell Anemia Receiving Hydroxyurea in Sub-Saharan Africa

Introduction. Children with sickle cell anemia enrolled in Realizing Effectiveness Across Continents with Hydroxyurea (REACH, NCT01966731) received open-label hydroxyurea at maximum tolerated dose (MTD) in four countries within sub-Saharan Africa (Tshilolo et al, NEJM 2019;380:121-131). Unlike children in the United States or Europe, a substantial proportion of REACH participants had splenomegaly at enrollment, and more developed splenomegaly while receiving hydroxyurea. Splenic enlargement in association with hydroxyurea treatment in sub-Saharan Africa is previously unrecognized, and its causes and consequences remain unclear. Methods. Palpable splenomegaly was evaluated at both the mid-clavicular and mid-axillary lines at each scheduled and unscheduled sick visit. The size of the spleen, defined as the greatest distance (cm) below the subcostal margin, was recorded in the REDCap trial database at all four clinical sites. Cross-sectional analysis was performed at baseline enrollment using four spleen categories (Not Palpable, 1-4 cm, ≥5 cm, or Splenectomy) with correlations for age, sex, site, growth parameters, alpha-thalassemia trait and G6PD deficiency. This analysis was repeated using the largest spleen size over the first two years on hydroxyurea, but examining two-year laboratory values and also the hydroxyurea dose at MTD, time to MTD, dose-limiting toxicities, and clinical outcomes including acute splenic sequestration, malaria infections, and sepsis. Results. A total of 606 children started hydroxyurea study treatment, including 6 (1.0%) with previous splenectomy, 59 (9.7%) with previous splenic sequestration, and 99 (16.3%) with palpable splenomegaly at enrollment (52 children with 1-4 cm and 47 with ≥5 cm). Large spleens (≥5 cm) were commonly observed at baseline at all clinical sites except Uganda, which identified only 1 child. Compared to those with no palpable spleen, children with large spleens at baseline had similar age and growth parameters, but were significantly more likely to have alpha-thalassemia (78.7% versus 56.2%, P=0.004) and also G6PD deficiency among males (28.0% versus 17.6%, P=0.32). Children with large spleens at enrollment also had a lower hemoglobin (Hb = 6.5 versus 7.3 g/dL, P<0.001) and lower platelet count (platelets = 227 versus 410 x 109/L, P<0.001), but equivalent fetal hemoglobin (HbF = 10.2 versus 9.4%, P=0.82). On hydroxyurea treatment with escalation to MTD, 262 children (43.7%) had palpable splenomegaly recorded, including 120 (20.0%) with spleens ≥5 cm. These large spleens were observed at all four clinical sites, with DRC having the most (52) and Uganda with the least (14). After 24 months of hydroxyurea treatment, laboratory differences were noted according to the cumulative occurrence of splenomegaly including a significantly lower hemoglobin and platelet count, higher absolute reticulocyte count, and lower hydroxyurea dose at MTD (Table). Large spleens were associated with a high cumulative incidence of laboratory dose-limiting toxicities, as well as a significantly higher risk of having clinically symptomatic malaria and receiving blood transfusions (Table). A total of 31 children (5.2%) on hydroxyurea treatment received elective splenectomy, including one partial splenectomy using arterial embolization. Conclusion. Children with sickle cell anemia living in sub-Saharan Africa have an increased risk of having palpable splenomegaly, which is further increased while receiving hydroxyurea treatment. Large spleen at baseline were associated with lower blood counts, consistent with hypersplenism. On hydroxyurea treatment, children with large spleens had significantly lower blood counts and more dose-limiting toxicities, which lowered their eventual hydroxyurea dose at MTD but still led to robust HbF responses. Children with large spleens were also at higher risk of developing malaria infections, receiving transfusions, and requiring surgical splenectomy. Splenic enlargement in association with hydroxyurea treatment was common in children with sickle cell anemia in the REACH trial; its cause remains unclear but the consequences include substantial laboratory toxicity and clinical morbidity. Investigating the etiologies and management of children with chronically enlarged spleens is crucial before expanding hydroxyurea access across Africa for sickle cell anemia. Disclosures Ware: Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees; Bristol Myers Squibb: Other: Research Drug Donation; Nova Laboratories: Membership on an entity's Board of Directors or advisory committees; CSL Behring: Membership on an entity's Board of Directors or advisory committees; Novartis: Other: DSMB; Agios: Membership on an entity's Board of Directors or advisory committees; Addmedica: Other: Research Drug Donation.

2327 Not Just a Cyst: Rare Presentation of LUQ Pain

INTRODUCTION: Lymphangioma is a benign congenital malformation of the lymphatic system that is relatively common in juvenile population. Rarely, it can be seen in adults and majority of the cases are reported in the head and neck region (70-75%). Typically, these malformations are asymptomatic. In this report, we present a rare case of splenic lymphangioma presenting with severe, intractable abdominal pain. CASE DESCRIPTION/METHODS: A 50-year-old female presented with three-month history of progressive abdominal pain located below the left subcostal margin. Initial vital signs and lab work were unremarkable. Physical exam was significant for tenderness in the left upper abdominal quadrant. Computed tomography (CT) scan of abdomen with contrast revealed a hypointense lesion measuring up to 1.8 cm in the posterior aspect of the spleen. Subsequently, a hypo-enhancing splenic mass in the corresponding location was demonstrated in T1-weighted MRI images (Image 1A). The spleen was normal-sized and there were no liver lesions. A total splenectomy was performed with resolution of the pain during the hospitalization. Histopathological examination of the lesion revealed multiple cystic cavities containing pale eosinophilic fluid (Image 1B) resembling lymph, consistent with splenic lymphangioma. The cystic cavities were lined with elongated cells with bland nuclei and scant cytoplasm (Image 1C). Immunohistochemically, the lesion stained positive with CD31 (Image 1D). DISCUSSION: Clinical presentation of splenic lymphangioma varies depending on the spleen size with majority of the patients being asymptomatic. The diagnosis is usually made incidentally on routine imaging studies. Based on the clinical data and imaging results, our differential diagnosis included hemangioma, complex splenic cyst, lymphangioma, lymphoma, and pseudocysts. The splenic lymphangioma was diagnosed on microscopic examination following a splenectomy which was done to avoid any further complications such as; infection, rupture, hemorrhage and pleural effusions. The treatment modality of choice was complete surgical resection in this patient given relevant history. Other therapeutic modalities such as aspiration or drainage have not shown effective results in the literature. Despite the rarity, early recognition of these lesions is important as the delay in surgical intervention can result in complications.

SUN-LB028 Diabetic Truncal Radiculopathy: An Underrecognized Cause of Thoracic and Abdominal Pain

Introduction: Diabetic polyneuropathy involves peripheral, cranial or autonomic nerves causing well known clinical presentations, easily identifiable by clinicians. Rarely, diabetic neuropathy affects the intercostal neural roots causing severe and difficult to treat thoracic and abdominal pain. We present an interesting case where diagnosis of thoracic radiculopathy was made 8 years later. Case Presentation: A 62-year-old man with type 1 diabetes mellitus presented with a history of severe right lower thoracic and flank pain. He was diagnosed with diabetes at age 20 and developed complications such as peripheral polyneuropathy. His pain was sharp and burning in nature in a “band like” distribution in the right lateral subcostal margin. The intensity ranged from 3-10/10. Exam showed tenderness to touch in the right lateral subcostal margin with no sensory deficits. He visited different specialists within a year in attempts to obtain pain relief. Therapeutic attempts done included opiates, duloxetine, gabapentin and NSAIDs with minimal improvement. Initial workup included a normal bone scan and thoracic spine MRI which showed degenerative disk disease at the level of T7-T8. Corticosteroid injection at T7-T9 resulted in no pain relief. Then a pain specialist made the diagnosis of DTR. A T10 nerve block was given with significant relief. Since then, he has received intermittent nerve blocks with complete resolution of his symptoms. Discussion: Diabetic neuropathy is the most common complication of diabetes mellitus. Truncal roots originating from the spinal cord can be affected causing a distinct disorder termed Diabetic Truncal Radiculopathy (DTR). DTR is a rare form of diabetic neuropathy that is significantly underdiagnosed. DTR is of three types - Cervical, thoracic and lumbosacral. It is commonly unilateral following the distribution of an intercostal nerve in a band like pattern. The pain is described as burning or aching in nature, frequently intensified at night associated with sensitivity to touch. The patients are usually middle aged or older adults with long standing history of diabetes. There is certain preference for men with DM type 2. It is a diagnosis of exclusion. EMG typically demonstrates denervation potentials in the intercostal, anterior abdominal wall and paraspinal muscles. The differential diagnosis is primarily based on the localization of neural injury. Involvement of upper thoracic nerves causes chest pain whereas lower thoracic nerves causes upper abdominal pain. The management is similar to other diabetic related neuropathies but challenging. Intensive glycemic control is ineffective in pain relief. DTR should be considered as a possible cause of atypical flank or chest wall pain not explained by other causes. Simple interventions such as intercostal nerve blocks are successful in improving the pain, quality of life and functionality of patients. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. s presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.

Single injection ultrasound-guided rectus sheath blocks for children: Distribution of injected anesthetic.

Single injection ultrasound-guided rectus sheath blocks are used for postoperative analgesia after midline abdominal incisions, but the ultrasonographic spread of medication posterior to the rectus muscle has not been investigated. The primary goal of this study was to determine the ultrasound-measured medication spread superiorly and inferiorly after single injection ultrasound-guided rectus sheath blocks and to calculate the incidence of cases with spread up to the subcostal margin and below of umbilicus. The secondary goals were to correlate the spread with the volume of medication administered and with the patient's age, weight, height, sex, and body mass index. Pediatric patients who underwent single injection ultrasound-guided rectus sheath blocks and underwent data collection of ultrasonographic information on medication spread along the plane of the posterior rectus sheath were identified retrospectively from an acute pain service database at UPMC Children's Hospital of Pittsburgh. Sixty-eight children, 10.2±4.2 (mean±SD) years old, 41.7±17.9kg, 140±24.6cm, had complete data collection. Mean injected volume of ropivacaine was 8.3±2.8mL (right) and 8.2±2.8mL (left). The average spread was measured as 3.9±1.4cm (right) and 3.4±1.3cm (left) cranial to the umbilicus, and 1.5±1.6cm (right) and 1.6±1.4cm (left) caudal to the umbilicus. Complete spread up to the level of the subcostal margin was observed in 52.9% (n=36; right) and 36.8% (n=25; left) of cases. The spread correlated closely with the volume of injected ropivacaine (right: 0.556, P<0.001; left: 0.541, P<0.001). The spread below umbilicus was 70.6% (n=48, right) and 80.9% (n=55, left). There was a positive strong correlation (P<0.001) between total medication spread and age (right: 0.608; left: 0.538), weight (right: 0.600; left: 0.540), and height (right: 0.593; left: 0.526). After single injection ultrasound-guided rectus sheath blocks, incomplete cephalocaudal medication spread can be expected within the posterior rectus sheath.

Cecal intubation time during colonoscopy: Do waist circumference and waist-to-hip ratio play better roles than body mass index?

Backgroundand Aim: Among the factors that influence cecal intubation time (CIT) are gender, the quality of bowel preparation, age, prior abdominal surgery, constipation, the experience of the endoscopist, body mass index (BMI), the amount of visceral fat, and waist circumference (WC). This study aimed to compare the effects of WC and waist-to-hip ratio (WHR) to that of BMI on CIT. Patients and Methods: One hundred patients had colonoscopy during the study period. The height and weight of each patient were taken using a stadiometer. BMI was calculated by dividing the weight in kilogram by the square of the height in meter. WC (cm) was measured at a point midway between the subcostal margin and the upper border of the iliac crest. Hip circumference (HC in cm) was measured at the greatest point between the iliac crest and the thighs. WHR was calculated by dividing the WC by the HC. Results: There were 60 (60%) males and 40 (40%) females. Mean age was 60.0 ± 13.1 years (range 27.0–87.0 years). Mean CIT was 1009.5 ± 410.7 s (range 329.0–2110.0 s). Mean BMI, WC, HC, and WHR were 25.5 ± 5.6, 92.2 ± 13.7 cm, 97.5 ± 11.7 cm, and 0.9 ± 0.1, respectively. Age ≥60 years and BMI ≤24.9 kg/m2 were significantly associated with a prolonged CIT in males. In both genders, WC and WHR were not significantly associated with CIT. Conclusion: Higher BMI, WC, and WHR were associated with shorter CIT. However, among these parameters, only BMI was found to be significantly associated with CIT in males.

Clinical features and VPS33B mutations in a family affected by arthrogryposis, renal dysfunction, and cholestasis syndrome

Arthrogryposis, renal dysfunction, and cholestasis (ARC) syndrome is an autosomal recessive disorder caused by mutations in the VPS33B or VIPAS39 gene. The aim of this study was to investigate the clinical features and VPS33B gene mutations of an infant with ARC syndrome. A 47-day-old female infant was referred to the hospital with the complaint of jaundiced skin and sclera for 45 days and abnormal liver function for 39 days. The patient had been managed in different hospitals, but the therapeutic effects were unsatisfactory due to undetermined diagnosis. Physical examination showed jaundice of the skin and sclera. Systemic skin was dry with desquamation in the limbs and trunk. There were no positive signs on cardiopulmonary examination. The liver was palpable 2.0 cm under the right subcostal margin. The hips and knees were flexed, and the extension was limited, with low muscular tone in the four limbs. Biochemical analysis demonstrated raised serum total bile acids, bilirubin (predominantly conjugated bilirubin) and transaminases, but the γ-glutamyl transpeptidase level was normal. Routine urine test revealed increased glucose as well as red and white blood cells. On genetic analysis, the infant was proved to be homologous for a VPS33B mutation c.1594C>T(p.R532X). She was definitely diagnosed to have ARC syndrome. Symptomatic and supportive therapy was given, but no improvement was observed, and the infant finally died at 3 months and 29 days of life.

Clinico-pathologic features of chronic myeloid leukemia and risk stratification according to Sokal score.

To compile the clinical and haematological parameters of chronic myeloid leukemia (CML) and risk stratification according to Sokal score in our population. A descriptive analysis. The Aga Khan University Hospital, during the period from August 1997 to August 2005. All patients with diagnosis of chronic myeloid leukemia treated as outpatient in haematology clinic, or admitted in haem-oncology wards in The Aga Khan University Hospital were included. Records were retrospectively analyzed for clinicopathologic features. Risk groups were assigned as per Sokal scoring. During the 8 years study period, 245 patients with chronic myeloid leukemia were analyzed, the median age of presentation was 35 years (range 11-73); with male to female ratio 1.69:1. At the time of diagnosis, 178 patients (72.6%), 48 (19.7%) and 17 (7.8%) of patients were in chronic, accelerated and blast phase of the disease respectively. Abdominal fullness was the frequent clinical presentation followed by fever. Laboratory parameters revealed mean hemoglobin 10.0 g/dl (range 4.6-15), mean total leukocyte count 168 x 10(9)/L (35-959) and mean platelets 408 x 10(9)/L (range 30-2335). The mean size of spleen at the time of presentation was 9.2 cm below the left subcostal margin. A large number of patients (46.2%) were in high risk group according to Sokal score i.e. > 1.2. CML occurred more commonly at younger age in this series. Most of these patients were in high risk group according to Sokal score.

Clinical feature and genetic analysis of a family affected by congenital bile acid synthesis defect type 2: identification of 2 novel mutations in AKR1D1 gene

Congenital bile acid synthesis defect type 2 (CBAS2) is an autosomal recessive disorder caused by biallelic mutations of AKR1D1 gene, which encodes the Δ4-3-oxo-steroid 5β-reductase. Cholestatic jaundice is the main clinical manifestation, accompanied by malabsorption of fat and fat-soluble vitamins. This paper reported the clinical and genetic features of a CBAS2 patient definitely diagnosed by AKR1D1 genetic analysis. An 8-month-old male infant was referred to the hospital with the complaint of jaundiced skin and sclera over 7 months. On physical examination, growth retardation and malnutrition were discovered besides mild jaundice of the skin and sclera. The liver was palpable 8 cm below the right subcostal margin with medium texture, and the spleen was not enlarged. On liver function test, elevated levels of bilirubin (predominantly conjugated bilirubin) and transaminases were detected, but serum total bile acids and γ-glutamyl transpeptidase levels were within the normal ranges. Liver histopathologic analysis showed disorganized bile ducts, obvious multinucleated giant cells, significant cholestasis in hepatocytes, together with portal and interstitial fibrosis and lymphocytic infiltration. Via next generation sequencing analysis and Sanger sequencing confirmation, the infant proved to be a compound heterozygote of the AKR1D1 variants c.579+2delT and c.853C>T(p.Q285X), two novel mutations originated from his mother and father, respectively. CBAS2 was thus definitely diagnosed, and chenodeoxycholic acid was given orally. As a result, the abnormal liver function and hepatomegaly were improved gradually. On a follow-up 3 months later, a soft liver was palpable 2.5 cm below the right subcostal margin, and all liver function indices recovered to normal ranges.

Anterior Quadratus Lumborum Block Versus Transversus Abdominis Plane Block with Liposomal Bupivacaine: A Case Report

A more posterior approach for abdominal wall blocks was described by Dr. Blanco as Quadratus Lumborum (QL) block. We performed the first reported transmuscular QL block using liposomal bupivacaine. We are describing the technique we used as well as other approaches used for QL block. This case report showed that QL block using a single dose of Liposomal Bupivacaine provided a sensory level and analgesia for 2 days. Our limited experience shows that this new type of block can prolong the analgesia along mid and lower thoracic dermatomes. to quadratus lumborum (QL) muscle has been sought for to potentially provide the patients with more visceral blockade and thus better analgesia [2,3]. Moreover, the effective period of this block can be prolonged by using liposomal bupivacaine [4]. This is the first report demonstrating injection of liposomal bupivacaine for a QL block. Description of the Case Institutional Review board of Cleveland Clinic waived written consent since there was no patient identifying information. The patient was a 32-year-old male with body mass index of 21.07 kg/m2 who was scheduled for a subtotal colectomy in the setting of ulcerative colitis. The surgery was scheduled to be performed through a midline incision extending from above the umbilicus to pubic symphysis. Preoperatively, our acute pain management service was consulted. The patient was initially offered an epidural catheter, which he refused due to previous ineffective analgesia. Thereafter, TAP and QL blocks were offered to the patient and the risks and benefits were explained. It was also explained to the patient that QL blocks and Liposomal bupivacaine use are not investigated thoroughly yet. The patient expressed understanding and agreed to proceed with the proposed set of blocks. The patient subsequently received a left QL (trans-muscular approach, type 3) and a right TAP single shot block for postoperative pain control to allow the right side to serve as a “control”. The blocks were performed in the induction room, with routine monitoring and midazolam (2 mg IV) sedation. The patient was initially placed in right lateral decubitus position. After sterile prepping and draping, a low frequency (2-5 MHz) curved array ultrasound transducer (Mindray, Nanshan, Shenzhen, China) covered in sterile plastic sheath was placed in the anterior axillary line between the subcostal margin and the

Cadaveric feasibility study on the use of ultrasound contrast to assess spread of injectate in the serratus anterior muscle plane.

Background:The Serratus anterior muscle plane (SAP) block has recently been described for the purpose of perioperative pain management following cases of trauma and breast surgery. It might prove a safer alternative to the other regional thoracic paravertebral and central neuraxial blockade techniques. There are no descriptive cadaveric studies in the pre-existing literature to delineate the anatomical plane for this novel technique. The main objectives for our study were to examine the location of the Serratus anterior muscle belly, assess the efficacy of achieving adequate delineation of the muscle plane utilising ultrasound imaging with agitated water as the contrast agent, and finally, to observe the extent of the cepahlo-caudal spread of the injectate in the SAP.Materials and Methods:Seven cadavers were studied. 20 mls of saline was injected into posterior axillary line (PAL) at the level of the 4-5th rib under ultrasound guidance. This was followed by injection of 10 mls of water with air (8 mls water and 2 mls of air). The presence of hyperechoic air bubbles in the fluid distended SAP (hypoechoic) area demonstrated the spread of water and air.Results:In 36% of cadavers, fully formed Serratus Anterior muscle belly was identified at the midaxillary line (MAL), 14% in PAL, and remaining 50% between PAL and MAL. The lower most limit of air-water spread was identified at the subcostal margin. Cephalad spread of contrast was noted in 2nd intercostal space ICS (7%), 3rd ICS (71%), and 4th ICS (22%).Conclusion:This study describes that the serratus anterior muscle is well-formed near the PAL and the injectate spread can be determined with the help of agitated water contrast on ultrasound. Furthermore, there was variability in the cephalad spread of the injectate.

Riedel Fight Song: a case report of riedel lobe presumed to be hepatomegaly

Riedel lobe is a liver variation occasionally confused with an abdominal mass or hepatomegaly. The clinical infrequency of this condition and its resemblance to more serious conditions can initiate unnecessary workup or surgery if not considered early. An otherwise healthy, 14-year-old girl presented to the gastroenterology (GI) clinic for follow-up to an emergency room (ER) visit with a 3-day history of right upper quadrant (RUQ) pain. She reported during the first 24 hours a sharp, constant, non-radiating pain increased from 2 to 6/10, improved with ice and ibuprofen, and aggravated with activity and laying on her right side. Physical exam was significant for tenderness in the RUQ and liver edge palpated 2 finger breadths below the subcostal margin anteriorly and laterally to the iliac crest. Pain was reproduced by rotation of her trunk. Laboratory findings were unremarkable. Before further testing, the pediatric radiologist consulted identified the hepatomegaly as a “Riedel lobe, elongated and functional.” Further history elicited a social wrestling match the night prior to onset; repeat physical exam suggested a strain in the right upper rectus abdominis muscle. Relative rest, ice and ibuprofen for pain were prescribed and patient education was conducted. First reported in 1888 when seven cases were confirmed by surgical exploration, the incidence of Riedel lobe is now known to occur in approximately 25% of 20-45 year-olds and 60% of 45-65 year-olds, with female: male ratio of 3:1. Potential morbidities include lobar torsion, mass effect induced obstruction, interference with laparoscopic surgical procedures and unnecessary imaging. Clues from the history and physical exam can help distinguish this variant from other liver pathology. Physician awareness, the use of Doppler ultrasonography, and pediatric radiology consult should be considered before the use of more intensely radiating imaging.

The use of four-point transversus abdominis plane block for liver resection.

Sir, Transversus abdominis plane (TAP) block is a safe and relatively easy block to perform for post-operative analgesia.[1] We describe the use of four TAP blocks and subsequent placement of two TAP catheters in a lady who underwent a combined anterior resection and liver resection for metastatic sigmoid cancer. Although epidurals have historically been a standard of practice for adjunct intra- and post-operative analgesia, TAP blocks have been introduced since 2001 by Rafi as a landmark technique and then in 2007 with ultrasound (US) guidance by Hebberd et al.[2] Our patient was a 34-year-old lady who underwent a laparotomy with sigmoid resection as well as a hepatectomy for a recently diagnosed metastatic sigmoid cancer. She had no other co-morbidities and was to receive general anaesthesia for the surgery. She declined an epidural placement, which is the standard of care for analgesia in our institution for such cases, due to a history of lower back pain. Her intra-operative course was remarkable only for persistent tachycardia with extensive resection; however, blood loss was minimal and haemodynamic instability was well controlled. Postoperatively, while still under general anaesthesia, four TAP blocks were carried out using the US for guidance. We used a Hitachi Aloka® machine with a probe frequency of 13 MHz and a 50 mm Stimuplex® needle. Two were subcostal injections and two injections, just superior to the iliac crest along the anterior abdominal wall. The probe was placed in the midline of the abdomen 2 cm below the xiphisternum and moved right, laterally along the subcostal margin to the anterior axillary line. The transversus abdominis muscle was identified lying beneath and extending lateral to the rectus abdominis muscle. The block needle was then guided, in plane, to a point just inferior to the right costal margin at the anterior axillary line such that the tip lay between the transversus abdominis and internal oblique muscle within the neurovascular fascial plane. About 10 ml of 0.5% ropivacaine were deposited in each site. Following this, two TAP catheters were placed in the inferior sites using a similar technique but with a 18-gauge Tuohy needle through which an epidural catheter was inserted. The catheters were anchored at 9 and 10 cm at the skin, respectively and secured with a Tegaderm™ dressing. An infusion was subsequently started via a filter, with 0.1% ropivacaine via the 2 catheters at 5 ml/h. This technique used for lower abdominal surgery before is scarcely described in literature for hepatic resection. The TAP block has been shown to reduce peri-operative opioid use in elective abdominal surgery.[3] Postoperatively, the lady was comfortable and relatively pain-free. She was mobilised on post-operative day one and discharged from the Intensive Care Unit the next day. The safety profile of four TAP blocks is good with a dose limit of 200 mg in total. The risk of inadvertent liver injury is also minimum using the US for guidance. The absolute contraindications to TAP blocks only include patient refusal and site infection. Her pain scores were satisfactory avoiding the risk profile related to epidural analgesia (EA). The recent prospect guidelines have suggested that there is a higher risk using EA for major laparotomies.[4] A recent article in IJA mentioned, that ‘the available evidence so far are in favour of US-guided TAP block as a simple and effective analgesic technique, especially for lower abdominal surgeries’.[5] Further trials are needed in a procedure specific manner to compare TAP blocks with EA and other analgesic regimes. In summary, the present study provides further evidence that TAP block can produce effective analgesia for upper abdominal and hepatic surgery. Given the added complications of EA, TAP blocks used more abundantly for liver resection may be the way forward for providing reasonable and satisfactory analgesic techniques.[6,7,8] Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

An Unusual Cause of Anterior Abdominal Wall Abscess

Question: A 71-year-old woman, who had no previous psychiatric history or abdominal surgery, was admitted to the hospital for left hypochondrial mass for 2 weeks, associated with an ache over the area and loss of appetite for the same duration. No fever was experienced. She was also noted to move her bowels every 2–3 days (previously daily) for the past 2 weeks. Physical examination revealed a 7-cm, immobile, firm mass about 6 cm below the left subcostal margin with normal overlying skin. Digital rectal examination was unremarkable. Initial abnormal laboratory tests were as follows: hemoglobin, 11.0g/dL; white blood cell count, 11.6 10/L; and sodium, 127 mmol/L. Supine and erect abdominal radiographs showed gaseous distension of the large bowel (Figure A). CT of the abdomen and pelvis was done (Figure B, C). What is your diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.

A Neonate with Acute Heart Failure: Chromosomally Integrated Human Herpesvirus 6–Associated Dilated Cardiomyopathy

A 4-day-old neonate was transferred to a tertiary heart center for further evaluation and management of sudden-onset acute heart failure (HF). The birth history was significant for being born at 37 weeks’ gestation to a 26-year-old mother with a probability 2 A0 blood type. There were no prenatal sonographic screenings of fetal cardiac status available. The mother had no history of smoking, diabetes, thyroid disease, autoimmune disorders, fever, or rash during pregnancy or before delivery. At birth, the patient’s Apgar scores were 8 and 8 at 1 and 5 minutes, respectively, weight was 3200 g (25th percentile), length 48 cm (10th percentile), and head circumference 36 cm (80th percentile). He developed respiratory distress on day 1 of life and was treated empirically to rule out sepsis. There was no history of fever, skin rash, nasal congestion, diarrhea, or sick contact noted. His respiratory status improved by day 2 of life, and he began oral feeding and otherwise progressed well until day 4 of life, when he was scheduled for discharge home but was noted to have a gallop rhythm. His echocardiogram showed a structurally normal heart but markedly dilated left ventricle (LV end-diastolic diameter was 3.24 cm (Z-score 6.7) with moderate mitral regurgitation and severely decreased systolic function, shortening fraction (SF) 11% (mean normal SF value is 36% with 95% predicted limits of 28% to 44%; Figure 1). When the patient arrived in cardiac intensive care unit, no dysmorphic features were noted on examination, his heart rate was 174 beats/min, he had delayed capillary refill (>5 seconds), diminished oxygenation (by near infrared spectroscopy), and had hypotension (systolic blood pressure in the range of 40-50 mm Hg). His S1 and S2 were normal, but a grade 1/6 systolic murmur at the left sternal border and gallop rhythm were noted. His lungs were clear to auscultation, but mild subcostal retractions were noted. The liver was palpable 3 cm below the right subcostal margin. The findings of his neurologic examination were normal, with normal muscle tone noted. He required resuscitation with inotropic support to achieve hemodynamic stability. Subsequently, a milrinone infusion was started for additional

Splenic Infarct: A Rare Presentation in a Pediatric Patient

A previously healthy 16-year-old male presented with a two day history of persistent epigastric pain. His physical examination was significant for tenderness in the left hypochondriac region with a palpable spleen 2cm below the left sub-costal margin. A CT scan of the abdomen showed a splenic infarct. Heterophile and EBV VCA IgM antibody test were positive. This is a rare case of infectious mononucleosis presenting with splenic infarct in an adolescent male without co-morbidities.

Trocar site hernia after laparoscopic sleeve gastrectomy using a specific open laparoscopy technique

BackgroundObesity is recognized as a risk factor for trocar site hernia (TSH) after laparoscopic surgery. Some recent studies have reported a TSH rate after bariatric surgery ranging from 0% to 1.6% using clinical evaluation and may underestimate the TSH rate. The objective of this study was to evaluate the TSH rate after sleeve gastrectomy (SG) by abdominal computed tomography (CT) scan. MethodsA retrospective review of all patients who underwent first-line SG and abdominal CT scan between March 2004 and February 2014 was performed. The primary endpoint was the incidence of TSH. Secondary endpoints were the site of TSH, the TSH rate with open laparoscopy using the authors’ technique, and risk factors for TSH after SG. ResultsDuring the period study, 1108 patients underwent first-line SG, including 10 cases of conversion to laparotomy (excluded from the present analysis). Of the remaining patients, 228 had abdominal CT scan (20.7%), with a mean age of 45.1 years (18–68 yr) and a mean BMI of 47.6 kg/m2 (33–75.4 kg/m2). The median time interval between SG and CT scan was 27 months (3–92 mo). CT scan revealed 44 TSH in 43 patients (18.8%). The site of the TSH was epigastric (16.6%), open laparoscopy (1.7%), right subcostal margin (0.8%), with no TSH in the left subcostal margin. In patients with>1 year of follow-up, the TSH rate was 19.7%. ConclusionThe TSH rate after bariatric surgery is underestimated. The authors’ open laparoscopy technique is a reliable technique with a low TSH rate. In the light of these results, the epigastric trocar site is systematically closed at the end of SG.

Novel Technique for Innervated Abdominal Wall Vascularized Composite Allotransplantation: A Multi-Layered Component Separation Approach.

Background: Abdominal wall vascularized composite allotransplant (AW-VCA) is currently used exclusively in conjunction with intestinal or multivisceral transplants and is the only non-functional VCA routinely preformed. However, applications of AW-VCA may expand if a functional graft with decreased immunosuppressive requirements can be designed. We hypothesize that it would be anatomically feasible to prepare a functional, neurotized and vascularized abdominal composite graft using a multi-layered component separation technique. Including vascularized bone in the graft design may decrease the immunosuppressive requirements by inducing immunologic chimerism. Methods: Two cadaver torsos were used. Adipocutaneous flaps were elevated from the midaxillary lines, preserving deep inferior epigastric artery (DIEA) perforators. A two layered component separation through the external and internal oblique fasciae was carried out, exposing segmental intercostal thoracolumbar nerves (see figure). Superiorly directed muscle release over the subcostal margin provided for a three-rib segment with attached rectus abdominis muscle. The remainder of the full thickness allograft was harvested, including its DIEAs. Flap inset into the recipient cadaver abdomen, with osteosynthesis fixation between donor and recipient ribs, was achieved.Figure: No Caption available.Results: The harvested grafts had an average size of 845±205cm2 with a total procurement time of 110 minutes. On one cadaver, 4 thoracolumbar nerves were isolated bilaterally, while the other cadaver yielded 3 nerves. The nerves were transected with an average length of 5.7±1.2cm. The DIEAs were transected with a length of 4.40±0.10cm. Conclusion: With the principles of component separation technique, we demonstrated a novel approach to harvest and transfer a neurotized osteomyofasciocutaneous abdominal wall allotransplant as a multi-pedicled, single functional unit. DISCLOSURES:Singh, D.: Other, KCI, Consultant, LifeCell, Consultant. Silverman, R.: Other, KCI, Chief Medical Officer.

The role of serine protease in the respiratory development (834.12)

The failure of complete diaphragm development, leading to a herniation or elevation of abdominal components into the pleural cavity, is thought to have a number of roles in the respiratory development. Here we reported a genetically modified mouse model, which have deletion in the gene coding for type II transmembrane serine protease‐1 (TMPRSS1), have defective diaphragms that mimic mild forms of the phenotypes of the diaphragm elevation in human. We notice the diaphragm of the genetically modified mouse line attached to the rib cage at a higher position compared to the wild type mice, as evidenced by the larger gap present between the subcostal margin and the diaphragm. As a result, the liver was shifted upward. Additionally, the average diameter and diaphragm area of these mice were found to be significantly smaller than those of wild type mice. While no difference in the thickness of the muscular layers was observed between the genetically modified and wild type mice, histology and morphometry analysis showed that the tendinous muscle of the genetically modified mice was significantly thinner than the wild type mice. The pulmonary functions, such as lung resistance and compliance, were also significantly impaired in the genetically modified mice. It is possible this is due to the higher position of the diaphragm and restricted expansion of the lung, since no significant differences in the weight or histology of lung tissue from hepsin KO and WT mice were detected. These observations suggest that during early development, the serine protease may play a role in maintaining structure and function of the diaphragm and we would suggest that a genetic analysis of this modified be performed in the patients of diaphragm elevation.