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By Donnie Rose Torres, MD October 3, 2013 ICU Conference Room

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1 By Donnie Rose Torres, MD October 3, 2013 ICU Conference Room
GRAND ROUNDS Mesenteric and Omental Cyst In An Infant By Donnie Rose Torres, MD October 3, 2013 ICU Conference Room

2 Objectives To present an approach to a case of abdominal distension in an infant To discuss the approach to diagnosis, incidence, management, complications and prognosis of patients with mesenteric and omental cyst To present hemangiolymphangioma as a histological finding for mesenteric and omental cyst

3 Patient’s Data Chief Complaint: ABDOMINAL DISTENSION RA DG,
1-month old female from Calbayog, Samar admitted from AFP Medical Center coordinated transfer to our institution last June 29, 2013 Chief Complaint: ABDOMINAL DISTENSION

4 History of Present Illness
Born to a 26-year old G1P1 (1001) non-smoker, non-alcoholic beverage drinker w/ PNCU w/ a private Calbayog, Samar BMHx wanted pregnancy (-) intake of abortifacients (+) exposure to Xray radiation at 1 month of pregnancy (+) UTI on the 1st trimester 1st trimester of pregnancy

5 History of Present Illness
4th and 5th mos of pregnancy - Normal abdominal UTZ and CAS 2nd trimester 7th mo - abdominal ultrasound revealed ascites, to consider toxic megacolon, to consider mass on the right abdomino-pelvic area 8th and 9th mos - right abdomino-pelvic mass 3rd trimester

6 History of Present Illness
FT via CS sec to fetal abdominal enlargement @ St. Camillus Hospital, Calbayog, Samar NMSAF, no cord coil 40 weeks by BS, APGAR 8,9 BW kg, BL – 47 cm, HC - 34 cm CC - 33 cm AC – 34 cm Abdomen not distended at that time Upon delivery

7 History of Present Illness
Routine NB care rendered 1st UO and BM w/in 24 hrs of life discharged with mother on the 3rd day of life on direct breastfeeding, with good suck and activity 1st to 3rd days of life follow-up UTZ: Abdomino-pelvic cystic mass, more on the right vs. massive ascites with septations, Normal liver, gall bladder, spleen, kidneys, urinary bladder and uterus Advised to seek consult in Manila 5th DOL

8 History of Present Illness
daily AC monitoring 10th DOL – 37cm 15th DOL – 39cm 20th DOL - 43cm 10-20th DOL progressive abdominal distension  AFP Medical Center  PCMC repeat UTZ - showed multi-loculated intraperitoneal fluid collection, subhepatic region down to pelvic cavity, to consider multi-loculated ascites vs ovarian pathology. Non visualized gall bladder, obscured aorta and pancreas. Normal ultrasound of the spleen and kidneys 23rd DOL

9 History of Present Illness
Pedia Sx - No indication for surgical intervention that time. PCMC  AFP Medical Center 23rd DOL Surgery Service  A> nonsurgical abdomen t/c ascites Admitted by Pedia service 23rd DOL (AFP Med Center)

10 History of Present Illness
LABS at AFP Med Center CBC TPAG Bilirubins Electrolytes ABG Hgb TP - 49 TB 10.57 Na 135 pH 7.41 Hct A – 39.1 B K 5.5 pCO2 29 Wbc 5.14 G – 9.9 B Cl 107 HCO3 18 Neu pO2 96 Lym ALT (2.37 x elevated) Crea <18 O2 sat 97% Plt AST 35 BE -5.8

11 History of Present Illness
PCMC  for Gynecology consult trans-abdominal UTZ: Prepubertal uterus, pelvoabdominal mass, ovarian new growth cannot be totally ruled out LDH, BHCG (0.15 IU)– Normal AFP - >1000 IU/ml Abdominal CT Scan: Loculated cyst vs Huge Pelvo-abdominal cystic mass 29th DOL beta HCG 0.15 mlU/ml ( )

12 History of Present Illness
patient remained stable, afebrile slight jaundice up to chest progression of the abdominal distension 36th DOL - coordinated transfer to PCMC for exploratory laparotomy Referred to Gyne, Surgery and Hematology 30-36th DOL

13 Review Of Systems General: (-) weight loss Skin: (-) excessive dryness
ENT: (-) epistaxis, (-) excessive salivation , (-) eye and ear discharge Neck: (-) limitation of movement Respiratory: (-) cough, (-) colds, (-) difficulty of breathing

14 Review Of Systems Gastrointestinal:
(-) vomiting, regular bowel movement, (-) constipation, (-) hematemesis, (-) melena, (-) hematochezia, (-) acholic stools Endocrine: (-) polyuria, (-) polydipsia Genitourinary: (-) discharge, (-) genital rashes, (-) hematuria Musculoskeletal: (-) limitation of movement Nervous system: (-) irritability, (-) changes in activity

15 Family History (+) history of DM – paternal side
(+) history of Hypertension – maternal side (-) history of Asthma, Malignancy, TB, Renal and Hepatic diseases on both sides 21 soldier 26 housewife

16 Immunization History (+) BCG
(+) Hepatitis B 1st dose - given upon birth

17 Nutritional History Exclusively breastfed

18 Past Medical History No history of blood transfusion
No allergies to drugs

19 Personal and Social History
lives with parents and 3 other relatives 2-storey, 3-bedroom house owned by their family (-) exposure to smoking (-) exposure to chronic cough use tap water for consumption garbage collected thrice a week

20 Physical Examination General Survey: awake, not irritable, active
Vital signs: BP: 80/50 Wt: 5.6 kgs (z score 2) BSA: 0.28 CR: 130 bpm Lt: 53 cm (z score 0) RR 34 cpm HC: 38 cm (z score 0) T: 36.7 oC CC: 35 cm AC: 44cm umbilical level, 46cm widest diameter Skin: jaundice from face to chest, warm, moist skin HEENT: open and flat anterior and posterior fontanelles, no molding, (+) erythematous maculopapular rashes on the face, anicteric sclerae, nonsunken eyeballs, pink palpebral conjunctiva, no nasoaural deformities and discharges, moist lips, no cleft lip, no cleft palate, no neck masses, no cervical lymphadenopathies

21 Physical Examination Chest and lungs:
no gross chest deformities, symmetric chest expansion, good air entry, clear breath sounds, no retractions Cardiovascular: adynamic precordium, no heaves nor thrills, regular rate and rhythm, distinct heart sounds, apex beat at 4th intercostals space mid-axillary line, no murmurs Genitalia: grossly female-looking genitalia Anus: patent anal opening Spine: straight spinal column, no spinal masses, no sacral dimpling, no tufts of hair Extremities: no polydactyly, no other deformities, no edema, full and equal pulses, good capillary refill time

22 Physical Examination Abdomen:
distended, AC – 44 cm umbilical level, 46 cm widest diameter (+) prominent abdominal veins normoactive bowel sounds Tympanitic soft (-) hepato-splenomegaly, (+) 5 x 6 cm, solid, ill-defined mass palpated midline

23 Neurologic Examination
awake, active CN I: not assessed CN II: (+) dazzle CN II , III: pupils 2-3 mm equally reactive to light CN III, IV, VI: full and equal EOM CN V: not assessed CN VII: no facial asymmetry CN VIII: not assessed CN IX, X: good gag reflex CN XI: not assessed CN XII: tongue at the midline, no fasciculation Motor: Normal muscle bulk; Normal muscle tone; No fasciculation Sensory: withdraws to pain stimuli (+) Babinski, bilateral, No clonus Signs of Meningeal Irritation: (-) nuchal rigidity, Brudzinski sign: negative, Kernig sign: negative   Reflexes: (+) rooting, palmar, Moro

24 On abd CT Scan and ff up UTZ: Physical Examination:
Salient Features 1 month old Female Progressive abdominal distension In Utero: * (+) exposure with Xray radiation * Ascites v R abdominopelvic mass at 7 to 9th mos on UTZ On abd CT Scan and ff up UTZ: T/C loculated ascites vs Huge Pelvo-abdominal cystic mass Physical Examination: * Jaundiced up to chest * Abdomen distended * Visible veins * (-) hepatosplenomegaly * 5 x 6 cm solid ill-defined mass palpated midline Labs: * increased AFP * increased ALT * Normal BHCG * Normal LDH

25 On abd CT Scan and ff up UTZ: Physical Examination:
Salient Features 1 month old Female Progressive abdominal disension In Utero: * (+) exposure with Xray radiation *Ascites v R abdominopelvic mass at 7 to 9th mos on UTZ On abd CT Scan and ff up UTZ: T/C loculated ascites vs Huge Pelvo-abdominal cystic mass Physical Examination: * Jaundiced up to chest * Abdomen distended * Visible veins * (-) hepatosplenomegaly * 5 x 6 cm solid ill-defined mass palpated midline Labs: * increased AFP * increased ALT * Normal BHCG * Normal LDH

26 Working Diagnosis Ascites vs Pelvo-abdominal Mass, Pobably:
1. Ovarian cyst 2. Germ cell tumor 3. Mesenteric and Omental cyst No wasting, no stunting

27 Approach to the Diagnosis
Abdominal distension hepatic ASCITES renal cardiac Ascites is defined by Nelsons as the accumulation of serous fluid within the pritoneal fluid Pregnancy/obesity tumors

28 Approach to the Diagnosis
miscellaneous trauma OLDER CHILDREN ASCITES neoplasia infection Gynecologic or GIT abN Hepatocellular disease

29 Approach to the Diagnosis
Spontaneous perforation of the bile duct biliary Perforation of choledocal cyst NEONATAL ASCITES urinary Complex urinary anomalies Perforation of bladder or ureteral tract chylous Ascites is defined by Nelsons as the accumulation of serous fluid within the peritoneal fluid Idiopathic Congenital lymphatic Abn Hernia Intususception Neoplasm External compression of lymphatics

30 Approach to the Diagnosis
Pertinent + Age group (< 3 months) Mild jaundice Abdominal distension Pertinent - Feeding intolerance Rare Direct hyperbilirubinemia UTZ findings biliary Pertinent + Abdominal distension NEONATAL ASCITES urinary Pertinent - Male predominance Prenatal UTZ oligohyramnios Metabolic acidosis Elevated BUN/Crea Electrolyte ABN chylous Ascites is defined by Nelsons as the accumulation of serous fluid within the peritoneal fluid Miscellaneous Cardiac Infection Pertinent + Congenital Abdominal distension UTZ and CT Scan findings Pertinent - Feeding intolerance Male predominance

31 Approach to the Diagnosis
Idiopathic CHYLOUS ASCITES Mesenteric/Omental Cyst Congenital lymphatic Abn Hernia Ovarian Mass Chylous Ascites – extravasation of milky chyle into the peritoneal cavity occur de novo as a result of trauma or obstruction of the lymphatic system MESENTERIC CYST External compression of lymphatics Intususception Tumors/Neoplasm Germ cell tumors hepatoblastoma

32 Assessment of Abdominal Mass Ch 78, by RH Sills Practical Algorithms in Hematology and Oncology

33 This is the plain abd CT Scan image of the patient.
To orient you, this is the axial cut at the level of the left kidney which is at L1 As you can see there is a large fluid collection in the abdomen predominantly occupying the mid to right hemiabdomen There is a mass effect into the adjacent structures in which in this slide the fluid pushing all the bowel loops to the contralateral side.

34 In this contrast-enhanced image, also an axial view this is at the level of the liver you can see the liver on the right, the gall bladder, the stomach and the spleen. In this level we can see the relationship of the liver to the superior portion of the fluid-filled mass.

35 This next image is another axial view of the abdomen at the level of bilateral kidneys after the contrast injection As you can see the previously described fluid collection in the abdomen has a fairly discernible thin border in this image and does not exhibit internal enhancement. Again, as previously said the mass effect to the surrounding structures is identified. So this large fairly defined pelvoabdominal cystic mass which appears to insinuate into the anterior pararenal space may represent a large massive ascites with probable loculations.

36 This is at the level of the pelvic cavity
This is at the level of the pelvic cavity. This is to show that the cystic mass occupies the pelvic region. The rest of the organs were unremarkable. Impression: Loculated ascites vs large pelvo-abdominal cyctic mass. Another differentials include large mesenteric/omental cyst, or ovarian in etiology.

37 Assessment of Abdominal Mass Ch 78, by RH Sills Practical Algorithms in Hematology and Oncology

38 Assessment of a Pelvic Mass Ch 80, by RH Sills Practical Algorithms in Hematology and Oncology

39 Working Diagnosis Ascites vs Pelvo-abdominal Mass, Pobably:
1. Ovarian cyst 2. Germ cell tumor 3. Mesenteric and Omental cyst No wasting, no stunting

40 LABS Course In the Wards CBC 6/29 Hgb 125.5 Hct 0.38 Platelet count
478 wbc 4.9 Basophils 0.01 Eosinophils 0.07 Neutrophils 0.25 Lymphocytes 0.52 Monocytes 0.15 Electrolytes 6/29 Na 135 K 4.8 Cl 108 Ca 2.65 P 2.09 Uric acid 201 Total prot 50.5 albumin 31 globulin 19.5 A/G ratio 1.6 1st Hosp day Jaundice Distended abdomen Prominent veins Soft No organomegaly Ill defined mass The rest of PE and Neuro exam NORMAL LABS On the 1st hospital day, patient was received at the emergency room, in good condition, with stable vital signs, afebrile, with note of jaundice on the face down to the chest with some maculopapular rashes on the face. Abdominal circumference was measured and noted to be distended at 44cm at the umbilical level and the widest diameter was measured at 46cm. It was noted to have prominent veins but soft and with no organomegaly. An ill-defined mass was palpable at midline of the abdomen measuring 5 x 6 cm. The rest of the physical examination was essentially normal. CBC, electrolytes, liver transaminases, BUN and Creatinine, albumin, as well as uric acid were normal. There was a note of indirect hyperbilirubinemia, with hyperphospatemia. LDH was noted to be 1.51x elevated and AFP was markedly increased. Patient was placed on heplock. Direct breastfeeding was continued. Patient was referred to Gynecology, Hematology and Surgery services for evaluation and management. LDH 934 AFP 8486 IU/ml AST 43 ALT 28 Bilirubins TB 5.6 DB 0.8 IB 4.7

41 Course In the Wards Heplock DBF Gyne, Hema and Surgery HEMA:
1st Hosp day Heplock DBF Gyne, Hema and Surgery HEMA: A> Pelvo-abdominal Mass prob ovarian in origin, r/o GCT Hydration Aluminum Hydroxide Monitoring of Tumor Markers JAUNDICE LDH BHCG AFP TUMOR MARKERS GYNE/SURG: A> Pelvo-abdominal Mass prob ONG Ex-Lap On the 1st hospital day, patient was received at the emergency room, in good condition, with stable vital signs, afebrile, with note of jaundice on the face down to the chest with some maculopapular rashes on the face. Abdominal circumference was measured and noted to be distended at 44cm at the umbilical level and the widest diameter was measured at 46cm. It was noted to have prominent veins but soft and with no organomegaly. An ill-defined mass was palpable at midline of the abdomen measuring 5 x 6 cm. The rest of the physical examination was essentially normal. CBC, electrolytes, liver transaminases, BUN and Creatinine, albumin, as well as uric acid were normal. There was a note of indirect hyperbilirubinemia, with hyperphospatemia. LDH was noted to be 1.51x elevated and AFP was markedly increased. Patient was placed on heplock. Direct breastfeeding was continued. Patient was referred to Gynecology, Hematology and Surgery services for evaluation and management.

42 Course In the Wards Hydration 2L/BSA VBG 6/30 pH 7.26 pCO2 26 HCO3
2nd Hosp day VBG 6/30 pH 7.26 pCO2 26 HCO3 11.7 BE -13.8 pO2 47 O2 sat 75% Electrolytes 6/30 Na 141 K 4.4 Cl 115 Ca 2.68 WARDS Gr 2/6 HS murmur 2nd ICS LPSB IVF mtn rate Electrolytes Na 137 K 5.6 Cl 108 Ca 2.75 2 d echo: PFO Salbutamol neb IVF mtn rate Hyperkalemia Hypercalcemia Rpt LABS Normokalemia Hypercalcemia Normal U/A Uncompensated Metabolic acidosis A> T/C Tumor Lysis Syndrome, Hypercalcemia of Malignancy On the 1st hospital day, patient was received at the emergency room, in good condition, with stable vital signs, afebrile, with note of jaundice on the face down to the chest with some maculopapular rashes on the face. Abdominal circumference was measured and noted to be distended at 44cm at the umbilical level and the widest diameter was measured at 46cm. It was noted to have prominent veins but soft and with no organomegaly. An ill-defined mass was palpable at midline of the abdomen measuring 5 x 6 cm. The rest of the physical examination was essentially normal. CBC, electrolytes, liver transaminases, BUN and Creatinine, albumin, as well as uric acid were normal. There was a note of indirect hyperbilirubinemia, with hyperphospatemia. LDH was noted to be 1.51x elevated and AFP was markedly increased. Patient was placed on heplock. Direct breastfeeding was continued. Patient was referred to Gynecology, Hematology and Surgery services for evaluation and management. Hydration 2L/BSA

43 Course In the Wards TUMOR LYSIS SYNDROME
2nd Hosp day Hyperkalemia Hyperphosphatemia Hyperuricemia Hyperuricosuria Hypocalcemia Lactic acidosis TUMOR LYSIS SYNDROME Vs Pre-treatment spontaneous TLS Treatment Targeted to specific metabolic disorder Hyperkalemia – pushes K back intracellularly Hyperphosphatemia – hydration, AlOH Acidosis – Hydration, NaHCO3 Laboratory TLS Cairo-Bishop definition TLS is defined as a group of metabolic complications that can occur after treatment of cancer,[1] usually lymphomas and leukemias, and sometimes even without treatment. These complications are caused by the breakdown products of dying cancer cells and include hyperkalemia, hyperphosphatemia, hyperuricemia and hyperuricosuria, hypocalcemia, and consequent acute uric acid nephropathy and acute renal failure. Cairo-Bishop definition In 2004, Cairo and Bishop defined a classification system for tumor lysis syndrome.[5] Laboratory tumor lysis syndrome: abnormality in two or more of the following, occurring within three days before or seven days after chemotherapy. uric acid > 8 mg/dL or 25% increase potassium > 6 meq/L or 25% increase phosphate > 4.5 mg/dL or 25% increase calcium < 7 mg/dL or 25% decrease Clinical tumor lysis syndrome: laboratory tumor lysis syndrome plus one or more of the following: increased serum creatinine (1.5 times upper limit of normal) cardiac arrhythmia or sudden death seizure A grading scale (0-5) is used depending on the presence of lab TLS, serum creatinine, arrhythmias, or seizures. The most common tumors associated with this syndrome are poorly differentiated lymphomas, such as Burkitt's lymphoma, and leukemias, such as acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). Other cancers (such as melanoma) have also been associated with TLS but are less common. Usually, the precipitating medication regimen includes combination chemotherapy, but TLS can be triggered in cancer patients by steroid treatment alone, and sometimes without any treatment—in this case the condition is referred to as "spontaneous tumor lysis syndrome".[2] Clinical TLS

44 Course In the Wards HYPERCALCEMIA OF MALIGNANCY Treatment
2nd Hosp day HYPERCALCEMIA OF MALIGNANCY Treatment Targeted to underlying cause Hydration – decreased Ca through dilution, incraeses renal Ca clearance Forced diuresis – increased Ca excretion, avoid volume overload, increase Ca reabsorption Bisphophonates – inhibit osteoclast activity Hypercalcemia of malignancy freq occurs in adults with wide variety of solid tumors but can also occur in children ALTHOUGH LESS OFTEN Hypercalcemia in patients with cancer is primarily due to increased bone resorption and release of calcium from bone. There are three major mechanisms by which this can occur: osteolytic metastases with local release of cytokines (including osteoclast activating factors); tumor secretion of parathyroid hormone-related protein (PTHrP); and tumor production of 1,25-dihydroxyvitamin D (calcitriol) [1-3]. These mechanisms will be reviewed here. Bisphosphonates are the most commonly used agents to treat hypercalcemia of malignancy. In addition, bisphosphonate therapy may prevent skeletal complications and perhaps improve survival in patients with multiple myeloma or breast cancer. These issues are all addressed elsewhere. (See "Treatment of hypercalcemia" and "Bisphosphonates and denosumab in patients with metastatic cancer" and "The use of bisphosphonates in patients with multiple myelo The initial step in the care of severely hypercalcemic patients is hydration and forced calciuresis. Because most of these patients are profoundly dehydrated, rehydration with 0.9 normal saline is the crystalloid of choice. Hydration helps decrease the calcium level through dilution. The expansion of extracellular volume also increases the renal calcium clearance. The rate of fluid therapy is based upon the following[4] : Degree of hypercalcemia Severity of dehydration Ability of the patient to tolerate rehydration - Vigilance to prevent volume overload is critical. Hydration is ineffective in patients with kidney failure because diuresis is impossible. Dialysis is necessary to correct hypercalcemia in patients with renal failure. Loop diuretics A loop diuretic (eg, furosemide) may be used with hydration to increase calcium excretion. This may also prevent volume overload during therapy. In contrast to loop diuretics, avoid thiazide diuretics because they increase the reabsorption of calcium. Bisphosphates - These agents will inhibit osteoclast activity for up to a month. However, these agents may take hours before reaching full therapeutic effect.[4] It has been reported in rhabdoid tumors of the kidney or cong mesoblastic nephroma and in children with neuroblastoma, medulloblastoma, leukemia, Burkitt lymphoma, dysgerminoma and rhabdomyosarcoma

45 Course In the Wards Pre-op Conference Pre-op Labs PT 7/7/13 patient
6th to 9th Hosp day Pre-op Conference Pre-op Labs PT 7/7/13 patient 10.80 control 11.3 activity 100 INR 0.95 PTT 35.8 28.1 Electrolytes 7/7 Na 135 K 4 Cl 109 Ca 2.55 P 1.78 Uric acid 240

46 Working Diagnosis Ascites vs Pelvo-abdominal Mass, Pobably:
1. Ovarian cyst 2. Germ cell tumor 3. Mesenteric and Omental cyst No wasting, no stunting

47 Course In the Wards Exploratory Laparotomy
10th Hosp day Exploratory Laparotomy Marsupialization of the mesenteric cyst Intra-op findings: Mesenteric cyst occupying almost all of the small intestine mesentery extending to the retroperitoneal space Intra-op findings: 5 x 7 cm omental cyst with chylous contents Milky ascites

48 Working Diagnosis Mesenteric and Omental Cyst
S/P Excision of Cyst and Marsupialization of Mesenteric Cyst (7/8/13), Chylous ascites No wasting, No stunting

49 This is a basic anatomy of the GI tract
The mesentery suspends the small and large intestine to the posterior abdominal wall it is also where the blood and lymphatics couse through This is the cut portion of the lesser omentum because which connects the lesser curvature of the stomach into the hepatic region This is the cut portion of the greater omentum attached to the greater curvature of the stomach which covers and drapes anteriorly the intestines

50 Mesenteric and Omental Cyst

51 History 1507: Benevieni while performing an autopsy descibed the mesenteric cyst on an 8-year-old boy 1842: Rokitansky recorded the first description of a chylous cyst 1852: Gairdner published the first report of an omental cyst 1880: Tillaux performed the first successful surgery for a cystic mass in the mesentery Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203: In 1907, the Italian anatomist Benevieni first reported a mesenteric cyst following an autopsy on an 8-year-old girl.[1] In 1842, von Rokitansky described a chylous mesenteric cyst.[2] Gairdner published the first report of an omental cyst in 1852.[3] Tillaux performed the first successful surgery for a cystic mass in the mesentery in 1880.[4]

52 Epidemiology Mesenteric and omental cysts are rare.
Incidence: 1 per 140,000 general hospital admissions 1 per 20,000 pediatric hospital admissions In a study from Egleston Children's Hospital at Emory University from , 14 patients were treated for mesenteric or omental cysts, which represents a prevalence of about 1 case per 11,250 admissions. 1/3 of cases: occur in children < 15 years old Mean age of children: 4.9 years old Mesenteric cysts > omental cysts(4.5x more) Mesenteric and omental cysts are rare; the incidence is about 1 per 140,000 general hospital admissions and about 1 per 20,000 pediatric hospital admissions.[5, 6, 7] In a study from Egleston Children's Hospital at Emory University from , 14 patients were treated for mesenteric or omental cysts, which represents a prevalence of about 1 case per 11,250 admissions.[8] Approximately one third of cases occur in children younger than 15 years.[9, 10, 11] The mean age of children affected is 4.9 years.[9, 11, 12, 13, 14, 15, 16] Mesenteric cysts are 4.5 times more common than omental cysts.[17]

53 Epidemiology “…48% were females. The mean age was 25 years, with a range from 1 day to 83 years.” Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203: “17 (age less than 10 years) with mesenteric cysts were managed in the hospital. The age ranged from 15 days to 10 years.” Prakash A. et al (Sep – Dec 2010). Early management of mesenteric cyst prevents catastrophes: a single centre analysis of 17 cases. Afr J Paediatr Surg (3):140-3 Mesenteric and omental cysts are rare; the incidence is about 1 per 140,000 general hospital admissions and about 1 per 20,000 pediatric hospital admissions.[5, 6, 7] In a study from Egleston Children's Hospital at Emory University from , 14 patients were treated for mesenteric or omental cysts, which represents a prevalence of about 1 case per 11,250 admissions.[8] Approximately one third of cases occur in children younger than 15 years.[9, 10, 11] The mean age of children affected is 4.9 years.[9, 11, 12, 13, 14, 15, 16] Mesenteric cysts are 4.5 times more common than omental cysts.[17]

54 Epidemiology “ There were nine females out of 16 (56%) and seven males (44%), with age range of years old.” Tan JJ, et al. (Sep 2009). Mesenteric cysts: an institution experience over\ 14 years and review of literature.World J Surg 33(9):1961-5 “The age ranged from 6 months to 68 years old with a mean of 22. Five of the patients were 10 years old or younger.” Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203: Mesenteric and omental cysts are rare; the incidence is about 1 per 140,000 general hospital admissions and about 1 per 20,000 pediatric hospital admissions.[5, 6, 7] In a study from Egleston Children's Hospital at Emory University from , 14 patients were treated for mesenteric or omental cysts, which represents a prevalence of about 1 case per 11,250 admissions.[8] Approximately one third of cases occur in children younger than 15 years.[9, 10, 11] The mean age of children affected is 4.9 years.[9, 11, 12, 13, 14, 15, 16] Mesenteric cysts are 4.5 times more common than omental cysts.[17]

55 Etiology Proposed theories:
Gross: “…mesenteric and omental cysts are thought to represent benign proliferations of ectopic lymphatics that lack communication with the normal lymphatic system.” “Cysts are thought to arise from lymphatic spaces associated with the embryonic retroperitoneal lymph sac, making them analogous to cystic hygromas which arise in the neck in association with the jugular lymph sac.” Egozi EI, Ricketts RR. Mesenteric and omental cysts in children. Am Surg. Mar 1997;63(3):287-90 Bliss DP Jr, Coffin CM, Bower RJ, et al. Mesenteric cysts in children. Surgery. May 1994;115(5):571-7. s proposed by Gross, mesenteric and omental cysts are thought to represent benign proliferations of ectopic lymphatics that lack communication with the normal lymphatic system.[9, 18, 19] Cysts are thought to arise from lymphatic spaces associated with the embryonic retroperitoneal lymph sac, making them analogous to cystic hygromas, which arise in the neck in association with the jugular lymph sac.[20] Another proposed etiology is lymphatic obstruction;[21] however, experimental occlusion of lymphatic channels in animals does not produce mesenteric or omental cysts because of the rich collaterals in the lymphatic system, which sheds doubt on this particular theory.[6, 18, 20] Other etiologic theories include (1) failure of the embryonic lymph channels to join the venous system, (2) failure of the leaves of the mesentery to fuse, (3) trauma, (4) neoplasia, and (5) degeneration of lymph nodes.[8]

56 Etiology Proposed theories: lymphatic obstruction
however, experimental occlusion of lymphatic channels in animals does not produce mesenteric or omental cysts because of the rich collaterals in the lymphatic system, which sheds doubt on this particular theory.[ Other etiologic theories include failure of the embryonic lymph channels to join the venous system failure of the leaves of the mesentery to fuse trauma neoplasia degeneration of lymph nodes Takiff H, Calabria R, Yin L, Stabile BE. Mesenteric cysts and intra-abdominal cystic lymphangiomas. Arch Surg. Nov 1985;120(11): Vanek VW, Phillips AK. Retroperitoneal, mesenteric, and omental cysts. Arch Surg. Jul 1984;119(7): Egozi EI, Ricketts RR. Mesenteric and omental cysts in children. Am Surg. Mar 1997;63(3): s proposed by Gross, mesenteric and omental cysts are thought to represent benign proliferations of ectopic lymphatics that lack communication with the normal lymphatic system.[9, 18, 19] Cysts are thought to arise from lymphatic spaces associated with the embryonic retroperitoneal lymph sac, making them analogous to cystic hygromas, which arise in the neck in association with the jugular lymph sac.[20] Another proposed etiology is lymphatic obstruction;[21] however, experimental occlusion of lymphatic channels in animals does not produce mesenteric or omental cysts because of the rich collaterals in the lymphatic system, which sheds doubt on this particular theory.[6, 18, 20] Other etiologic theories include (1) failure of the embryonic lymph channels to join the venous system, (2) failure of the leaves of the mesentery to fuse, (3) trauma, (4) neoplasia, and (5) degeneration of lymph nodes.[8]

57 Incidence PPS PCMC

58 Clinical Presentation
incidental finding during laparotomy present with abdominal distention and few associated symptoms other than vague abdominal pain with or without a palpable mass mass may be huge, simulating ascites acute presentation in children is that of a small-bowel obstruction, which may be associated with intestinal volvulus or infarction These masses can be detected using prenatal UTZ appear as a sonolucent Mesenteric and omental cysts can be discovered as an incidental finding during laparotomy for another condition or they can manifest as an acute life-threatening intra-abdominal catastrophe.[22] Children generally present with abdominal distention and few associated symptoms other than vague abdominal pain with or without a palpable mass.[21, 23] The mass may be huge, simulating ascites.[24] The most common mode of acute presentation in children is that of a small-bowel obstruction, which may be associated with intestinal volvulus or infarction.[11, 13, 15] Approximately 10% of patients with mesenteric and omental cysts present with an acute abdominal emergency.[22] These masses can be detected using prenatal ultrasonography and appear as a sonolucent mass. The prenatal differential diagnosis includes dilated bowel (eg, intestinal atresia), dilated stomach (eg, pyloric atresia), gastrointestinal duplication, hydronephrosis, ovarian cyst, and cystic teratoma. Ricketts RR. Mesenteric and omental cysts. In: Pediatric Surgery. 5th ed. 1998:

59 Clinical Presentation
Analysis of the younger patients in this series shows that they have shorter duration of symptoms and are more likely to present as surgical emergencies. Although the average size and volume are identical for adults, they were fewer retroperitoneal cysts in the younger age group. This resulted in a higher resectability and a significant lower recurrence. Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203:

60 Clinical Presentation
“In a series of 82 children who underwent surgery for various causes of intestinal volvulus, mesenteric cysts were the underlying etiology in 3.65% of cases.” Maung M, Saing H. Intestinal volvulus: an experience in a developing country. J Pediatr Surg. May 1995;30(5): A very unusual presentation of a mesenteric cyst is that of an irreducible inguinal hernia. Mohanty SK, Bal RK, Maudar KK. Mesenteric cyst--an unusual presentation. J Pediatr Surg. May 1998;33(5):792-3. In a series of 82 children who underwent surgery for various causes of intestinal volvulus, mesenteric cysts were the underlying etiology in 3.65% of cases.[25] A very unusual presentation of a mesenteric cyst is that of an irreducible inguinal hernia.[1] The differential diagnosis includes intestinal duplication cyst; ovarian, choledochal, pancreatic, splenic, or renal cysts; hydronephrosis; cystic teratoma; hydatid cyst; and ascites.[22]

61 Diagnostics Plain radiographs Abdominal Ultrasound
CT Scan of the abdomen Radionuclide scan of biliary tract CT Scan with angiography Radiography Plain abdominal radiography may reveal a gasless, homogeneous, water-dense mass that displaces bowel loops laterally or anteriorly in the presence of a mesenteric cyst or posteriorly in the presence of an omental cyst.[8, 21] Fine calcifications can sometimes be observed within the cyst wall.[13, 26, 27] Ultrasonography The imaging modality of choice is abdominal ultrasonography.[11, 15, 16, 27] Ultrasonography reveals fluid-filled cystic structures, commonly with thin internal septi and sometimes with internal echoes from debris, hemorrhage, or infection.[9, 16, 27, 28] However, these can be confused with large ovarian cysts in the fetus and newborn. Enteric duplication cysts, on the other hand, are thick-walled structures that share a common muscular wall with the adjacent bowel. They also have a clearly visible mucosal lining on ultrasonography. Ultrasound image demonstrating a thin-walled mesenteric cyst with an internal septum. CT scanning Abdominal CT scanning adds minimal additional information, although it can reveal that the cyst is not arising from another organ such as the kidney, pancreas, or ovary.[7] Radionuclide scanning of the biliary tract excludes choledochal cysts from diagnostic consideration.[22] Contrast CT scanning aided with angiography may be useful in cases of large omental cysts.[29] Ultrasound image demonstrating a thin-walled mesenteric cyst with an internal septum

62 Diagnostics Diagnostic includes abdominal computed tomography, ultrasound and MRI. Barium enema examination or intravenous pyelogram may be used in special cases. Casarroto, A. et al (May ). Mesenteric cyst: case report and review of the literature. G Chir (5):239-42 USG was not conclusive in all. Abdominal CT scan with intravenous contrast was diagnostic in nine patients. Five patients had volvulus on exploration. Prakash A. et al (Sep – Dec 2010). Early management of mesenteric cyst prevents catastrophes: a single centre analysis of 17 cases. Afr J Paediatr Surg (3):140-3 Diagnostic includes abdominal computed tomography, ultrasound and MRI. Barium enema examination or intravenous pyelogram may be used in special cases. Casarroto, A. et al (May ). Mesenteric cyst: case report and review of the literature. G Chir (5):239-42 USG was not conclusive in all. Abdominal CT scan with intravenous contrast was diagnostic in nine patients. Five patients had volvulus on exploration. Prakash A. et al (Sep – Dec 2010). Early management of mesenteric cyst prevents catastrophes: a single centre analysis of 17 cases. Afr J Paediatr Surg (3):140-3

63 Gross Findings “…occur anywhere in the mesentery of the gastrointestinal tract from the duodenum to the rectum, and they may extend from the base of the mesentery into the retroperitoneum.” In a series of 162 patients: 60% : small-bowel mesentery 24% in the large-bowel mesentery 14.5% in the retroperitoneum They most commonly occur in the ileal mesentery of the small bowel or the sigmoid mesentery of the colon. Omental cysts are confined to the lesser or greater omentum. Large mesenteric cyst arising from the small-bowel mesentery. Huge omental cyst within the greater omentum Small omental cyst arising on a pedicle from the greater omentum in the region of the transverse colon. Multiple mesenteric cysts, some filled with chyle, arising from the jejunal mesentery. “Mesenteric cysts can occur anywhere in the mesentery of the gastrointestinal tract from the duodenum to the rectum, and they may extend from the base of the mesentery into the retroperitoneum.In a series of 162 patients, 60% of mesenteric cysts occurred in the small-bowel mesentery, 24% in the large-bowel mesentery, and 14.5% in the retroperitoneum. They most commonly occur in the ileal mesentery of the small bowel or the sigmoid mesentery of the colon.[5] Omental cysts are confined to the lesser or greater omentum.[22] Vanek VW, Phillips AK. Retroperitoneal, mesenteric, and omental cysts. Arch Surg. Jul 1984;119(7): Mollitt DL, Ballantine TV, Grosfeld JL. Mesenteric cysts in infancy and childhood. Surg Gynecol Obstet. Aug 1978;147(2):182-4.

64 Gross Findings There were 23/162 (14%) patients with retroperitoneal cysts and 139/162 (86%) with mesenteric cysts. Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203: Cysts were located in small intestinal mesentery in 14 cases and 3 were in the sigmoid mesentery. Prakash A. et al (Sep – Dec 2010). Early management of mesenteric cyst prevents catastrophes: a single centre analysis of 17 cases. Afr J Paediatr Surg (3):140-3 The cyst was located in the small bowel mesentery in 91 patients (60%), in the large bowel mesentery in 37 (24%) and in the retroperitoneum in 23 (4.5%) In 11 cases, the location was not described. Large mesenteric cyst arising from the small-bowel mesentery. Huge omental cyst within the greater omentum Small omental cyst arising on a pedicle from the greater omentum in the region of the transverse colon. Multiple mesenteric cysts, some filled with chyle, arising from the jejunal mesentery. “Mesenteric cysts can occur anywhere in the mesentery of the gastrointestinal tract from the duodenum to the rectum, and they may extend from the base of the mesentery into the retroperitoneum.In a series of 162 patients, 60% of mesenteric cysts occurred in the small-bowel mesentery, 24% in the large-bowel mesentery, and 14.5% in the retroperitoneum. They most commonly occur in the ileal mesentery of the small bowel or the sigmoid mesentery of the colon.[5] Omental cysts are confined to the lesser or greater omentum.[22]

65 Gross Findings They ranged in size in the 8 patients in whom this information is known from 4 to 36 cm in diameter. The estimated volume of the cysts ranged in these patients from 11 to 5600 cc. Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203:

66 Gross Findings Large mesenteric cyst arising from the small-bowel mesentery. Huge omental cyst within the greater omentum Small omental cyst arising on a pedicle from the greater omentum in the region of the transverse colon. Multiple mesenteric cysts, some filled with chyle, arising from the jejunal mesentery. Vanek VW, Phillips AK. Retroperitoneal, mesenteric, and omental cysts. Arch Surg. Jul 1984;119(7): Mollitt DL, Ballantine TV, Grosfeld JL. Mesenteric cysts in infancy and childhood. Surg Gynecol Obstet. Aug 1978;147(2):182-4.

67 Histologic Findings “Histologically, all were lymphangiomatous mesenteric cysts.” Prakash A. et al (Sep – Dec 2010). Early management of mesenteric cyst prevents catastrophes: a single centre analysis of 17 cases. Afr J Paediatr Surg (3):140-3 None of the cysts were malignant. Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203: Large mesenteric cyst arising from the small-bowel mesentery. Huge omental cyst within the greater omentum Small omental cyst arising on a pedicle from the greater omentum in the region of the transverse colon. Multiple mesenteric cysts, some filled with chyle, arising from the jejunal mesentery. “Mesenteric cysts can occur anywhere in the mesentery of the gastrointestinal tract from the duodenum to the rectum, and they may extend from the base of the mesentery into the retroperitoneum.In a series of 162 patients, 60% of mesenteric cysts occurred in the small-bowel mesentery, 24% in the large-bowel mesentery, and 14.5% in the retroperitoneum. They most commonly occur in the ileal mesentery of the small bowel or the sigmoid mesentery of the colon.[5] Omental cysts are confined to the lesser or greater omentum.[22]

68 Histologic Findings Although most of the cysts were benign, three had foci of malignancy and another had a focus of gastrointestinal stromal tumor. None of the cases recurred during follow-up. Tan JJ, et al. (Sep 2009). Mesenteric cysts: an institution experience over 14 years and review of literature.World J Surg 33(9):1961-5 Although most of the cysts were benign, three had foci of malignancy and another had a focus of gastrointestinal stromal tumor. None of the cases recurred during follow-up. Tan JJ, et al. (Sep 2009). Mesenteric cysts: an institution experience over 14 years and review of literature.World J Surg 33(9):1961-5

69 Differentials intestinal duplication cyst Ovarian cyst
choledochal, pancreatic, splenic, or renal cysts Hydronephrosis cystic teratoma hydatid cyst Ascites Ricketts RR. Mesenteric and omental cysts. In: Pediatric Surgery. 5th ed. 1998: “differential diagnosis: lymphangiomas, sarcomas, adenocarcinomas and intestinal duplications should be considered.” Casarroto, A. et al (May ). Mesenteric cyst: case report and review of the literature. G Chir (5):239-42 The differential diagnosis includes intestinal duplication cyst; ovarian, choledochal, pancreatic, splenic, or renal cysts; hydronephrosis; cystic teratoma; hydatid cyst; and ascites.[22]

70 Surgery In children, the most common indication for surgical intervention is the presence of an abdominal mass with or without signs of intestinal obstruction. The surgical treatment of choice for retroperitoneal and mesenteric cysts is complete enucleation. If this could not be accomplished, the next best alternative would be excision of the cyst with, if necessary, the resection of a portion of the adherent bowel. The last acceptable choice is the marsupialization of the cyst. Partial excision or simple drainage of the cyst usually results to recurrence. Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203: In children, the most common indication for surgical intervention is the presence of an abdominal mass with or without signs of intestinal obstruction. Various complications have been associated with mesenteric and omental cysts, including intestinal obstruction (most common), volvulus, hemorrhage into the cyst, infection, rupture, cystic torsion, and obstruction of the urinary and biliary tract.[22] Malignant transformation of mesenteric cysts has occurred in adults,[5] but malignant mesenteric and omental cysts have not been reported in children.[22] The surgical treatment of choice for retroperitoneal and mesenteric cysts is complete enucleation. If this could not be accomplished, the next best alternative would be excision of the cyst with, if necessary, the resection of a portion of the adherent bowel. The last acceptable choice is the marsupialization of the cyst. Partial excision or simple drainage of the cyst usually results to recurrence.

71 Surgery Surgical treatment is indicated also in asymptomatic patients; laparoscopic approach is the "gold standard". Laparotomic approach should be used in the cases of impossibility of total enucleation or in the cases of malignant degeneration. Casarroto, A. et al (May ). Mesenteric cyst: case report and review of the literature. G Chir (5):239-42 Surgical treatment is indicated also in asymptomatic patients; laparoscopic approach is the "gold standard". Laparotomic approach should be used in the cases of impossibility of total enucleation or in the cases of malignant degeneration.

72 Surgery A simultaneous intestinal resection was performed in 56 of the 134 patients in whom this information is known. In 37 cases, a resection of the small intestine was performed. In 3 cases, a right hemicolectomy was necessary with an ileotransverse anastomosis. There was 1 case each for mesenteric and retroperitoneal cyst requiring resection of the sigmoid colon. Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203: Laparoscopic surgical excision of the cyst was performed in 3 (19%) patients, laparotomy in 12 (75%), and 1 patient refused surgery. Tan JJ, et al. (Sep 2009). Mesenteric cysts: an institution experience over 14 years and review of literature.World J Surg 33(9):1961-5

73 Correct Diagnosis Diagnosis was proven in all cases of Laparotomy.
The correct pre-operative diagnosis was made in only 3 cases out of 162 and 2 of these were patients with retroperitoneal cysts. Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203: The correct pre-operative diagnosis was made in 30 out of 122 patients (25%) in whom this information is know, 45% were operated as emergencies.

74 Course in the wards LABS Admitted at ICU for post-op care 10th HD
CBC 7/08 Hgb 100 Hct 0.29 Platelet count 639 wbc 6.5 Basophils 0.01 Eosinophils Neutrophils 0.80 Lymphocytes 0.14 Monocytes 0.05 Electrolytes 7/8 Na 133 K 4.2 Cl 111 Ca 2.32 Total prot 42.30 albumin 23.70 globulin 18.70 A/G ratio 1.30 Creatinine 34 BUN 0.90 CBC 7/09 Hgb 128 Hct 0.37 Platelet count 568 wbc 3.5 Basophils Eosinophils 0.01 Neutrophils 0.24 Lymphocytes 0.61 Monocytes 0.14 PT 7/8/13 patient 10.90 control 11.3 activity 100 INR 0.96 PTT 35 27 Intra-op: had 1 hypotensive episode, BP 60/40 while draining 400 cc chyle from omental cyst and from peritoneal cavity EBL: 20cc Admitted at ICU for post-op care ABG 7/8 pH 7.33 pCO2 27 HCO3 14.2 BE -10.2 pO2 123 O2 sat 99% LABS

75

76 Course in the wards NPO OGT was kept open CBG monitoring Omeprazole IV
10th HD NPO OGT was kept open CBG monitoring Omeprazole IV IVF hydration – 2L/BSA Cefuroxime – post-op antibiotics Pain management

77 Course in the wards Minimal OGT output  Clamped
11th HD Minimal OGT output  Clamped Serous output on the post-op site Abdomen nondistended, soft, nontender Trans-out to Surgery wards Maintained on NPO Hydration and meds continued

78 Course in the wards Problems: IVF decreased to maintenance rate
12 to 14th HD CBC 7/12 Hgb 107.7 Hct 0.32 Platelet count 402 wbc 3.5 Basophils 0.02 Eosinophils 0.08 Neutrophils 0.22 Lymphocytes 0.56 Monocytes 0.12 Problems: Edematous Hypoglycemia Anemia Decreased leukocyte hypoalbuminemia IVF decreased to maintenance rate Cefuroxime increased to 150mkday Trial feeding Minimal milky, serous output from the drain Total prot 42.20 albumin 23.80 globulin 18.50 A/G ratio 1.30 45.90 28.00 17.80 1.60

79 CHYLOUS ASCITES treatment
Course in the wards 15th to 17th HD Albumin and pRBC transfusion Decreased milky output from post-op site Referred to GI: dietary specifications Treat the underlying cause. CHYLOUS ASCITES treatment Role of octreotide?? Repeated paracentesis “Multiple case reports describe the use of octreotide, a somatostatin analog, in the management of chylous ascites, typically at a dose of 100 mcg administered subcutaneously 3 times per day.” Yilmaz M, Akbulut S, Isik B, Ara C, Ozdemir F, Aydin C, et al. Chylous ascites after liver transplantation: incidence and risk factors. Liver Transpl. Sep 2012;18(9): Diuretic therapy GI service: Resume DBF Shift IVF to HL D/C Omeprazole “A combination of total parenteral nutrition and subcutaneous octreotide has been used to successfully treat congenital chylous ascites in a newborn.” Olivieri C, Nanni L, Masini L, Pintus C. Successful management of congenital chylous ascites with early octreotide and total parenteral nutrition in a newborn. BMJ Case Rep. Sep ;2012 Salt and water restriction Because chylous ascites is a manifestation rather than a disease by itself, the prognosis depends on the treatment of the underlying disease or cause. Supportive measures can relieve the symptoms. These measures include repeated paracentesis, diuretic therapy, salt and water restriction, elevation of legs with use of supportive stockings, and dietary measures. Lymphatic flow increases after the ingestion of a fatty meal. The fatty acids derived from short-chain and medium-chain triglycerides diffuse directly across enterocytes into the portal venous system. Their absorption does not affect lymphatic flow. However, the fatty acids derived from long-chain triglycerides are re-esterified into triglycerides in the enterocyte. They are then incorporated into chylomicrons which subsequently enter the lymphatic system. A low-fat diet with medium-chain triglyceride supplementation can reduce the flow of chyle into the lymphatics.[24] Typically, medium-chain triglyceride oil is administered orally at a dose of 15 mL 3 times per day at meals. However, this approach is frequently not successful. One recent case report described the successful use of orlistat (Xenical) in a patient who had difficulty complying with a low-fat diet.[25] If chylous ascites persists despite dietary management, the next step may involve bowel rest and the institution of total parenteral nutrition.[15] Bowel rest and total parenteral nutrition are postulated to be beneficial in patients with posttraumatic or postsurgical chylous ascites. Paracentesis can result in immediate symptom relief; however, reaccumulation of fluid usually follows, and patients may require repeated paracentesis. Some authorities have advocated large-volume paracentesis. Morbidity from a single tap is usually low, but complications, such as peritonitis and hemorrhage, can occur. Transfusion of albumin and/or RBCs during paracentesis may help prevent hypovolemia in patients with hypoalbuminemia or anemia. Multiple case reports describe the use of octreotide, a somatostatin analog, in the management of chylous ascites, typically at a dose of 100 mcg administered subcutaneously 3 times per day.[15, 16, 26, 27, 28] A combination of total parenteral nutrition and subcutaneous octreotide has been used to successfully treat congenital chylous ascites in a newborn.[29] Experimental work in humans has shown that somatostatin can significantly decrease postprandial increases in triglyceride levels. This effect cannot be explained by either inhibition of gastric emptying or inhibition of exocrine pancreatic secretion.[30, 31] Octreotide is most likely effective in chylous ascites on account of its ability to inhibit lymphatic flow. Indeed, in a canine model, infusion of somatostatin resulted in a decrease in lymph flow, measured via a cannula inserted into the thoracic duct.[32] Postsurgical chylous ascites usually resolves with supportive therapy. Early reoperation is indicated when the site of leakage is apparent and if the patient is a good operative candidate.[33] Case reports now describe the laparoscopic treatment of chylous leaks, using suture ligation and fibrin glue to control the leak.[34] In another report, fibrin glue applied to absorbable mesh was useful in patients with large areas of diffuse lymphatic leakage.[35] Another report describes the treatment of chylous ascites after laparoscopic Nissen fundoplication with percutaneous injection of tissue glue (ie, N -butyl-cyanoacrylate mixed with ethiodol) into the thoracic duct.[36] Dietary measures Bowel rest and TPN “low-fat diet with medium-chain triglyceride supplementation can reduce the flow of chyle into the lymphatics.” Weinstein LD, Scanlon GT, Hersh T. Chylous ascites. Management with medium-chain triglycerides and exacerbation by lymphangiography. Am J Dig Dis. Jul 1969;14(7):500-9.

80 Course in the wards Rpt LDH: 678 Cefuroxime x 7 days – d/c
17th – 19th HD Rpt LDH: 678 Rpt AFP: 8486  1467 IU/ml Cefuroxime x 7 days – d/c 1cc output from the drain  removed Multivitamins and Zinc Transferred to AFPMC AFP Normal for age The normal range of AFP for adults and children is variously reported as under 50, under 10, and under 5 ng/mL.[12][13] At birth, normal infants have AFP levels 4 or more orders of magnitude above this normal range, that decreases to a normal range over the first year of life.[14][15][16][17][18][19] During this time, the normal range of AFP levels spans approximately 2 orders of magnitude.[16] Correct evaluation of abnormal AFP levels in infants must take into account these normal patterns.[16] Very high AFP levels may be subject to hooking (see Tumor marker), which results in the level being reported significantly lower than the actual concentration.[20] This is important for analysis of a series of AFP tumor marker tests, e.g. in the context of post-treatment early surveillance of cancer survivors, where the rate of decrease of AFP has diagnostic value. FP is measured in pregnant women through the analysis of maternal blood or amniotic fluid, as a screening test for a subset of developmental abnormalities. Some of the diseases in which AFP will be elevated in a person are listed below: Omphalocele[21] Down Syndrome (decrease)[22] Hepatocellular carcinoma/hepatoma: ↑ α-fetoprotein[23] Neural tube defects: ↑ α-fetoprotein in amniotic fluid and maternal serum[23][24] Nonseminomatous germ cell tumors Yolk sac tumor[23] Ataxia telangectasia: Elevation of AFP is used as one factor in the diagnosis of ataxia telangiectasia.[25] Tumors: AFP can also be used as a biomarker to detect a subset of tumors in non-pregnant women, men, and children. A level above 500 nanograms/milliliter of AFP in adults can be indicative of hepatocellular carcinoma, germ cell tumors, and metastatic cancers of the liver. A peptide derived from AFP that is referred to as AFPep is claimed to possess anti-cancer properties.[26]

81 Complications “Various complications …., including intestinal obstruction (most common), volvulus, hemorrhage into the cyst, infection, rupture, cystic torsion, and obstruction of the urinary and biliary tract. Ricketts RR. Mesenteric and omental cysts. In: Pediatric Surgery. 5th ed. 1998: Complications are rare: rupture, infection and intestinal obstruction. Pisano, G. et al (Aug 2004). Acute abdomen due to rupture of mesenteric cysts. Observations on a clinical case and review of the literature. Minerva Chir. 59(4): “Various complications have been associated with mesenteric and omental cysts, including intestinal obstruction (most common), volvulus, hemorrhage into the cyst, infection, rupture, cystic torsion, and obstruction of the urinary and biliary tract.[22] Malignant transformation of mesenteric cysts has occurred in adults,[5] but malignant mesenteric and omental cysts have not been reported in children Complications are rare: rupture, infection and intestinal obstruction. Pisano, G. et al (Aug 2004). Acute abdomen due to rupture of mesenteric cysts. Observations on a clinical case and review of the literature. Minerva Chir. 59(4):

82 Prognosis Malignant transformation of mesenteric cysts has occurred in adults, but malignant mesenteric and omental cysts have not been reported in children Ricketts RR. Mesenteric and omental cysts. In: Pediatric Surgery. 5th ed. 1998: Only 1 patient developed a recurrence of the cyst and required second operation. This patient had retroperitoneal cyst which was partially excised at the 1st operation. She is doing well 7 years after marsupialization of the cyst, despite the persistent drainage of fluid. Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203:

83 Prognosis 5 out of 162 (3%) patients were found to have malignant cysts. 10 cysts recurred, requiring 2nd operation. 5 of these recurrences in the 23 patients (22%) whose cysts are retroperitoneal in location (p<0.001). All cancers were found in adult patients. There were 3 deaths out of 162 (1.9%). 1 death reported in retroperitoneal cysts. Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203:

84 Recurrence There is a statistically significant correlation between the recurrence of the cyst and location. 5/10 recurrences (50%) occurred in retroperitoneal cysts. There is no correlation between age, duration of symptom, diameter or volume of cyst and to RECURRENCE. Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203: Retroperitoneal cysts are technically more difficult to excise completely because of their proximity to blood vessels and other organs, and therefore a RECURRENCE is more likely. There is a statistically significant correlation between the recurrence of the cyst and location. 5/10 recurrences (50%) occurred in retroperitoneal cysts. There is no correlation between age, duration of symptom, diameter or volume of cyst and to RECURRENCE. Kurtz, RJ et al. (Jan 2010). Mesenteric and retroperitoneal cyst. Annals of Surgery. Ann Surgery 203:  Retroperitoneal cysts are technically more difficult to excise completely because of their proximity to blood vessels and other organs, and therefore a RECURRENCE is more likely.

85 No atypia nor malignancy seen.
Tissue Diagnosis Impression: Hemangiolymphangioma Histologic sections of omentum and mesenteric portions show several, small vascular channels and lymphatic spaces set in fibrocollagenous stroma that contain occasional lymphoid aggregation. No atypia nor malignancy seen.

86 Histologic Findings Cystic lymphangiomas vs mesenteric and omental cysts Cystic lymphangiomas: have an endothelial cell lining, foam cells, and thin walls that contain lymphatic spaces, lymphoid tissue, and smooth muscle. Mesenteric cysts: lack smooth muscle and lymphatic spaces, and the cells lining the cysts are cuboidal or columnar in nature Takiff H, Calabria R, Yin L, Stabile BE. Mesenteric cysts and intra-abdominal cystic lymphangiomas. Arch Surg. Nov 1985;120(11): Bliss DP Jr, Coffin CM, Bower RJ, et al. Mesenteric cysts in children. Surgery. May 1994;115(5):571-7. Cystic lymphangiomas are sometimes differentiated from mesenteric and omental cysts. Cystic lymphangiomas have an endothelial cell lining, foam cells, and thin walls that contain lymphatic spaces, lymphoid tissue, and smooth muscle. Mesenteric cysts lack smooth muscle and lymphatic spaces, and the cells lining the cysts are cuboidal or columnar in nature

87 Histologic Findings Lymphangiomas are more diffuse and occur in the mesentery or retroperitoneum, and patients may present earlier in life than those with mesenteric or omental cysts. In a series of 191 patients with lymphangioma, 4.7% of patients presented with lymphangioma in the mesentery. Alqahtani A, Nguyen LT, Flageole H, Shaw K, Laberge JM. 25 years' experience with lymphangiomas in children. J Pediatr Surg. Jul 1999;34(7):1164-8 An omental cyst has the same histologic characteristics but is confined to the greater or lesser omentum

88 Hemangiolymphangioma
Lymphangiomas : malformations of the lymphatic system, which is the network of vessels responsible for returning to the venous system excess fluid from tissues These malformations can occur at any age and may involve any part of the body, but 90% occur in children less than 2 years of age and involve the head and neck. These malformations are either congenital or acquired. Congenital lymphangiomas: Turner syndrome, isolated case. Acquired lymphangiomas: trauma, inflammation, or lymphatic obstruction Lymphangiomas : malformations of the lymphatic system, which is the network of vessels responsible for returning to the venous system excess fluid from tissues These malformations can occur at any age and may involve any part of the body, but 90% occur in children less than 2 years of age and involve the head and neck. These malformations are either congenital or acquired. Congenital lymphangiomas are often associated with chromosomal abnormalities such as Turner syndrome although they can also exist in isolation. Lymphangiomas are commonly diagnosed before birth using fetal ultrasonography Acquired lymphangiomas may result from trauma, inflammation, or lymphatic obstruction.

89 Hemangiolymphangioma
soft, slow-growing, "doughy" mass. lymphangiomas are usually treated for cosmetic reasons only Complications: such as respiratory distress when a lymphangioma compresses the airway Treatment: aspiration, surgical excision, laser and radiofrequency ablation and sclerotherapy Most lymphangiomas are benign lesions that result only in a soft, slow-growing, "doughy" mass. Since they have no chance of becoming malignant, lymphangiomas are usually treated for cosmetic reasons only. Rarely, impingement upon critical organs may result in complications, such as respiratory distress when a lymphangioma compresses the airway. Treatment includes aspiration, surgical excision, laser and radiofrequency ablation and sclerotherapy.

90 Hemangiolymphangioma
 3 subtypes: Capillary, Cavernous and Cystic Lymphangioma based on their microscopic characteristics A fourth subtype, the hemangiolymphangioma is also recognized. Capillary lymphangiomas Cavernous lymphangiomas Cystic hygromas Hemangiolymphangioma Lymphangiomas have traditionally been classified into three subtypes: capillary and cavernous lymphangiomas and cystic hygroma. This classification is based on their microscopic characteristics. A fourth subtype, the hemangiolymphangioma is also recognized. Capillary lymphangiomas Capillary lymphangiomas are composed of small, capillary-sized lymphatic vessels and are characteristically located in the epidermis Cavernous lymphangiomas Composed of dilated lymphatic channels, cavernous lymphangiomas characteristically invade surrounding tissues Cystic hygromas Cystic hygromas are large, macrocystic lymphangiomas filled with straw-colored, protein-rich fluid. Hemangiolymphangioma As suggested by their name, hemangiolymphangiomas are lymphangiomas with a vascular component.

91 Review of literature INTRODUCTION:
“…..There are several classifications of these formations, among which the one based on histopathologic features including 6 groups has been most commonly used: 1) cysts of lymphatic origin--lymphatic (hilar cysts) and lymphangiomas; 2) cysts of mesothelial origin--benign or malignant mesothelial cysts; 3) enteric cysts; 4) cysts of urogenital origin; 5) dermoid cysts; and 6) pseudocysts--infectious or traumatic etiology.” Kirurski, O. et al. (2002) Mesenteric cysts. Acta Med Croatica 56(3): Mesenteric cysts and cystic mesenteric tumors are very rare abdominal growths. They may be localized all over the mesentery, from duodenum to rectum, however, they are mostly found in the ileum and right colon mesentery. There are several classifications of these formations, among which the one based on histopathologic features including 6 groups has been most commonly used: 1) cysts of lymphatic origin--lymphatic (hilar cysts) and lymphangiomas; 2) cysts of mesothelial origin--benign or malignant mesothelial cysts; 3) enteric cysts; 4) cysts of urogenital origin; 5) dermoid cysts; and 6) pseudocysts--infectious or traumatic etiology.

92 Review of literature PATIENTS AND METHODS:
Two adult female patients treated at the Department of Surgery, Zabok General Hospital, are presented. The diagnosis of mesenteric cyst was based on explorative laparotomy indicated for a cystic abdominal growth and characteristic palpatory finding, US and CT findings. In both patients, the cysts were successfully treated by total cystectomy. Histopathologic findings pointed to lymphatic cysts. Control US finding at 3 months postoperatively was normal in both patients. Kirurski, O. et al. (2002) Mesenteric cysts. Acta Med Croatica 56(3): PATIENTS AND METHODS: Two adult female patients treated at the Department of Surgery, Zabok General Hospital, are presented. The diagnosis of mesenteric cyst was based on explorative laparotomy indicated for a cystic abdominal growth and characteristic palpatory finding, US and CT findings. In both patients, the cysts were successfully treated by total cystectomy. Histopathologic findings pointed to lymphatic cysts. Control US finding at 3 months postoperatively was normal in both patients.

93 Review of literature DISCUSSION:
Cystic lymphangioma mostly occurs in the first decade of life, with a female predominance. It is usually accompanied by acute abdominal symptomatology. Lymphatic cysts occur later in life (1:100,000 in adults and 1:20,000 in children), also show female predominance, and as a rule are asymptomatic. A mesenteric cyst, especially lymphatic, should be suspected in the presence of painless abdominal tumor, with occasionally painful abdominal pressure, normal laboratory findings, and good general condition in a female patient. Kirurski, O. et al. (2002) Mesenteric cysts. Acta Med Croatica 56(3): Cystic lymphangioma mostly occurs in the first decade of life, with a female predominance. It is usually accompanied by acute abdominal symptomatology. Lymphatic cysts occur later in life (1:100,000 in adults and 1:20,000 in children), also show female predominance, and as a rule are asymptomatic. A mesenteric cyst, especially lymphatic, should be suspected in the presence of painless abdominal tumor, with occasionally painful abdominal pressure, normal laboratory findings, and good general condition in a female patient.

94 Review of literature DISCUSSION:
cystic mesenteric tumor is mostly used to refer to cystic lymphangiomas and lymphatic cysts Cystic lymphangiomas: smooth muscle tissue is found, with endothelial lining towards the cavity. The wall of hilar mesenteric cysts does not contain smooth muscle tissue, however, they also show endothelial lining towards the cavity. Exact differentiation between these two entities is necessary for the disease prognosis. Kirurski, O. et al. (2002) Mesenteric cysts. Acta Med Croatica 56(3): The term of cystic mesenteric tumor is mostly used to refer to cystic lymphangiomas and lymphatic cysts. Cystic lymphangiomas: smooth muscle tissue is found, with endothelial lining towards the cavity. The wall of hilar mesenteric cysts does not contain smooth muscle tissue, however, they also show endothelial lining towards the cavity. Exact differentiation between these two entities is necessary for the disease prognosis.

95 Review of literature DISCUSSION:
Lymphangiomas are prone to recurrence and infiltrating growth. The diagnosis should be made by use of all standard methods of abdominal tumor diagnosis, with ultrasonography (US) and computed tomography (CT), and especially nuclear magnetic resonance providing most information of the growth size and localization. Total cystectomy is the therapeutic method of choice. Open method has been preferred, although reports on successful cystectomy by the laparoscopic method have already appeared in the literature. Kirurski, O. et al. (2002) Mesenteric cysts. Acta Med Croatica 56(3): DISCUSSION: Lymphangiomas are prone to recurrence and infiltrating growth. The diagnosis should be made by use of all standard methods of abdominal tumor diagnosis, with ultrasonography (US) and computed tomography (CT), and especially nuclear magnetic resonance providing most information of the growth size and localization. Total cystectomy is the therapeutic method of choice. Open method has been preferred, although reports on successful cystectomy by the laparoscopic method have already appeared in the literature.

96 Review of literature CONCLUSION:
Intraoperative differentiation between lymphatic cyst and lymphangioma is of utmost importance, and can only be achieved by histopathologic examination of the cyst wall. If intraoperative biopsy cannot be performed or the finding is uncertain, each cyst should be extirpated in toto due to the above mentioned risk associated with cystic lymphangioma. Kirurski, O. et al. (2002) Mesenteric cysts. Acta Med Croatica 56(3):

97 Pre-natal Surveillance
With the widespread use of prenatal ultrasonography, mesenteric and omental cysts are being diagnosed in utero. No role for treating these cysts in utero is recognized. If cysts are discovered prenatally, intervention during early infancy is indicated to prevent potential complications such as obstruction and intestinal volvulus. With the widespread use of prenatal ultrasonography, mesenteric and omental cysts are being diagnosed in utero.[22] No role for treating these cysts in utero is recognized. If cysts are discovered prenatally, intervention during early infancy is indicated to prevent potential complications such as obstruction and intestinal volvulus. Kirurski, O. et al. (2002) Mesenteric cysts. Acta Med Croatica 56(3):

98 Final Diagnosis Mesenteric and Omental Cyst (Hemangiolymphangioma)
S/P Excision of Cyst and Marsupialization of Mesenteric Cyst (7/8/13), Chylous ascites, resolved No wasting, No stunting

99 Summary 1-month old female from Samar
Diagnosed in utero with ascites vs right pelvoabdominal cystic mass confirmed by ff up UTZ and CT scan during neonatal period Tumor markers were monitored On X-lap, has mesenteric and omental cyst with chylous ascites Underwent excision and marsupialization of respective cysts Tissue biopsy of Hemangiolymphangioma

100 Update Thank you and Good day!


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