DISCLAIMER: Results of database and or Internet searches are subject to the limitations of both the database(s) searched, and by your search request. It is the responsibility of the requestor to determine the accuracy, validity and interpretation of the results.

Date: 19 November 2018 Sources Searched: Embase, Medline

Primary Intestinal Lymphangiectasia (Waldmann’s Disease) and Pregnancy

See full search strategy

1. Exacerbation of primary intestinal lymphangiectasia during late pregnancy and recovery after delivery: A case report and literature review. Author(s): Lu, Jianyang; Zhai, Hongbo Source: Medicine; Sep 2017; vol. 96 (no. 35); p. e7928 Publication Date: Sep 2017 Publication Type(s): Case Reports Journal Article Review PubMedID: 28858117 Available at Medicine - from Europe PubMed Central - Open Access Available at Medicine - from Ovid (Journals @ Ovid) - Remote Access Abstract:RATIONALEPrimary intestinal lymphangiectasia (PIL) is a rare disease characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen. Main clinical features include intermittent , . Scattered case reports suggested that PIL is compatible to pregnancy, but with increased complications.PATIENT CONCERNSA 34-year-old woman with endoscopically diagnosed PIL presented to antenatal our clinic at 10 weeks into gestation. She reported strict adherence to low-/high-protein diet with medium-chain triglycerides (MCTs) supplementation. She was general well except for moderate and hypoalbuminemia. At 33 weeks, she developed diarrhea, nausea, and vomiting, with decreased fetal movements. One week later, she had an asthma attack. Nonstress test showed frequent variable deceleration.DIAGNOSESThe diagnosis of PIL was established endoscopically 8 years earlier.INTERVENTIONSHypoalbuminemia was corrected with intravenous albumin administration. She also received therapy to promote fetal lung maturation in anticipation to early termination of the pregnancy.OUTCOMESA cesarean section was carried out at 34 weeks due to fetal distress. The baby girl was apparently healthy: weighing 2160 g, with an Apgar score of 9 at both 1 and 5 minutes. Symptoms dissipated rapidly after the delivery. The last follow-up visit at 15 months was unremarkable for both the mother and infant.LESSONSPIL could be compatible with pregnancy, but requires strict adherence to dietary treatment, proper management of the symptoms (e.g., hypoalbuminemia), particularly during late gestation. Database: Medline

2. Severe early-onset intrahepatic cholestasis of pregnancy-management dilemmas Author(s): Kotlinska M.; Hingorani J. Source: BJOG: An International Journal of Obstetrics and Gynaecology; Mar 2017; vol. 124 ; p. 147- 148 Publication Date: Mar 2017 Publication Type(s): Conference Abstract Available at BJOG: An International Journal of Obstetrics & Gynaecology - from Wiley Online Library Science , Technology and Medicine Collection 2017 Available at BJOG: An International Journal of Obstetrics & Gynaecology - from Unpaywall Abstract:Introduction Intrahepatic cholestasis of pregnancy (ICP) is a disorder of defective bile acid transport that results in systemic accumulation of bile acids, and typically presents with intense pruritus. It adversely affects maternal wellbeing and fetal outcomes. Commonly it occurs in the third trimester with about 80% of cases presenting after 30 weeks of gestation. However, it may develop as early as first trimester with just a few case reports in the literature. Methods We present a case of severe ICP that was diagnosed in early second trimester, did not respond to treatment with ursodeoxycholic acid (UDCA) and in consequence required delivery before 34 weeks of gestation. Results A 20-year-old nulliparous Caucasian woman was investigated with liver function tests and bile acids due to severe pruritus that started at 12 weeks of gestation. There were no signs of dermatological condition or jaundice on clinical examination. The only remarkable past medical history was intestinal lymphangiectasia and in childhood. At 18 weeks of gestation bile acids were 88 mumol/L with unremarkable liver function tests. The ultrasound scan of the liver was normal and so was the hepatitis screen. Patient was started on UDCA, topical emollients, antihistamines and given a course of K. Despite the treatment, the bile acids continued to rise reaching maximum level of 211 mumol/L. Subsequently, she was tried on rifampicin 150 mg twice daily with some effect. The treatment was temporarily stopped, due to fetal dysrhythmia. Considering that raised levels of bile acids are arrhythmogenic and likely cause of fetal ectopic beats the rifampicin was restarted. Despite the drop in bile acids to 123 mumol/L the patient's symptoms continued to be severe and significantly affected her mental state. Fetal surveillance (ultrasound scan, cardiotocogram) remained normal, but in view of the debilitating symptoms the patient underwent elective caesarean section at 32+ weeks. Conclusion Typical treatment for ICP consists of topical emollients, antihistamines, UDCA and possibly . Cholestyramine that has been used by some authors as indicated by the literature, has not been subjected to randomised trials and is not in clinical use, as suggested by RCOG. Rifampicin is not licensed for treatment for ICP but it has been used with very good results in other liver pathologies, including gallstones and primary biliary cirrhosis. In these cases, treatment with rifampicin resulted in significant decrease in serum levels of transaminases and total bile acids, as well as an improvement in pruritus. Database: EMBASE

3. Pregnancy and Waldmann disease. Author(s): Tourlakis, D; Hatziveis, K; Spiliopoulos, E Source: La Clinica terapeutica; 2008; vol. 159 (no. 3); p. 173-174 Publication Date: 2008 Publication Type(s): Case Reports Journal Article PubMedID: 18594747 Abstract:We present a case of a woman with intestinal lymphangiectasia and pregnancy. She had been treated with albumin transfusions beginning from the age of 2 years. No major complications were caused to the pregnancy. During pregnancy she was given albumin transfusions at regular intervals, while measuring total proteins, albumin and concentrations in the serum. Due to the increasing needs in proteins as the pregnancy went on, the interval between each transfusion went decreasing. Finally, at 39 weeks of gestation she gave birth by cesarean section due to placenta previa to a healthy female fetus. A worsening of hypoproteinemia and edema was noted during puerperium and lactation. Weaning improved her clinical status. The baby was followed by pediatricians and found her in good health. Summing up, normal pregnancy is compatible with the condition. A strict maternal and fetal care during pregnancy and puerperium is recommended. Database: Medline

4. Waldmann disease and pregnancy Author(s): Sanchez-Sanchez M.M.; Garcia-Robles R.M.; Leiva A.; Moran E.; Tejerizo-Garcia A.; Tejerizo-Lopez L.C. Source: Clinica e Investigacion en Ginecologia y Obstetricia; Dec 2002; vol. 29 (no. 10); p. 370-374 Publication Date: Dec 2002 Publication Type(s): Article Abstract:Waldmann disease, also called intestinal lymphangiectasis, is a protein-loosing enteropathy caused by anomalous intestinal lymphatic canals. The condition is transmitted by autosonal dominant inherance. Diet therapy including medium chain triglycerides improves the disease course. The condition occurs in early chilhood. We report a case observed in a pregnancy woman with intestinal lymphangiectasis. Shee delivered a full-term infant and experienced no major complications. Database: EMBASE

5. Primary intestinal lymphangiectasia, nephrotic syndrome and clomid-induced triplet pregnancy. Author(s): Al-Mushaikih, A A Source: Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology Association; Sep 2001; vol. 7 (no. 3); p. 116-118 Publication Date: Sep 2001 Publication Type(s): Journal Article PubMedID: 19861780 Available at Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology Association - from PubMed Database: Medline

6. Waldman disease and pregnancy Author(s): Quemere M.P.; Descargues G.; Verspyck E.; Marpeau L. Source: Journal de Gynecologie Obstetrique et Biologie de la Reproduction; 2000; vol. 29 (no. 5); p. 517-519 Publication Date: 2000 Publication Type(s): Article PubMedID: 11011282 Abstract:Waldman disease, also called intestinal lymphangiectasis, is a protein-loosing enteropathy caused by anomalous intestinal lymphatic canals. The condition is transmitted by autosomal dominant inherance. Diet therapy including medium chain triglycerides improves the disease course. The condition occurs in early childhood. We report a case observed in a pregnant woman with intestinal lymphangiectasis. She delivered a full-term infant and experienced no major complications. Database: EMBASE

7.Pregnancy and intestinal lymphangiectasia. Management of foetal growth Author(s): Lizee B.; Ginies J.L.; Grimal I.; Chaboussou J.P.; Bouderlique C. Source: Revue Francaise de Gynecologie et d'Obstetrique; 1997; vol. 92 (no. 4); p. 263-265 Publication Date: 1997 Publication Type(s): Article Abstract:Pregnancy is a rare event in a woman with intestinal lymphangiectasia. In this clinical context, foetal growth is compromised by the insufficient placental blood supply secondary to maternal hypoalbuminaemia. The authors report the case of a woman with intestinal lymphangiectasia who became pregnant at the age of 33 years. She presented with generalized oedema, hypoproteinaemia and lymphocytopenia. Management of the disease consisted of a low- fat, high-protein diet, with vitamin and iron supplements and repeated albumin infusions. This treatment restored a normal blood volume, measured regularly by Evans blue test. Postal growth, measured ultrasonographically, was normal. Transient foetal was observed at the 33rd week of amenorrhoea and an episode of maternal hypovolaemia at the 35th week was responsible for transient abnormalities of Doppler indices. The child was born at 37 and a half weeks and weighed 2900 g. Clinical examination was normal. The search for ascites and gastrointestinal protein leak was negative. This case shows that rigorous management of intestinal lymphangiectasia during pregnancy can allow term delivery of a normal birth weight infant. Ultrasound surveillance can reveal signs suggestive of foetal disease, but there is no fully reliable antenatal diagnosis. The presence of a healthy neonate when the disease occurs in a familial context does not formally excluse the diagnosis, as the disease usually only present after a free interval of several years. Database: EMBASE

8. Intestinal lymphangiectasia: presentation in pregnancy and association with herpes zoster and alopecia. Author(s): Ghoshal, U C; Gupta, R; Aggarwal, R; Puri, A S; Naik, S R Source: Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology; Oct 1997; vol. 16 (no. 4); p. 153-154 Publication Date: Oct 1997 Publication Type(s): Case Reports Journal Article PubMedID: 9357190 Abstract:We report a woman with intestinal lymphangiectasia whose symptoms were wrongly attributed to pregnancy; the diagnosis was made in the postpartum period. She also developed alopecia and herpes zoster. Database: Medline

9. [Conception and pregnancy without complications in parenteral home nutrition]. Author(s): Voigt, H J; Sailer, D; Kolb, S; Frobenius, W Source: Zeitschrift fur Geburtshilfe und Perinatologie; 1989; vol. 193 (no. 4); p. 198-200 Publication Date: 1989 Publication Type(s): Case Reports English Abstract Journal Article PubMedID: 2508340 Abstract:A case is reported of conception and successful completion of a pregnancy in a 31 year old woman maintained on home parenteral nutrition while suffering from a primary distension of the intestinal lymphatic vessels and intestinal loss of protein. A healthy, 3400 g female infant was born near term by cesarean section. This case confirms the few so far published experiences showing that conception and maintenance of the pregnancy under total parenteral nutrition needs no or just a slight adaptation of the regimen. Database: Medline

10. Case report. Pregnancy and intestinal lymphangiectasis (familial neonatal hypoproteinaemia) Author(s): Liu D.T.Y.; Sherman A.M. Source: Australian and New Zealand Journal of Obstetrics and Gynaecology; 1980; vol. 20 (no. 1); p. 58-59 Publication Date: 1980 Publication Type(s): Article PubMedID: 6930252 Available at The Australian & New Zealand journal of obstetrics & gynaecology - from Patricia Bowen Library & Knowledge Service West Middlesex University Hospital NHS Trust (lib302631) Local Print Collection [location] : Patricia Bowen Library and Knowledge Service West Middlesex university Hospital. Abstract:A patient with a history of familial intestinal lymphangiectasis and exudative enteropathy was observed during 2 consecutive pregnancies. Normal pregnancy is compatible with the condition and pregnancy need not aggravate the disease. Database: EMBASE

Strategy 543803

# Database Search term Results

1 Medline ("Primary intestinal 132 lymphangiectasia").ti,ab

2 Medline ("Waldmann* disease").ti,ab 2

3 Medline exp "LYMPHANGIECTASIS, 552 INTESTINAL"/

4 Medline (1 OR 2 OR 3) 584

5 Medline (PREGNAN*).ti,ab 453596

6 Medline exp PREGNANCY/ 845618

7 Medline (5 OR 6) 942791

8 Medline (4 AND 7) 13

9 EMBASE ("Primary intestinal 163 lymphangiectasia").ti,ab

10 EMBASE ("Waldmann* disease").ti,ab 37

11 EMBASE exp "LYMPHANGIECTASIS, 775 INTESTINAL"/

12 EMBASE (9 OR 10 OR 11) 792

13 EMBASE (pregnan*).ti,ab 0

14 EMBASE exp PREGNANCY/ 614189

15 EMBASE (13 OR 14) 819260

16 EMBASE (12 AND 15) 16

17 EMBASE ((lymphangiectasis OR 645 lymphangiectasia) ADJ2 intestinal).ti,ab 18 EMBASE (15 AND 17) 16

19 Medline ((lymphangiectasis OR 577 lymphangiectasia) ADJ2 intestinal).ti,ab

20 Medline (7 AND 19) 16