Exacerbation of a Maternal Hiatus Hernia in Early Pregnancy Presenting with Symptoms of Hyperemesis Gravidarum: Case Report and Review of the Literature

Arch Gynecol Obstet (2011) 283:409–414 DOI 10.1007/s00404-010-1719-3

MATERNO-FETAL MEDICINE

Exacerbation of a maternal hiatus hernia in early pregnancy presenting with symptoms of hyperemesis gravidarum: case report and review of the literature

Lukas Schwentner • Christine Wulff • Rolf Kreienberg • Daniel Herr

Received: 6 July 2010 / Accepted: 13 October 2010 / Published online: 2 November 2010 Ó Springer-Verlag 2010

Abstract maternal diaphragmatic hernia. Usually, maternal dia- Case report We report on a 30-year old woman pre- phragmatic hernias become clinically obvious in advanced senting with symptoms of hyperemesis gravidarum and stage of pregnancy, in contrast hyperemesis gravidarum is subsequent vomiting at the end of the first trimester normally occurring in the first trimester and is usually self- (12 ? 0 weeks of gestation). The patient was initially limiting. Guiding symptoms for hyperemesis gravidarum presented with nausea and vomiting, without any signs or are nausea and vomiting, but these clinical findings can symptoms of intra-abdominal disorders. On the 2nd day, also be unspecific symptoms of a maternal diaphragmatic symptoms became worse and she complained right sided hernia. Therefore, especially mild variants of maternal upper abdominal pain, therefore abdominal ultrasound was diaphragmatic hernias in early pregnancy can be misdiag- performed, showing no remarkable findings, explaining the nosed as hyperemesis gravidarum. Nevertheless, the rising disorder. Clinical symptoms increased and the patient intra-abdominal pressure while vomiting obviously can complained suddenly severe dyspnoea and intractable trigger exacerbation of a pre-existing maternal diaphrag- cough. Therefore, immediately an X-ray examination of matic hernia. We therefore speculate that there could be an the thorax was performed showing a severe left sided association between physiological changes in early preg- diaphragmatic hiatus hernia with consecutive displaced nancy, for example in gastric motility, and the exacerbation stomach into the thoracic cavity, making immediate sur- of the pre-existing maternal hiatus hernia. gical intervention necessary. Conclusion Hence a diaphragmatic hernia should always Discussion Diaphragmatic hernias complicating preg- be excluded, if symptoms of nausea and vomiting are nancy are a rare event, they normally occur in later periods intractable, mediastinal shift with dyspnoea occurs, failure of pregnancy due to the rising intra-abdominal pressure of conservative treatment especially after 20th week of mainly caused by the enlargement of the uterus. Also gestation and in late onset of assumed hyperemesis maternal diaphragmatic hernias during pregnancy are gravidarum. usually associated with minor complains. However, they can be life-threatening, due to mediastinal shift and cardio- Keywords Hyperemesis gravidarum Á respiratory failure. The majority of maternal diaphragmatic Maternal diaphragmatic hernia Á Pregnancy hernias complicating pregnancies occur in antenatal period, most of them in the third trimester. More than 90% of maternal diaphragmatic hernias complicating pregnancy Introduction are localized on the left side of the maternal diaphragma. We present a case of an early onset life-threatening Mild symptoms of maternal diaphragmatic hernia can imitate hyperemesis gravidarum and therefore especially cases without more obvious symptoms are often misdiag- & L. Schwentner ( ) Á C. Wulff Á R. Kreienberg Á D. Herr nosed as hyperemesis gravidarum. In most of the cases Department of Gynecology and Obstetrics, University Ulm, Prittwitzstraße 43, 89075 Ulm, Germany maternal diaphragmatic hernias become clinically obvious e-mail: [email protected] in the third trimester, when intra-abdominal pressure is 123 410 Arch Gynecol Obstet (2011) 283:409–414

rising because of the enlarging uterus. In contrast to this, with markedly reduced air entry into the left lung. SpO2 nausea and vomiting normally occurs in up to 80% of was 84% and after receiving 4 l O2 blood oxygen increased pregnant women the first trimester. We are presenting a up to 94%. The blood samples taken revealed rising case of a clinically severe diaphragmatic hernia at the end infection parameters (CRP 231.1 mg/l) and additively of the first trimester, making immediate surgical interven- elevated brain natriuretic peptide (BNP 485.6 pg/ml) tion necessary. indicating a cardiac insufficiency. Pancreatic-lipase (pancreatic-lipase 98 U/l) was still elevated; all other parameters were within the physiological limits. Case report Because of the sudden dyspnoea and the other clinical findings, the most important differential diagnose was the A 30-year old woman classified as gravida 1 and nullipara, exacerbation of a maternal diaphragmatic hernia and she 12 ? 0 weeks of gestation, with clinical symptoms of therefore underwent an X-ray examination of the thorax, hyperemesis gravidarum presented at our department. She showing a collapsed left lung with the stomach displaced described regular vomiting up to 6 times a day; in addition into the thoracic cavity. She was therefore immediately food intake was impossible. She also showed normal urine transferred to ICU and was operated 8 h after diagnosis. parameters without indication of an urinary tract infection, Intra-operatively, a left sided maternal diaphragmatic her- except of a ketonurie. On clinical examination she showed nia with a dislocated stomach into the thoracic cavity was no signs or symptoms of intra-abdominal disorders and no diagnosed. (Fig. 1) The stomach, showing three small symptoms of dyspnoea. The transvaginal ultrasound ischemic serosa defects, was relocated and the hernia was revealed a vital and normally advanced pregnancy with a surgically closed. She also received a left sided thorax crown-rump length (CRL) of 51 mm. The medical history drainage to allow expansion and ventilation of the left of the patient included a hereditary double kidney on the sided lung. Post-operatively, she was transferred back to right side. She received boluses for volume resuscitation as ICU. Directly after operation, the elevated infection well as antiemetic agents. On the next day, she reported parameters reached a peak of CRP 351.1 mg/l and leuko- slight right sided upper abdominal pain. In clinical exam- cytes of 14.6 Giga/l. In the control-gastroscopy, she pre- ination, the abdomen was not rigid without pressure- sented an ischemic gastritis and was therefore nourished induced pain. We took blood samples showing elevated parenterally for 3 days. In the post-operative X-rays of the infection parameters (CRP 152.5 mg/l/Leukocytes 21.5 thorax, the pulmonary situation improved dramatically, as Giga/l) and slightly elevated pancreatic-enzymes (pancreatic- well as clinical symptoms (Fig. 2). Post-operatively, she lipase 101 U/l). Because of the symptoms and laboratory was instructed for regular CPAP to prevent insufficient parameters differential diagnoses were hepatobiliary dis- ventilation. Furthermore, she received antibiotic prophy- orders, gastric or intestinal perforation and pancreatitis. laxis (Ceftriaxone 1.5 g 1-0-1 i.v.) and Pantoprazol (40 mg Therefore, ultrasound examination of the abdomen was 1-0-0). She recovered well after operation, but alimentation performed. However, examination on the left side upper was still a problem, because of regular vomiting after abdomen was unremarkable. During the night, the patient operation which was explained due to the ischemic gas- suddenly developed excessive vomiting and cough. She tritis. Fifth day after operation, a slow nutrition assembly then presented suddenly with dyspnoea and left sided was possible. However, the patient developed an infection thoracic pain. On clinical examination, she was pale, had a complication with a Candida infection of the thorax remarkable tachycardia of 110 beats/min, a mild hypoten- drainage and she therefore received Fluconazole (200 mg sion (RR 105/72 mmHg) and a guarded and rigid abdomen 1-0-0) for 3 weeks after diagnosis of the mycosis. Infection

Fig. 1 Emergency X-ray examination of the thorax showing the dislocated stomach on the left side in the thorax (pre-operatively) (a) a/p projection (b) side projection

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Fig. 2 (a) X-ray examination on the ﬁrst day after operation showing still a compromised lung on the left side (a/p projection). (b) X-ray examination on the 8th day after operation showing good recovering of the left sided lung (post-operatively). (c) X-ray examination on the 2nd day after operation (a/p projection).

parameters were decreasing constantly, before discharge of morning sickness protects the embryo and the pregnant hospital CRP was 194.1 and leukocytes 13.1 Giga/l. She woman by causing vomiting to subsequently avoid foods was discharged from hospital after 19 days (14 ? 5 weeks that contain teratogenic and abortifacient substances [6]. of gestation). The follow-up of the patient was unremark- The incidence of hyperemesis gravidarum is estimated able, also regularly blood investigations showed decreasing around 0.5% of live births [2, 3]. The incidence is varying infection parameters, being first within normal limits in literature between 0.3 and 1.5% of live births and some 24 days after operation (27 days after admission to hospi- authors reported variances caused by ethnical background tal). The ultrasound second trimester screening for fetal and culture [4]. Before applying intravenous supply to the anatomy anomalies showed a normal fetus with estimated patients suffering from hyperemesis gravidarum the mor- 405 g. (BPD 49.8/FOD 64.9/CM 3.8/TCD 22.6/NB 6.9/ tality of this disease was 159 deaths per one million live ATD 51.5/ASD 51.6/FL 35.5/HL 34.6 mm). All anatomic births in Great Britain [8]. Even now dehydration and features displayed normal in this ultrasound investigation. electrolyte disorders are relatively common seen compli- Because of the maternal diaphragmatic hernia complicating cations [7]. Although several studies presenting theories of this pregnancy, a cesarian section was performed with the molecular pathogenesis in hyperemesis gravidarum, 38 ? 5 weeks of gestation without any complications until now there is no consensus concerning the pathogen- (2.960 g, Apgar 9/10/10, ph 7.34/7.41, 50 cm). esis of this disease. Nutrition deficiency, fluid deficiency and electrolyte imbalance can require hospital admission for intravenous substitution and antiemetic therapy [5]. Review Hyperemesis gravidarum is most prevalent during, but certainly not limited to, the first trimester of pregnancy [7] Hyperemesis gravidarum is a condition of intractable when both the corpus luteum and the placenta are pro- vomiting during pregnancy leading to fluid, electrolyte, ducing gestational hormones and the body is adapting to acid–base and nutrition deficiency. Up to 80% of women the pregnant state. This disease typically occurs between experience some form of nausea and vomiting [1]. Since 4th and 10th week of gestation and normally resolves majority of pregnant women experience nausea and vom- by the latest 20th week of gestation. In literature also iting during their pregnancy, a functional role of morning cases with several severe complications of hyperemesis sickness is considered by some authors. It is speculated that gravidarum have been published, reporting wernicke 123 412 Arch Gynecol Obstet (2011) 283:409–414 encephalopathy [9], central pontine myelinolysis [10], onset of symptoms to surgery and mortality as well as vasospasm of cerebral arteries [11], peripheral neuropathy length of hospital stay [25]. [12], and coagulopathy due to vitamin K deficiency [13, Diaphragmatic hernias complicating pregnancy are a 14]. Also mechanical complications of hyperemesis grav- rare condition. About 37 cases of this complication in idarum have been reported such as esophageal rupture [15] pregnancy have been published in literature dating back to with consecutive pneumomediastinum or rupture of a 1928 [20]. The mean age of presentation was 28 years and choledochus cyst [16]. In most patients intravenous sub- 67% of the patients were multiparous [20]. 52% of the stitution of fluid and electrolyte is sufficient to relieve patients presented in the antenatal period, whereas most of symptoms and prevent serious complications. If this ther- the cases occurred in the third trimester. The most common apy is not sufficient antiemetic drugs should be applied, symptom of presentation was upper abdominal pain and sometimes all forms of conventional treatment fail and full vomiting occurring in 61% of the cases [20]. 32% of the parenteral nutrition has to be applied to prevent the patients patients showed mediastinal shift and in only 6% respira- from severe complications [17]. Nevertheless, the maternal tory failure was seen [20]. The majority of diaphragmatic condition can become life-threatening and termination of hernias was left sided presenting in 94% of the cases. the pregnancy can be required. It is estimated that up to 2% Operative repair was attempted in 30% during antenatal of pregnancies complicated by hyperemesis gravidarum are period and 50% during postnatal period. The rest of the terminated [18][19]. cases were managed conservatively. In only 30% of the Maternal diaphragmatic hernia complicating pregnancy cases reported, delivery was by caesarean section [20]. is a very rare, but life-threatening disease. Diaphragmatic Recently, a case of 29-year old women presenting in third hernias are a very common cause of minor complaints in pregnancy at 27th week of gestation with diaphragmatic pregnancy, the more rare congenital and traumatic dia- hernia was published, presenting the first case of a simul- phragmatic defects may predispose to strangulation and taneous maternal and fetal diaphragmatic hernia. In addi- incarceration of the abdominal viscera within the thorax as tion, recently Ting [27] was reporting a case of a 42-year the uterus enlarges [20]. Diaphragmatic hernias are clas- old woman with the diagnosis of a diaphragmatic hernia at sified as congenital, hiatus and traumatic. Congenital 19 weeks of gestation. hernias usually present in the neonatal period with a large variability of symptoms. These congenital hernias normally perforate the diaphragma through the foramen of Discussion Bochdalek or less frequently the foramen of Morgagni. These congenital hernias occur in 1 of 2,500 live births This case is presenting an extremely rare event of an early [21] and are normally located on the left side (84%). Right onset diaphragmatic hernia during pregnancy. The patient sided congenital hernias occur in approximately 14% of was presenting already at the end of the first trimester. the cases and bilateral hernias in 2%, respectively. Both Normally, this complication of pregnancy becomes clini- right sided and bilateral hernias are associated with a cally obvious in a later period of pregnancy. The circum- worse prognosis [22]. It is always difficult to differ the stance of an early onset of symptoms can make diagnosis aetiology of diaphragmatic hernias when they become more complicated, because 80% of the women complain clinically obvious [23]. Hiatus hernias present in adult life, nausea and vomiting during pregnancy. Especially, the due to mechanical forces and tissue degeneration are ordinary hyperemesis gravidarum, which is occurring most probable etiological factors [26]. There are congenital often in this period of time, is normally self-limiting until hernias, which are not presenting clinically although the 12 weeks of gestation. Therefore, cases without symptoms structural deficiency is existing since perinatal period, of mediastinal shift or dyspnoea are difficult to diagnose. therefore congenital factors can never be ruled out com- Also because of the reason that X-ray examinations in pletely. Hiatus hernias and its complications also exist in pregnant women need strict medical indication, especially the pediatric literature [24]. Symptoms reach from inci- in the first trimester. The threshold doses for fetal death, dential findings to a life-threatening disease. These cata- malformations and mental retardation which are deter- strophic symptoms are mainly caused by compression of ministic effects, are reported to be 100–200 mGy or higher the intra-thoracic organs. In fatal cases, mediastinal shift [30]. The relative risk for childhood cancer due to a radi- with consecutive obstructive shock can be caused by a ation at an absorbent dose of 10 mGy during embryonic/ severe diaphragmatic hernia. The diagnosis of a dia- fetal development has been estimated at 1.4-fold higher phragmatic hernia is based on the clinical examination and [30]. The fetal load of X-ray beam, if the fetus is not X-ray examination of the thorax. The treatment of dia- irradiated directly, is very low with doses less than phragmatic hernias in adults is based on surgery, either per 0.01 mGy [30]. An anterior–posterior X-ray study of the laparascopy or laparatomy. There is a direct relation from complete abdomen in pregnancy is associated with an 123 Arch Gynecol Obstet (2011) 283:409–414 413 estimated mean of 7.5 mGy radiation dose for the fetus and 9. Michel ME, Alanio E, Bois E, Gavillon N, Graesslin O (2010) is therefore definitive below 100 mGy [31]. Although, the Wernicke encephalopathy complicating hyperemesis gravidarum: a case report. Eur J Obstet Gynecol Reprod Biol 149(1):118–119 fetal absorbent doses are quite low, strict medical indica- 10. Valiulis B, Kelley RE, Hardjasudarma M, London S (2001) tion is necessary for the use of X-ray diagnostic in Magnetic resonance imaging detection of a lesion compatible pregnancy. with central pontine myelinolysis in pregnant patient with Therefore a rare differential diagnose women with recurrent vomiting and confusion. J Neuroimaging 11(4):441– 443 intractable vomiting, especially in later periods of preg- 11. Kanayama N, Khatun S, Belayet HM, Yamashita M, Yonezawa nancy, is a diaphragmatic hernia. But, also women suf- M, Kobayashi T, Terao T (1998) Vasospasm of cerebral arteries fering from allegedly hyperemesis gravidarum in the first in hyperemesis gravidarum. Gynecol Obstet Invest 46(2): trimester, who are suddenly showing symptoms of dysp- 139–141 12. 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