Case Report A 14-Year-Old Girl with Recurrent Vulvar Abscess Richina Bicette MD 1, Gayathri Tenjarla MD 2, Subra Kugathasan MD 2, Adina Alazraki MD 3, Lisa Haddad MD, MS, MPH 4,* 1
Emory University School of Medicine, Atlanta, Georgia Department of Pediatric Gastroenterology, Emory University, Atlanta, Georgia 3 Department of Pediatric Radiology, Emory University, Atlanta, Georgia 4 Department of Gynecology and Obstetrics, Emory University, Atlanta, Georgia 2
a b s t r a c t Background: Inflammatory bowel disease is a relatively common condition that may present in a myriad of fashions, from the more frequently seen symptoms of diarrhea and abdominal pain to the less likely presentations with fistulas and abscesses. Case: A 14-year-old female with a presumed diagnosis of a Bartholin's gland cyst was treated for over 1 year with both medical and surgical interventions without her symptoms ever completely resolving. It was later found that these recurring vulvar abscesses were a manifestation of Crohn's disease. Conclusion: While the patient's history and physical are both very important in determining cause for pathology, we must also realize the importance of re-examining and broadening our differential diagnosis when faced with a patient who has failed multiple avenues of care. Key Words: Inflammatory bowel disease, Crohn's disease, Vulvar abscess
Introduction
Vulvar abscesses are well-described gynecologic conditions typically associated with simple infections that develop most commonly within Bartholin or Skene glands. They have also been described originating from skin and hair follicles, or wounds in the vulvar region. However, in rare situations, the abscess may be an initial presenting symptom of inflammatory bowel disease (IBD). Ulcerative colitis (UC) and Crohn's disease are the two forms of IBD. Key features of UC includes diffuse mucosal inflammation that extends proximally from the rectum and is restricted to the colon while Crohn's disease is characterized by chronic, relapsing inflammation frequently associated with perianal abcesses and fistulas. This case report describes an adolescent patient with recurring vulvar abscesses treated surgically and with prolonged courses of antibiotics. These abscesses persisted for over 1 year without resolution, at which time further evaluation ultimately demonstrated inflammatory bowel disease. Case
A 14-year-old virginal female was referred from an outside facility with a 16-month history of a recurring vulvar abscess. These symptoms began at age 12. Of note, her medical history was also significant for difficulty with
The authors indicate no conflicts of interest. * Address correspondence to: Lisa Haddad, MD, MS, MPH, Department of Gynecology and Obstetrics, Emory University School of Medicine, 49 Jesse Hill Jr. Drive, Atlanta, Georgia 30303; Phone: 404-778-1385; fax: 404-778-1382 E-mail address: [email protected] (L. Haddad).
feeding as an infant requiring G-tube feeds for a few months, as well as a history of chronic diarrhea for several years. The diarrhea was attributed to lactose intolerance, although it improved minimally on a lactose-free diet and no further work-up was pursued. Otherwise there was no significant past medical or surgical history; menarche at 9 with normal monthly menses was reported. The vulvar abscess was evaluated initially in the emergency room in February 2011 for what was believed to be a Bartholin's cyst. The cyst was drained in the emergency department, however over the following months, it continued to enlarge and cause the patient discomfort. Pain was severe such that she was unable to sit down comfortably at school. She was subsequently taken to the operating room twice to have the cyst drained and surgically resected, first in May 2011 and again in July 2011 after reaccumulation. A culture from a sample obtained at her second surgery grew Acinetobacter iwoffii. Consequently, she was placed on trimethoprim/sulfamethoxazole (TMP/SMX). She remained on antibiotics for ten months, without ever having full remission of the abscess. The patient reported periodic improvement, but her pain and vulvar swelling completely resolved. During this period, the cyst reaccumulated and spontaneously drained several times, which caused significant distress for the patient. After failure of both surgical and medical treatment, she was referred to our tertiary care facility for further evaluation. Upon presenting to our tertiary care facility, a limited physical examination demonstrated right labial swelling with induration extending across the length of the labia majora. There was no discrete mass palpated. Additionally, vesicular inflamed lesions were noted below the labial swelling and on the left lower labia across from the right
1083-3188/$ - see front matter Ó 2013 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpag.2013.07.010
2
R. Bicette et al. / J Pediatr Adolesc Gynecol xxx (2013) 1e4
Fig. 1. On presentation to our facility, prior to infliximab initiation. Note lesions inferior to right (R) and left (L) labiae.
sided lesion (Fig. 1). Given the patient's age, discomfort in the region, and virginal status, the physical evaluation was limited. There was no open lesion for culturing at that time. A magnetic resonance imaging (MRI) of the pelvis with and without contrast was obtained to further delineate the abscess. The MRI displayed a rim enhancing fluid collection within the right posterior perineum/buttock region consistent with an abscess, measured 2.2 cm 9 mm 4 cm, and was associated with multiple linear perianal fistula tracts (Fig. 2). An additional smaller abscess was noted
posteriorly in the right labia majora (Fig. 3). There were no definite inflammatory changes of bowel and her uterus and adnexae were normal. The fistula tracts seen on MRI raised suspicion for IBD, and the pediatric gastroenterology team was consulted on an outpatient basis. While awaiting the gastroenterology consult, it was decided by the primary team to discontinue TMP/SMX and initiate ciprofloxacin and metronidazole in order to broaden antibiotic coverage, which significantly improved her symptoms. However the patient continued to complain of a foul smelling vaginal discharge. She was admitted for further evaluation, at which time, serological evaluation was significant for mild iron deficiency anemia, while serum chemistries, ESR, CRP were all within normal limits. The Prometheus IBD sgi Diagnostic test (San Diego, CA), a panel that combines serologic, genetic and inflammatory markers, was performed in August 2012. The findings of increased antibodies for both anti-Saccharomyces cerevisiae antibody (ASCA) and outer membrane porin C (OmpC) were suggestive of a diagnosis of Crohn's Disease. She further underwent upper endoscopy and colonoscopy with biopsies, which were normal except for mild inactive gastritis. A diagnosis of Crohn's disease was made as a result of the colonoscopy based on multiple perianal fistula tracts, perineal abcesses and positive serologies. The antibiotics were discontinued and she began induction therapy with infliximab. Within two treatments during the induction phase, her fistulae stopped draining, there was remarkable improvement in her diarrhea and abdominal pain (Fig. 4) and she began gaining weight. She has had no recurrences since the initiating infliximab therapy. Comment
Here we present a case of a young girl with persistent vulvar symptoms for over one year, despite treatment, who was later determined to have an underlying inflammatory bowel disease as the etiology for her symptoms. Inflammatory bowel disease is a chronic inflammatory condition,
Fig. 2. Coronal T1 post contrast images. Arrows highlight the perianal fistulous tract leading to the right. Intense phlegmonous enhancement of the surrounding soft tissue in the right buttock.
R. Bicette et al. / J Pediatr Adolesc Gynecol xxx (2013) 1e4
3
Fig. 3. Axial fluid sensitive (T2) fat suppressed and T1 post contrast images at the level of the perineum. Arrows identify a transphincteric fistula at 5-6 o'clock leading to a large perianal abscess. Arrowhead shows thickening and enhancement of the right labia majora.
with the two major forms being Crohn's disease and Ulcerative Colitis. While both are idiopathic in nature, they are diagnostically defined by their clinical, pathologic, and endoscopic features.1 Usually unveiling itself in the second or third decade of life, Crohn's disease is a relapsing transmural inflammatory condition that can affect any area of the gastrointestinal tract, from the mouth to the anus.2 Although the development of complications such a strictures, fistulas, and abscesses are a known consequence of Crohn's disease, the initial clinical presentation is
Fig. 4. After second dose of infliximab; already demonstrating a decrease in labial swelling and lesions.
largely dependent on disease location and typically includes diarrhea, abdominal pain, fever, clinical signs of bowel obstruction, as well as passage of blood or mucus or both.3 At diagnosis, the disease is located in the terminal ileum in 47%, the colon in 28%, the ileocolon in 21%, and the upper gastrointestinal tract in 3%.3 Disease behavior is classified as non-stricturing and non-penetrating in 70% of patients, stricturing in 17%, and penetrating (fistulas, abscesses or both) in 13% of all patients at diagnosis.3 Our patient was initially assumed to have a Bartholin's gland abscess, but other causes were not considered at that time. Although uncommon, there have been case reports of similar abscesses occurring in infants and virginal females, thus a broader differential could have been considered and reduced the time for diagnosis. The location of this patient's abscess was also inconsistent with what would be considered a typical Bartholin's gland abscess, and more closely represented an external labial abscess. Further, her history of chronic diarrhea may have suggested an alternative GI origin for her symptoms. The culture finding of Acinetobacter lwoffii should have triggered a further work-up as it is an atypical organism in the vulvar region that is a part of the normal flora of the oropharynx and skin in approximately 25% of healthy individuals.4 The differential for a vulvar abscess is vast and quite broad, however, patient history and associated symptoms are imperative to narrow our differential and focus our initial work up. Another diagnostic consideration that was entertained when the patient presented to the tertiary care facility was hidradenitis suppurativa, since at that time she had multiple inflamed cystic lesions present in her vulvar region. Hidradenitis suppurativa is a chronic, recurrent inflammatory disease affecting skin that bears apocrine glands. It usually develops after puberty and is manifested by painful deep-seated inflamed lesions, including sinus tracts and abscesses.5 These abscesses are most frequently located in the genitofemoral area, such as in our patient,
4
R. Bicette et al. / J Pediatr Adolesc Gynecol xxx (2013) 1e4
however, they are usually multi-focal, extensive in nature, and respond quite well to either mono- or combination antibiotic therapy. There is a relationship between hidradenitis suppurativa and inflammatory bowel disease, with an increased frequency among patients with Crohn's disease, affecting up to 17% of such patients.5 A relationship between the two conditions is supported by clinical, histologic and epidemiologic similarities, such as sinus tracts, abscesses, granulomatous inflammation, and onset after puberty.5 Other things to consider in a patient with a vulvar abscess include chronic diverticulitis, genital herpes, other sexually transmitted diseases, and Methicillin-resistant Staphylococcus aureus. There have also been case reports of neoplasms presenting as vulvar abscesses, including myeloid sarcoma, adenocarcinoma, and small cell carcinoma. Conclusions
In summary, we present a case of prolonged patient suffering due to a delay in diagnosis of a relatively common condition, although infrequently diagnosed with this initial presentation. While this case report does not
suggest a routine work-up for inflammatory bowel disease in all patients who present with vulvar abscesses, it does demonstrate the importance of a careful examination of the differential diagnosis, with consideration to rare presentations of common conditions. This case also emphasizes the value of a thoughtful re-evaluation of a patient's history when there have been multiple instances of treatment and care, yet little to no response to therapy. In this instance, being able to expound upon our differential diagnosis and recognize some overlooked findings led to the evaluation and diagnosis of a chronic disease. With proper treatment we anticipate this patient to experience a higher quality of life. References 1. Podolsky DK: Inflammatory bowel disease. N Engl J Med 2002; 347:417 2. Baumgart DC, Sandborn WJ: Inflammatory bowel disease: clinical aspects and established and evolving therapies. Lancet 2007; 369:1641 3. Louis E, Collard A, Oger AF, et al: Behaviour of Crohn's disease according to the Vienna classification: changing pattern over the course of the disease. Gut 2001; 49:777 4. Regalado NG, Martin G, Antony SJ: Acinetobacter Iwoffii: bacteremia associated with acute gastroenteritis. Travel Med Infect Dis 2009; 7:316 5. Jemec GB: Clinical practice. Hidradenitis suppurativa. N Engl J Med 2012; 366: 158
Emory University School of Medicine, Atlanta, Georgia Department of Pediatric Gastroenterology, Emory University, Atlanta, Georgia 3 Department of Pediatric Radiology, Emory University, Atlanta, Georgia 4 Department of Gynecology and Obstetrics, Emory University, Atlanta, Georgia 2
a b s t r a c t Background: Inflammatory bowel disease is a relatively common condition that may present in a myriad of fashions, from the more frequently seen symptoms of diarrhea and abdominal pain to the less likely presentations with fistulas and abscesses. Case: A 14-year-old female with a presumed diagnosis of a Bartholin's gland cyst was treated for over 1 year with both medical and surgical interventions without her symptoms ever completely resolving. It was later found that these recurring vulvar abscesses were a manifestation of Crohn's disease. Conclusion: While the patient's history and physical are both very important in determining cause for pathology, we must also realize the importance of re-examining and broadening our differential diagnosis when faced with a patient who has failed multiple avenues of care. Key Words: Inflammatory bowel disease, Crohn's disease, Vulvar abscess
Introduction
Vulvar abscesses are well-described gynecologic conditions typically associated with simple infections that develop most commonly within Bartholin or Skene glands. They have also been described originating from skin and hair follicles, or wounds in the vulvar region. However, in rare situations, the abscess may be an initial presenting symptom of inflammatory bowel disease (IBD). Ulcerative colitis (UC) and Crohn's disease are the two forms of IBD. Key features of UC includes diffuse mucosal inflammation that extends proximally from the rectum and is restricted to the colon while Crohn's disease is characterized by chronic, relapsing inflammation frequently associated with perianal abcesses and fistulas. This case report describes an adolescent patient with recurring vulvar abscesses treated surgically and with prolonged courses of antibiotics. These abscesses persisted for over 1 year without resolution, at which time further evaluation ultimately demonstrated inflammatory bowel disease. Case
A 14-year-old virginal female was referred from an outside facility with a 16-month history of a recurring vulvar abscess. These symptoms began at age 12. Of note, her medical history was also significant for difficulty with
The authors indicate no conflicts of interest. * Address correspondence to: Lisa Haddad, MD, MS, MPH, Department of Gynecology and Obstetrics, Emory University School of Medicine, 49 Jesse Hill Jr. Drive, Atlanta, Georgia 30303; Phone: 404-778-1385; fax: 404-778-1382 E-mail address: [email protected] (L. Haddad).
feeding as an infant requiring G-tube feeds for a few months, as well as a history of chronic diarrhea for several years. The diarrhea was attributed to lactose intolerance, although it improved minimally on a lactose-free diet and no further work-up was pursued. Otherwise there was no significant past medical or surgical history; menarche at 9 with normal monthly menses was reported. The vulvar abscess was evaluated initially in the emergency room in February 2011 for what was believed to be a Bartholin's cyst. The cyst was drained in the emergency department, however over the following months, it continued to enlarge and cause the patient discomfort. Pain was severe such that she was unable to sit down comfortably at school. She was subsequently taken to the operating room twice to have the cyst drained and surgically resected, first in May 2011 and again in July 2011 after reaccumulation. A culture from a sample obtained at her second surgery grew Acinetobacter iwoffii. Consequently, she was placed on trimethoprim/sulfamethoxazole (TMP/SMX). She remained on antibiotics for ten months, without ever having full remission of the abscess. The patient reported periodic improvement, but her pain and vulvar swelling completely resolved. During this period, the cyst reaccumulated and spontaneously drained several times, which caused significant distress for the patient. After failure of both surgical and medical treatment, she was referred to our tertiary care facility for further evaluation. Upon presenting to our tertiary care facility, a limited physical examination demonstrated right labial swelling with induration extending across the length of the labia majora. There was no discrete mass palpated. Additionally, vesicular inflamed lesions were noted below the labial swelling and on the left lower labia across from the right
1083-3188/$ - see front matter Ó 2013 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpag.2013.07.010
2
R. Bicette et al. / J Pediatr Adolesc Gynecol xxx (2013) 1e4
Fig. 1. On presentation to our facility, prior to infliximab initiation. Note lesions inferior to right (R) and left (L) labiae.
sided lesion (Fig. 1). Given the patient's age, discomfort in the region, and virginal status, the physical evaluation was limited. There was no open lesion for culturing at that time. A magnetic resonance imaging (MRI) of the pelvis with and without contrast was obtained to further delineate the abscess. The MRI displayed a rim enhancing fluid collection within the right posterior perineum/buttock region consistent with an abscess, measured 2.2 cm 9 mm 4 cm, and was associated with multiple linear perianal fistula tracts (Fig. 2). An additional smaller abscess was noted
posteriorly in the right labia majora (Fig. 3). There were no definite inflammatory changes of bowel and her uterus and adnexae were normal. The fistula tracts seen on MRI raised suspicion for IBD, and the pediatric gastroenterology team was consulted on an outpatient basis. While awaiting the gastroenterology consult, it was decided by the primary team to discontinue TMP/SMX and initiate ciprofloxacin and metronidazole in order to broaden antibiotic coverage, which significantly improved her symptoms. However the patient continued to complain of a foul smelling vaginal discharge. She was admitted for further evaluation, at which time, serological evaluation was significant for mild iron deficiency anemia, while serum chemistries, ESR, CRP were all within normal limits. The Prometheus IBD sgi Diagnostic test (San Diego, CA), a panel that combines serologic, genetic and inflammatory markers, was performed in August 2012. The findings of increased antibodies for both anti-Saccharomyces cerevisiae antibody (ASCA) and outer membrane porin C (OmpC) were suggestive of a diagnosis of Crohn's Disease. She further underwent upper endoscopy and colonoscopy with biopsies, which were normal except for mild inactive gastritis. A diagnosis of Crohn's disease was made as a result of the colonoscopy based on multiple perianal fistula tracts, perineal abcesses and positive serologies. The antibiotics were discontinued and she began induction therapy with infliximab. Within two treatments during the induction phase, her fistulae stopped draining, there was remarkable improvement in her diarrhea and abdominal pain (Fig. 4) and she began gaining weight. She has had no recurrences since the initiating infliximab therapy. Comment
Here we present a case of a young girl with persistent vulvar symptoms for over one year, despite treatment, who was later determined to have an underlying inflammatory bowel disease as the etiology for her symptoms. Inflammatory bowel disease is a chronic inflammatory condition,
Fig. 2. Coronal T1 post contrast images. Arrows highlight the perianal fistulous tract leading to the right. Intense phlegmonous enhancement of the surrounding soft tissue in the right buttock.
R. Bicette et al. / J Pediatr Adolesc Gynecol xxx (2013) 1e4
3
Fig. 3. Axial fluid sensitive (T2) fat suppressed and T1 post contrast images at the level of the perineum. Arrows identify a transphincteric fistula at 5-6 o'clock leading to a large perianal abscess. Arrowhead shows thickening and enhancement of the right labia majora.
with the two major forms being Crohn's disease and Ulcerative Colitis. While both are idiopathic in nature, they are diagnostically defined by their clinical, pathologic, and endoscopic features.1 Usually unveiling itself in the second or third decade of life, Crohn's disease is a relapsing transmural inflammatory condition that can affect any area of the gastrointestinal tract, from the mouth to the anus.2 Although the development of complications such a strictures, fistulas, and abscesses are a known consequence of Crohn's disease, the initial clinical presentation is
Fig. 4. After second dose of infliximab; already demonstrating a decrease in labial swelling and lesions.
largely dependent on disease location and typically includes diarrhea, abdominal pain, fever, clinical signs of bowel obstruction, as well as passage of blood or mucus or both.3 At diagnosis, the disease is located in the terminal ileum in 47%, the colon in 28%, the ileocolon in 21%, and the upper gastrointestinal tract in 3%.3 Disease behavior is classified as non-stricturing and non-penetrating in 70% of patients, stricturing in 17%, and penetrating (fistulas, abscesses or both) in 13% of all patients at diagnosis.3 Our patient was initially assumed to have a Bartholin's gland abscess, but other causes were not considered at that time. Although uncommon, there have been case reports of similar abscesses occurring in infants and virginal females, thus a broader differential could have been considered and reduced the time for diagnosis. The location of this patient's abscess was also inconsistent with what would be considered a typical Bartholin's gland abscess, and more closely represented an external labial abscess. Further, her history of chronic diarrhea may have suggested an alternative GI origin for her symptoms. The culture finding of Acinetobacter lwoffii should have triggered a further work-up as it is an atypical organism in the vulvar region that is a part of the normal flora of the oropharynx and skin in approximately 25% of healthy individuals.4 The differential for a vulvar abscess is vast and quite broad, however, patient history and associated symptoms are imperative to narrow our differential and focus our initial work up. Another diagnostic consideration that was entertained when the patient presented to the tertiary care facility was hidradenitis suppurativa, since at that time she had multiple inflamed cystic lesions present in her vulvar region. Hidradenitis suppurativa is a chronic, recurrent inflammatory disease affecting skin that bears apocrine glands. It usually develops after puberty and is manifested by painful deep-seated inflamed lesions, including sinus tracts and abscesses.5 These abscesses are most frequently located in the genitofemoral area, such as in our patient,
4
R. Bicette et al. / J Pediatr Adolesc Gynecol xxx (2013) 1e4
however, they are usually multi-focal, extensive in nature, and respond quite well to either mono- or combination antibiotic therapy. There is a relationship between hidradenitis suppurativa and inflammatory bowel disease, with an increased frequency among patients with Crohn's disease, affecting up to 17% of such patients.5 A relationship between the two conditions is supported by clinical, histologic and epidemiologic similarities, such as sinus tracts, abscesses, granulomatous inflammation, and onset after puberty.5 Other things to consider in a patient with a vulvar abscess include chronic diverticulitis, genital herpes, other sexually transmitted diseases, and Methicillin-resistant Staphylococcus aureus. There have also been case reports of neoplasms presenting as vulvar abscesses, including myeloid sarcoma, adenocarcinoma, and small cell carcinoma. Conclusions
In summary, we present a case of prolonged patient suffering due to a delay in diagnosis of a relatively common condition, although infrequently diagnosed with this initial presentation. While this case report does not
suggest a routine work-up for inflammatory bowel disease in all patients who present with vulvar abscesses, it does demonstrate the importance of a careful examination of the differential diagnosis, with consideration to rare presentations of common conditions. This case also emphasizes the value of a thoughtful re-evaluation of a patient's history when there have been multiple instances of treatment and care, yet little to no response to therapy. In this instance, being able to expound upon our differential diagnosis and recognize some overlooked findings led to the evaluation and diagnosis of a chronic disease. With proper treatment we anticipate this patient to experience a higher quality of life. References 1. Podolsky DK: Inflammatory bowel disease. N Engl J Med 2002; 347:417 2. Baumgart DC, Sandborn WJ: Inflammatory bowel disease: clinical aspects and established and evolving therapies. Lancet 2007; 369:1641 3. Louis E, Collard A, Oger AF, et al: Behaviour of Crohn's disease according to the Vienna classification: changing pattern over the course of the disease. Gut 2001; 49:777 4. Regalado NG, Martin G, Antony SJ: Acinetobacter Iwoffii: bacteremia associated with acute gastroenteritis. Travel Med Infect Dis 2009; 7:316 5. Jemec GB: Clinical practice. Hidradenitis suppurativa. N Engl J Med 2012; 366: 158
