Baby girl with right labia majora mass
A 26-day-old female neonate, born at 36 weeks of gestation, presents to the clinic with a visible swelling in the right labia majora.
THE CASE
A 26-day-old female neonate, born at 36 weeks of gestation, presents to the clinic with a visible swelling in the right labia majora. The patient was born to a 21-year-old female via vaginal birth. Group B Streptococcus was present at birth and adequately treated with 3 doses of penicillin G. The mother developed cholestasis during pregnancy and was treated with ursodiol. Paternal age was 23 years, and the family history was unremarkable.
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Physical examination
On physical examination, the infant was in no acute distress with normal vital signs. An abnormal 4-cm, soft, bulging mass was visualized on the right labia majora that was nontender, nonerythematous, nonfluctuant, and nonreducible (Figure 1). The patient’s abdomen was soft, nontender, nondistended, and with normal bowel sounds. No signs of bowel obstruction, strangulation, or acute abdomen were noted. A clinical diagnosis of inguinal hernia was made with suspected protrusion of an ovary versus an intraabdominal/pelvic organ, and a pelvic ultrasound was ordered to investigate further.
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Ultrasound revealed a normal right ovary in the pelvic cavity and the left ovary in the subcutaneous tissue of the right labia majora measuring proximally 1.2 cm x 1.9 cm x 1.9 cm (Figures 2A, 2B). Performed at 40 days of postnatal age, Color Doppler ultrasonography showed normal blood vessels and flow along with the round ligament extending into the mass. A consultation with pediatric surgery was arranged for the following day.
Differential diagnosis
Because the causes of labial masses in the infant are diverse, differentiation is important for management. Differential diagnosis includes: hydrocele, lipoma, indirect-direct inguinal hernia, femoral hernia, lymphadenopathy, epidermal inclusion cyst, cystic lymphangioma, and cyst of the canal of Nuck (Table). Although inguinal hernia is a diagnosis commonly obtained by history and physical examination, high-resolution, high-frequency sonography with color Doppler imaging plays an important role as the diagnostic imaging modality of choice.
An infant with an inguinal hernia should be thoroughly evaluated to determine whether hernia sac contents are present, because the herniated structures are at an increased risk of incarceration and infarction.1,2 Ultrasound can differentiate direct, indirect, and femoral hernias, as well as differentiate a hernia from other causes of swelling such as a hydrocele or lymph node.3 In an infant presenting with an inguinal hernia, the authors support that ultrasonography be the diagnostic modality of choice. The reported diagnostic accuracy of ultrasound is 93% for acute groin conditions.4
In the present case, the infant was diagnosed with a nonreducible right inguinal hernia.
Preoperative ultrasonography demonstrated an unusual finding of an intact right ovary with a suspected herniation of the left (contralateral) ovary into the right labia majora. The left ovary was reported to be incarcerated but not strangulated.
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Epidemiology
The highest incidence of hernia is found in premature infants, and its prevalence is approximately 25% of births.5,6 Inguinal hernias are more common on the right side, occurring in as many as 60% of cases.7,8 An ovary in an inguinal hernia is predisposed to strangulation and torsion, with an increased risk of infertility if not managed promptly.9
Etiology and pathophysiology
Indirect inguinal hernias in infants occur because of abnormal fetal development. The etiology is fundamentally similar in females and males but can present with different etiologies depending on sex. The occurrence of a sliding inguinal hernia necessitates a review of embryologic development. In males, the testes pass through the processus vaginalis during the 7th to 9th month of gestation. As the testes pass through the processus vaginalis, the processus elongates, then subsequently obliterates once the male testes have passed through it. This process closes the internal inguinal ring proximally, while persisting distally as the tunica vaginalis of the testes. Failure of the proximal part to close results in patency and potential for a bowel loop or other organ to pass through the inguinal ring as an indirect inguinal hernia. It may also persist and form a hydrocele.10
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The canal of Nuck is the female derivative of the processus vaginalis (males). This canal normally closes around the 7th month, which is earlier than in males.10 The fetal ovary is an abdominal organ and possesses a gubernaculum that extends from its lower pole to a point corresponding to the abdominal inguinal ring in the fetus, through which it continues into the labia majora. Instead of descending, as do the testes, the gubernaculum pulls the ovary medially, where it lies adjacent to the uterus. The intraabdominal portion of the gubernaculum ovarii attaches to the lateral portion of the developing uterus. The segment extending from the ovary to the uterus becomes the ligament of the ovary, and the distal segment extending from the uterus to the labia majora becomes the round ligament of the uterus.10–13 Hence, when the canal of Nuck remains patent, it allows for the development of an indirect inguinal hernia. In short, in addition to a possible loop of bowel herniating into the inguinal ring, the ipsilateral ovary, fallopian tube, or even uterus may be pulled down into the inguinal canal by the descending gubernaculum, forming a hernia of any of the named structures.9,11-13
Discussion
To the authors’ knowledge, this case is the first report of an unusual herniation of contralateral ovaries.
Inguinal hernia is a common congenital anomaly in the female pediatric population. Even more common is an inguinal hernia in a premature infant.5,6 About 1% to 2% of newborns have inguinal hernias, and the female-to-male ratio ranges between 1:4 and 1:10.14 Spontaneous regression occurs frequently. The possibility of regression, however, decreases when abdominal or pelvic organs such as intestinal bowel or ovaries are herniated.15 Further, herniated organs increase the chance of other complications such as incarceration and ovarian torsion among other surgical emergencies.16 Therefore, early diagnosis is crucial to avoid irreversible organ damage, and as has been demonstrated, ultrasonography and color Doppler ultrasonography are helpful radiologic study methods.
An asymptomatic labia majora mass in a female infant should raise a high level of suspicion for a prolapsed abdominal or pelvic organ. Prompt clinical diagnosis supported by imaging confirmation is necessary to determine the most appropriate management. Imaging studies also help rule out other pathologies sharing a similar presentation but requiring different management.
Inguinal hernias in females must be evaluated promptly. Females with a herniated ovary, fallopian tube, or uterus are at an increased risk of infertility if not diagnosed and managed in a timely manner.9 The clinical presentation of an ovarian inguinal hernia is nonspecific and may include incidental soft tissue mass as seen in this case. Inguinal hernias are most often found by a child’s parents at bath time or by pediatricians during a well-child visit.10 History may include an intermittent bulge in the groin, labia, or scrotum that is most apparent during episodes of increased intraabdominal pressure (eg, crying, straining). Pathologic differentials can range from (non)communicating hydroceles to undescended testes and inguinal adenopathy. Age at presentation is variable. Females can present with an inguinal hernia even years after birth.16
Timing of surgery is an important issue. With ovarian inguinal hernias, ovarian strangulation has been reported in up to 32% of cases. This percentage emphasizes the importance of early surgical repair in irreducible hernias, even in the asymptomatic patient population.9 Researchers report that 90% of complications can be avoided if repair is undertaken within 1 month of diagnosis.17 Additionally, it has been reported that repair undertaken within 2 weeks decreased the rate of incarceration by half compared with a 30-day wait.18 Further, most patients can undergo repair safely in an ambulatory setting. Exceptions include premature infants and older children with significant risk factors such as cardiac or respiratory problems.
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It is important to remember that, although rare, prolapsed organs in females may include the ipsilateral ovary, uterus, or the contralateral ovary. Therefore, the content of the hernia sac must be thoroughly evaluated at surgery, leaving room for unexpected presentation once the inguinal sac has been ligated. Routine contralateral exploration is left to the discretion of the surgeon, as more recent reports suggest that this leads to an unnecessary number of procedures because only 20% of patients presenting with a unilateral hernia will develop a contralateral hernia.
When surgical intervention returns no contents in the hernia sac, it is possible that the contents (left ovary) visualized on preoperative ultrasound reduced into the pelvic cavity prior to surgical operation. Even in cases of hernia presurgical reduction, undergoing surgery is advised to prevent future complications.10 Further, despite a thorough history, physical exam, and radiologic evidence to aid diagnosis, presentation during surgery can be unexpected and care should be taken to prepare for unforeseen intraoperative findings.
Treatment and outcome
An elective bilateral inguinal hernia repair was performed in the patient 2 weeks later. The right inguinal region was the first side to be operated on, and it was determined that neither intraabdominal contents nor the ovary were present in the sac. The left hernia sac was visualized in a similar manner, and it was again determined that neither intraabdominal contents nor the ovary were present in the sac. The postoperative diagnosis was unilateral right inguinal hernia with left-side negative exploration.
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The patient made a full recovery and was seen in the pediatric clinic for follow-up. No complications or other medical problems were noted.
Acknowledgments: The authors thank the Seaver Foundation. Dr Lozano is an NIH (GM082773) and Seaver Faculty Scholar.
REFERENCES
1. Hennelly K, Shannon M. A 3-month-old female with an inguinal mass. J Emerg Med. 2011;40(1):33-36.
2. Laing FC, Townsend BA, Rodriguez JR. Ovary-containing hernia in a premature infant: sonographic diagnosis. J Ultrasound Med. 2007;26(7):985-987.
3. Tilt L, Kessler DO. Rapid evaluation of an inguinal mass in a female infant using point-of-care ultrasound. Pediatr Emerg Care. 2014;30(5):366-367.
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6. Walsh SZ. The incidence of external hernias in premature infants. Acta Paediatr. 1962;51:161-164.
7. Rowe MI, Clatworthy HW Jr. The other side of the pediatric inguinal hernia. Surg Clin North Am. 1971;51(6):1371-1376.
8. Ziegler MM. Diagnosis of inguinal hernia and hydrocele. Pediatr Rev. 1994;15(7):286-288.
9. Boley SJ, Cahn D, Lauer T, Weinberg G, Kleinhaus S. The irreducible ovary: a true emergency. J Pediatr Surg. 1991;26(9):1035-1038.
10. Glick PL, Boulanger SC. Inguinal hernias and hydroceles. In: Coran AG, Adzick NS, Krummel TM, et al, eds. Pediatric Surgery. 7th ed. Philadelphia, PA: Elsevier Saunders; 2012:985-1001.
11. Shadbolt CL, Heinze SB, Dietrich RB. Imaging of groin masses: inguinal anatomy and pathologic conditions revisited. Radiographics. 2001;21:S261-S271.
12. Moore KL, Persaud TVN. The urogenital system. In: Moore KL, Persaud TVN, eds. The Developing Human: Clinically Oriented Embryology. 7th ed. Philadelphia, PA: WB Saunders; 2003:324-325.
13. Merriman TE, Auldist AW. Ovarian torsion in inguinal hernias. Pediatr Surg Int. 2000;16(5-6):383-385.
14. Huang CS, Luo CC, Chao HC, Chu SM, Yu YJ, Yen JB. The presentation of asymptomatic palpable movable mass in female inguinal hernia. Eur J Pediatr. 2003;162(7-8):493-495.
15. Oudesluys-Murphy AM, Teng HT, Boxma H. Spontaneous regression of clinical inguinal hernias in preterm female infants. J Pediatr Surg. 2000;35(8):1220-1221.
16. Goldstein IR, Potts WJ. Inguinal hernia in female infants and children. Ann Surg. 1958;148(5):819-822.
17. Wiener ES, Touloukian RJ, Rodgers BM, et al. Hernia survey of the Section on Surgery of the American Academy of Pediatrics. J Pediatr Surg. 1996;31(8):1166-1169.
18. Zamakhshary M, To T, Guan J, Langer JC. Risk of incarceration of inguinal hernia among infants and young children awaiting elective surgery. CMAJ. 2008;179(10):1001-1005.
Dr Barillas-Cerritos is pediatric attending, Woodhull Medical and Mental Health Center, Department of Pediatrics, New York University, New York, and assistant professor in pediatrics, St. George’s University School of Medicine, Grenada. Mr Speirs is an MD candidate at St. George’s University School of Medicine, Grenada. Dr Lozano is assistant professor of psychiatry, genetics and genomic sciences, and pediatrics, Icahn School of Medicine at Mount Sinai, New York, New York. The authors have nothing to disclose in regard to affiliations with or financial interests in any organizations that may have an interest in any part of this article.
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