Bifid Fifth Rib in a 9-Year-Old Girl With Chest Pain
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Nova Southeastern University College of Osteopathic Medicine Ft Lauderdale, Fla
To the Editor: A nine-year-old girl was brought to our outpatient pediatric clinic complaining of chest pain on the right side. Her parents said that the discomfort began after a minor fall. The girl had been engaged in normal recreational activity when she was pushed down, and she landed on her right side.
On arrival at the clinic, the patient reported moderate pain of 2 hours' duration. The pain was localized to the right lateral thorax, did not radiate, and was not associated with any respiratory distress. Deep inspiration did not exacerbate the pain. The patient denied loss of consciousness, seizures, nausea, vomiting, or dyspnea. She was alert, appropriately oriented, and appeared comfortable. She had no medical or surgical history, and her family history was unremarkable for heart disease or seizures. Her immunizations were up to date, and she had progressed appropriately through school.
A physical examination revealed a well-developed, well-nourished Hispanic girl, with no external signs of trauma. Her blood pressure, heart rate, and respiratory rate were all within normal limits, and oxygen saturation while breathing room air was 99%. A baseline electrocardiogram revealed a sinus arrhythmia without ectopy. The QRS axis was normal, and there were no ST segment abnormalities. A chest examination found minimal discomfort to light and deep palpation of the right lateral thorax. The discomfort was localized to the midaxillary line and was not accompanied by any surrounding ecchymosis or bony crepitation. Her lung sounds were clearly audible in all fields bilaterally. Heart sounds were also normal, with no appreciable murmurs, rubs, or gallops. The patient's abdomen was soft and nontender to palpation, and the extremities were all intact with good distal pulses and brisk capillary refill. Skin examination findings were negative for any obvious deformity, discoloration, edema, or other lesion.
A chest radiograph was performed as part of the initial work-up (Figure). The radiograph film did not show any fracture or obvious soft tissue injury, and there were no cardiovascular or pulmonary pathologic findings, such as pneumothorax. However, the film did show a bifid fifth rib on the right side. The radiographic findings correlated with those of the physical examination finding of palpable tenderness to the right lateral thorax. A recommendation was made to follow up with the patient clinically. The patient's parents were advised that the radiographic findings likely represented a normal anatomic variant. The patient was discharged to home with instructions to take ibuprofen every 6 hours as needed for pain.
The girl returned for a follow-up examination several days later and reported no complaints. Her pain had resolved completely and could not be reproduced on palpation or with movement. The patient was discharged to home without further incident or investigation.
Little information exists in the medical literature about the clinical significance of bifid ribs. An abstract by Osawa et al1 discusses bifid ribs as anatomic variants found on routine cadaveric dissection. One syndrome, however, does appear in association with bifid ribs. Basal cell nevus syndrome, also called Gorlin-Goltz syndrome, is a multisystem disorder that predominately affects the white population. Cutaneous manifestations of this disease include epidermal cysts, palmoplantar pits, facial milia, and subcutaneous calcifications.2 Skeletal defects are also found, including "costal anomalies such as bifid, splayed, or synostotic ribs, and ribs associated with the cervical spine."3 The disorder, though rare, is well described in the medical literature. The incidence is estimated at 1 per 600,000 live births, and it is most commonly inherited as an autosomal dominant trait. Although our patient demonstrated none of the obvious stigmata of this syndrome, 60% to 70% of patients with diagnosed Gorlin-Goltz syndrome demonstrate rib anomalies.3,4 The American Academy of Family Physicians recommends that screening for this disorder include oral and skin examinations, radiographic imaging of the chest and skull, and magnetic resonance imaging of the brain.4
Wattanasirichaigoon and colleagues5 described patterns of rib defects occurring in a retrospective review of 47 cases. The authors suggested that rib anomalies can occur in isolation or as part of vertebral malformations. Rib fusion was the most commonly described defect, reported in 72% of the 47 patients, and bifid ribs occurred in 28% of the patients.5 The small patient population makes it difficult to extrapolate about relevant clinical manifestations.
In a small study6 conducted at the Children's Hospital Medical Center in Cincinnati, Ohio, 27 children underwent computed tomography or magnetic resonance imaging to investigate the origin of palpable, asymptomatic anterior chest wall lesions. Radiologists were able to discern the source of the lesion in 26 of the children studied. In addition to one bifid rib, other normal variants included "prominent anterior convex ribs in 10 patients; `tilted' sternum in six; prominent asymmetric costal cartilage in four... and well-defined, small (<1 cm) subcutaneous nodule adjacent to costal cartilage in five." No patients in that study required further intervention or treatment. The authors report that further radiologic imaging for asymptomatic palpable chest wall lesions is of low yield.
Based on a review of the current medical literature on bifid ribs, it is advisable to perform a thorough physical examination on any patient found to have a bifid rib. Careful oral and cutaneous screenings can rule out lesser-known genetic syndromes. Physical findings such as odontogenic cysts, palmar pits, or irregular subcutaneous calcifications, warrant more intensive radiologic and genetic investigation. If the remainder of the physical examination findings is unremarkable and the patient is asymptomatic, additional clinical or radiologic investigation is of low yield.
References
1. Osawa T, Sasaki T, Matsumoto Y, Tsukamoto A, Onodera M, Nara E, et al. Bifid ribs observed in the third and fourth ribs . Kaibogaku Zasshi.1998 :73;633 –635.
2. Crutchfield CE, Geiger J, Gorlin RJ, Ahmed I. What syndrome is this? Pediatr Dermatol.2000; 17:484 –486.
3. Bakaeen G, Rajab LD, Sawair FA, Hamdan MA, Dallal ND. Nevoid basal cell carcinoma syndrome: a review of the literature and a report of a case. Int J Pediatr Dent.2004; 14:279 –287.
4. Bitar GJ, Herman CK, Dahman MI, Hoard MA. Basal cell nevus syndrome: guidelines for early detection. Am Fam Physician. 2002;65:2501 –2504.
5. Wattanasirichaigoon D, Prasad C, Schneider G, Evans JA, Korf BR. Rib defects in patterns of multiple malformations: a retrospective review and phenotypic analysis of 47 cases . Am J Med Genet A. 2003;122:63 –69.
6. Donnelly LF, Taylor CN, Emery KH, Brody AS. Asymptomatic, palpable, anterior chest wall lesions in children: is cross-sectional imaging necessary? . Radiology.1997; 202:829 –831.
DIPESH BATRA, MD, Attending Pediatric Physician
Central Florida Health Care, Inc Avon Park, Fla Clinical Assistant Professor of Pediatrics Nova Southeastern University College of Osteopathic Medicine Ft Lauderdale, Fla(BENJAMIN J. LAWNER, EMT-P)
To the Editor: A nine-year-old girl was brought to our outpatient pediatric clinic complaining of chest pain on the right side. Her parents said that the discomfort began after a minor fall. The girl had been engaged in normal recreational activity when she was pushed down, and she landed on her right side.
On arrival at the clinic, the patient reported moderate pain of 2 hours' duration. The pain was localized to the right lateral thorax, did not radiate, and was not associated with any respiratory distress. Deep inspiration did not exacerbate the pain. The patient denied loss of consciousness, seizures, nausea, vomiting, or dyspnea. She was alert, appropriately oriented, and appeared comfortable. She had no medical or surgical history, and her family history was unremarkable for heart disease or seizures. Her immunizations were up to date, and she had progressed appropriately through school.
A physical examination revealed a well-developed, well-nourished Hispanic girl, with no external signs of trauma. Her blood pressure, heart rate, and respiratory rate were all within normal limits, and oxygen saturation while breathing room air was 99%. A baseline electrocardiogram revealed a sinus arrhythmia without ectopy. The QRS axis was normal, and there were no ST segment abnormalities. A chest examination found minimal discomfort to light and deep palpation of the right lateral thorax. The discomfort was localized to the midaxillary line and was not accompanied by any surrounding ecchymosis or bony crepitation. Her lung sounds were clearly audible in all fields bilaterally. Heart sounds were also normal, with no appreciable murmurs, rubs, or gallops. The patient's abdomen was soft and nontender to palpation, and the extremities were all intact with good distal pulses and brisk capillary refill. Skin examination findings were negative for any obvious deformity, discoloration, edema, or other lesion.
A chest radiograph was performed as part of the initial work-up (Figure). The radiograph film did not show any fracture or obvious soft tissue injury, and there were no cardiovascular or pulmonary pathologic findings, such as pneumothorax. However, the film did show a bifid fifth rib on the right side. The radiographic findings correlated with those of the physical examination finding of palpable tenderness to the right lateral thorax. A recommendation was made to follow up with the patient clinically. The patient's parents were advised that the radiographic findings likely represented a normal anatomic variant. The patient was discharged to home with instructions to take ibuprofen every 6 hours as needed for pain.
The girl returned for a follow-up examination several days later and reported no complaints. Her pain had resolved completely and could not be reproduced on palpation or with movement. The patient was discharged to home without further incident or investigation.
Little information exists in the medical literature about the clinical significance of bifid ribs. An abstract by Osawa et al1 discusses bifid ribs as anatomic variants found on routine cadaveric dissection. One syndrome, however, does appear in association with bifid ribs. Basal cell nevus syndrome, also called Gorlin-Goltz syndrome, is a multisystem disorder that predominately affects the white population. Cutaneous manifestations of this disease include epidermal cysts, palmoplantar pits, facial milia, and subcutaneous calcifications.2 Skeletal defects are also found, including "costal anomalies such as bifid, splayed, or synostotic ribs, and ribs associated with the cervical spine."3 The disorder, though rare, is well described in the medical literature. The incidence is estimated at 1 per 600,000 live births, and it is most commonly inherited as an autosomal dominant trait. Although our patient demonstrated none of the obvious stigmata of this syndrome, 60% to 70% of patients with diagnosed Gorlin-Goltz syndrome demonstrate rib anomalies.3,4 The American Academy of Family Physicians recommends that screening for this disorder include oral and skin examinations, radiographic imaging of the chest and skull, and magnetic resonance imaging of the brain.4
Wattanasirichaigoon and colleagues5 described patterns of rib defects occurring in a retrospective review of 47 cases. The authors suggested that rib anomalies can occur in isolation or as part of vertebral malformations. Rib fusion was the most commonly described defect, reported in 72% of the 47 patients, and bifid ribs occurred in 28% of the patients.5 The small patient population makes it difficult to extrapolate about relevant clinical manifestations.
In a small study6 conducted at the Children's Hospital Medical Center in Cincinnati, Ohio, 27 children underwent computed tomography or magnetic resonance imaging to investigate the origin of palpable, asymptomatic anterior chest wall lesions. Radiologists were able to discern the source of the lesion in 26 of the children studied. In addition to one bifid rib, other normal variants included "prominent anterior convex ribs in 10 patients; `tilted' sternum in six; prominent asymmetric costal cartilage in four... and well-defined, small (<1 cm) subcutaneous nodule adjacent to costal cartilage in five." No patients in that study required further intervention or treatment. The authors report that further radiologic imaging for asymptomatic palpable chest wall lesions is of low yield.
Based on a review of the current medical literature on bifid ribs, it is advisable to perform a thorough physical examination on any patient found to have a bifid rib. Careful oral and cutaneous screenings can rule out lesser-known genetic syndromes. Physical findings such as odontogenic cysts, palmar pits, or irregular subcutaneous calcifications, warrant more intensive radiologic and genetic investigation. If the remainder of the physical examination findings is unremarkable and the patient is asymptomatic, additional clinical or radiologic investigation is of low yield.
References
1. Osawa T, Sasaki T, Matsumoto Y, Tsukamoto A, Onodera M, Nara E, et al. Bifid ribs observed in the third and fourth ribs . Kaibogaku Zasshi.1998 :73;633 –635.
2. Crutchfield CE, Geiger J, Gorlin RJ, Ahmed I. What syndrome is this? Pediatr Dermatol.2000; 17:484 –486.
3. Bakaeen G, Rajab LD, Sawair FA, Hamdan MA, Dallal ND. Nevoid basal cell carcinoma syndrome: a review of the literature and a report of a case. Int J Pediatr Dent.2004; 14:279 –287.
4. Bitar GJ, Herman CK, Dahman MI, Hoard MA. Basal cell nevus syndrome: guidelines for early detection. Am Fam Physician. 2002;65:2501 –2504.
5. Wattanasirichaigoon D, Prasad C, Schneider G, Evans JA, Korf BR. Rib defects in patterns of multiple malformations: a retrospective review and phenotypic analysis of 47 cases . Am J Med Genet A. 2003;122:63 –69.
6. Donnelly LF, Taylor CN, Emery KH, Brody AS. Asymptomatic, palpable, anterior chest wall lesions in children: is cross-sectional imaging necessary? . Radiology.1997; 202:829 –831.
DIPESH BATRA, MD, Attending Pediatric Physician
Central Florida Health Care, Inc Avon Park, Fla Clinical Assistant Professor of Pediatrics Nova Southeastern University College of Osteopathic Medicine Ft Lauderdale, Fla(BENJAMIN J. LAWNER, EMT-P)