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Ann Thorac Surg 2001;72:1755-1758
© 2001 The Society of Thoracic Surgeons
a Division of Plastic and Reconstructive Surgery, Department of Surgery, American University Medical Center, Beirut, Lebanon
b Division of Cardiothoracic Surgery, Department of Surgery, American University Medical Center, Beirut, Lebanon
c Department of Pediatrics, American University Medical Center, Beirut, Lebanon
Accepted for publication November 30, 2000.
* Address reprint requests to Dr Kaddoura, Division of Plastic and Reconstructive Surgery, Department of Surgery, American University Medical Center, PO Box 113-6044, Beirut, Lebanon
e-mail: imadkaddoura{at}hotmail.com
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Abstract |
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Introduction |
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Expansion thoracoplasty utilizing various surgical techniques, including splitting the sternum or the rib cage and maintaining the separation with methacrylate [2], rib grafts [3], homologous bone grafts [4], or lateral rib cage expansion using staggered superiosteal rib osteotomies and rigid titanium miniplate and screw augmentation and stabilization[5], have served patients only for a short period, after which most reported patients outgrew their static chest expansion [1–8]. This is the probable cause of why there are only two patients, reported by Barnes and associates [3] and Sharoni and colleagues [6], who survived such procedures to reach their third year of life. We do not know whether they are still alive today.
We used a modified bailey rib approximator to provide a calibrated dynamic separation for the sternum after the primary procedure of midsternotomy and methyl methacrylate sternoplasty performed at 4 months of age failed to provide a lasting solution, with recurrence of the infant’s symptoms of restricted respiration.
The patient was a male infant born at term in a peripheral hospital. He was delivered by cesarean section because of reported fetal distress. Birth weight was 3,050 g, and his length was 48 cm. The baby was cyanotic at birth. Results of an echocardiogram were normal. The patient was discharged home at 5 days of age.
At 2 months of age, the patient was referred to the American University of Beirut Medical Center because of recurrent cyanosis and tachypnea. He was found to be in respiratory distress with partial oxygen tension of 50 mm Hg, partial carbon dioxide tension of 51 mm Hg, and oxygen saturation of 93% at 100% oxygen. He was intubated in the emergency room. Physical examination revealed a weight of 4,300 g and length of 52 cm. The chest appeared hypoplastic with a circumference of 30.2 cm (abdominal circumference 38 cm). The skeletal survey showed underdeveloped costochondral junctions but normal pelvis and phalanges. Computed tomographic scan confirmed the diagnosis of asphyxiating thoracic dystrophy (Jeune syndrome).
The patient was maintained on mechanical ventilation and fed by nasogastric tube for 1 week. Repeated attempts to wean him from the respirator failed, and a cardiothoracic surgeon was consulted for possible expansion of the chest. The patient was taken to the operating room where the sternum was split and expanded by a midsternotomy using a 2.5-cm methyl methacrylate prosthesis prepared according to physical measurements, chest roentgenogram, and computed tomographic scan. It was secured in place with 0 Tevdek taken through holes drilled in it and the sternum. The overlying skin was undermined and advanced to cover the prosthetic reconstruction. The patient’s symptoms improved: physical examination and chest roentgenogram showed lung expansion. The patient’s chest circumference 1 week postoperatively was 33 cm. He was discharged home 10 days postoperatively to be followed up closely.
During the following months, he did relatively well except for two episodes of pneumonia, manifested by cyanosis, tachypnea, and decreased feeding and activity. Each time he was hospitalized, cultures were obtained, and he was treated with antibiotics.
At 11 months of age, the patient was hospitalized because of severe cyanosis and tachypnea. His weight was 6,250 g and height 64 cm. His chest circumference was 35 cm, just 2 cm more than the immediate poststernal expansion. He required intubation, was weaned from the respirator 1 week later but continued to have recurrent episodes of desaturation necessitating repeated episodes of intubation and mechanical ventilation. It was obvious that he needed another procedure to replace the sternal prosthesis with a larger one or with another dynamic instrument that could provide sternal separation during the critical few months that followed. We opted for the second option.
A pediatric sternal spreader was modified at the Department of Medical Engineering: (Fig 1). One limb on each side was used instead of two, and the sternum huggers on both sides were fitted with two hooks each plus two slits that would allow suturing to the sternum for additional stability. The spreading lever of the instrument was replaced by a hexagonal 2.7 mm A-O (Synthes; Mathys Medical, Bettlach, Switzerland) screw that was calibrated to spread 6 mm with a full turn and that could be exteriorized outside the skin. The serrated shaft proper was shortened to a total width of 8 cm.
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In 1995, Davis and associates [5] reported a 4-year-old child with Jeune asphyxiating dystrophy in whom lateral rib cage expansion was done using staggered subperiosteal rib osteotomies and rigid titanium miniplate augmentation and stabilization with primary skin closure. The procedure allowed a safe unilateral chest wall expansion in addition to maintaining the protection of the anterior mediastinal structures and bone growth, with moderate respiratory symptoms. The procedure had to be repeated on the contralateral side, and longer follow-up was needed to determine whether chest growth would continue after such a procedure.
In 1998, Sharoni and colleagues [6] reported a 3-month-old child with Jeune syndrome who was treated with the technique of Todd and colleagues [2, 8] followed by removal of the methyl methacrylate and replacement of it with bilateral pectoralis major muscle flaps. The child was weaned successfully 1 month later, and a computed tomographic scan showed good lung aeration. The child was alive at 18 months of age. This patient and that reported by Barnes and associates [3] are probably the only two reported patients with severe asphyxiating thoracic dystrophy who had surgery and survived.
Provided that lung growth potential is normal in infants with asphyxiating thoracic dystrophy, surgical expansion of the rib cage should allow further development of the lungs and partial resolution and stabilization of the pulmonary hypoplasia and associated clinical problems. However, most, if not all, previously described surgical techniques of chest wall expansion, including resection of costochondral cartilage [7, 8] and splitting the sternum and maintaining the separation with rib graft or prosthetic interposition [2–4], were static and did not provide dynamic progressive expansion of the chest in patients who presented early in life with the severe form of this condition.
Although this instrument might provide a dynamic solution and could be utilized at the initial procedure, we are aware of its possible limitations and possible complications. First, this is too much hardware to place in an infant; its size, shape, and performance need to be refined. We cannot reach conclusions from one case about the best time of insertion, its duration of installment, and the optimal time for removal. As with any foreign material placement, in this case stainless steel, possible complications include secondary infection of the sternum and the skin with possible dehiscence of the instrument and displacement. Longer follow-up will be required before the value of such an instrument can be evaluated. Because of the rarity of such an entity, it will require a multicenter study. Only then can we reach conclusions about whether such an instrument provides a superior solution to the existing techniques.
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This article has been cited by other articles:
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  C.-H. Chen, H.-C. Liu, T.-T. Hung, and C.-H. Chen Restrictive Chest Wall Deformity as a Complication of Surgical Repair for Pectus Excavatum Ann. Thorac. Surg., February 1, 2010; 89(2): 599 - 601. [Abstract] [Full Text] [PDF]
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W. J. Norberg Long-term follow-up of asphyxiating thoracic dystrophy: Results of first report of thoracoplasty Ann. Thorac. Surg., December 1, 2002; 74(6): 2226 - 2226. [Full Text] [PDF]
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I. L. Kaddoura, M. Y. Obeid, S. M. Mroueh, and A. A. Nasser Reply Ann. Thorac. Surg., December 1, 2002; 74(6): 2226 - 2226. [Full Text] [PDF]
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