PEDIATRIC SURGERY UPDATE ©

Midline Cervical Cleft

Midline cervical cleft is a rare congenital malformation of the ventral neck that, despite being recognized for more than a century and a half, remains unfamiliar enough to many clinicians that it is frequently overlooked or misclassified at the moment of presentation. The lesion was first documented in the mid-nineteenth century, described in greater detail in the early twentieth century under the heading of thyroglossal anomalies, and given its current name in the 1950s. Even now the total experience recorded in the world literature is modest, amounting to only a few hundred reported cases, and its true frequency is uncertain because isolated instances are often considered too trivial to publish. Available estimates place it at roughly one to two percent of congenital anterior neck defects, with one population-based approximation suggesting an occurrence on the order of one in seventy thousand births. Historically a female predominance of up to two to one was proposed, but larger pooled analyses have found the sexes affected almost equally, and one extensive review even reported a slight male excess, so that no convincing sex predilection can currently be claimed. A tendency toward higher frequency in individuals of European ancestry has been suggested but rests on limited data. The malformation is present from birth, although its subtlety in the neonate means the diagnosis may not be made until infancy, when impaired neck mobility becomes apparent. The classic presentation is a triad. Cranially there is a small nipple-like or hood-like skin tag; along the midline there is a linear atrophic groove of dysmorphic or absent skin, most often lined by a reddened, desquamating epithelium; and caudally there is a blind-ending sinus or pit that may intermittently discharge mucoid or serous fluid. Beneath the atrophic surface lies the anatomical feature of greatest clinical importance, a fibrous cord that runs longitudinally from the region of the mandibular symphysis to the sternum or manubrium. This cord, together with the paucity of overlying skin, tethers the anterior neck, producing a web, limiting extension, and effacing the normal cervico-mental angle. Over time the traction transmitted to the developing mandible can generate a bony spur, encourage mandibular hypoplasia and retrognathia, and contribute to an open-bite tendency and an appearance of micrognathia. Not all three components of the triad are invariably present; pooled data indicate that most patients display the full triad, while a minority present with only one or two elements, the linear atrophic groove being the most consistent finding and the caudal sinus and cephalic nodule somewhat less frequent. The embryological basis remains debated, but the most widely accepted hypothesis attributes the defect to failure of midline fusion of the first and second, and possibly the third, branchial arches during the third and fourth weeks of intrauterine development. In this model, a persistent midline furrow allows abnormal deposition and migration of mesodermal cells within the ectoderm, which then differentiate into the skeletal muscle, glandular tissue, and mucosal surface characteristic of the lesion. Competing theories have invoked a bronchogenic origin, persistence of thyroglossal remnants, vertical outgrowth of tongue musculature, pressure necrosis from the pericardial roof, and localized vascular ischemia, none of which fully accounts for the consistent anatomy or histology. The condition is regarded as a caudal variant of the number 30 facial cleft in the standard craniofacial classification, and although it is overwhelmingly sporadic, a small number of familial occurrences, including transmission between a grandparent and grandchild, have prompted genetic study. Exome analyses have suggested a polygenic contribution, with reported associations involving loss of a pregnancy-associated plasma protein and mutations affecting several candidate genes, but no single causal mutation has been established. Histopathology varies by region of the specimen. The cephalic tag typically shows normal skin, sometimes with cartilage or muscle. The linear cleft demonstrates keratinized stratified squamous epithelium with parakeratosis and a characteristic absence of epithelial adnexa in the dermis, with fibrosis replacing the superficial musculature. The caudal tract is lined by pseudostratified ciliated columnar epithelium, at times of respiratory type, and contains seromucinous glands that explain the mucoid discharge. A more recent and clinically consequential observation is that the subcutaneous cord is not merely fibrous but frequently contains a bundle of mature striated muscle lying superficial to the platysma, in the plane normally occupied by subcutaneous fat. This contractile band, demonstrable by its response to cautery and by imaging, appears to be the true agent of neck tethering, and its previous under recognition is thought to explain many of the recurrences reported after incomplete surgery. Because the diagnosis is essentially clinical, imaging is used chiefly to confirm the lesion and exclude concurrent pathology rather than to establish the diagnosis. Ultrasonography is the reasonable first-line modality, being noninvasive and well tolerated, and it typically shows an avascular blind-ending sinus tract with an associated fibrous band in the dermis while allowing assessment of the thyroid and adjacent structures. Cross-sectional imaging is reserved for atypical presentations, suspected associated anomalies, or surgical planning; magnetic resonance imaging characteristically reveals thinning of the epidermis and a linear tract that is hypointense on T1 and hyperintense on T2 without involvement of deep bony or cartilaginous structures. The principal entities in the differential diagnosis are thyroglossal duct cyst, which elevates with tongue protrusion and swallowing and relates to the hyoid, and branchial cleft anomalies, which are usually lateral, along with dermoid and epidermoid cysts. Associated malformations, when they occur, include clefting of the lip, mandible, tongue, or sternum, absence of portions of the hyoid or thyroid cartilage, and, more rarely, midline hemangioma and cardiac defects, so inspection of the oral cavity, tongue, and chest is prudent. Treatment is surgical, and the cardinal principle on which the literature is unanimous is that the entire lesion, and above all the fibrous or fibromuscular cord, must be excised completely; mere transection or partial resection reliably produces recurrence and persistent contracture. Early intervention, generally advocated before one year of age and by some authors within the first months of life, is favored because the lesion is smaller and less mature, the resection and resulting scar are minimized, and growth restriction of the mandible may be limited. The closure technique remains the chief area of debate. Simple linear closure can suffice for small, immature lesions when adjacent tissue is lax and no established contracture is present, but it carries a higher risk of recurrent contracture and vertical scar banding. Consequently most surgeons employ some geometric closure, with single, double, or opposing Z-plasty being the most common, since these lengthen the contracted midline, reorient the scar into natural skin creases, and help recreate the cervico-mental angle; W-plasty has also been used successfully. Hypertrophic scarring is the most frequently encountered complication, particularly along the limbs of a Z-plasty, while recurrence has been observed almost exclusively when excision was incomplete or a straight-line repair was chosen. Long-term morphometric follow-up suggests that after release the chin grows at a normal rate but shows no catch-up, remaining somewhat posteriorly displaced, leaving unresolved whether the retrognathia reflects an intrinsic developmental defect or the mechanical legacy of the cord. In sum, midline cervical cleft is a rare but clinically distinctive anomaly whose recognition depends on appreciating its characteristic triad and its tethering cord. Prompt clinical diagnosis, judicious use of ultrasonography, and early complete excision of the fibrous and muscular band, followed by a tension-free geometric closure, offer reliable restoration of neck contour and mobility and minimize the functional and esthetic sequelae that otherwise accrue with age. References: 1- D'Souza JN, Valika T, Maddalozzo J. Surgical management of midline cervical cleft. Int J Pediatr Otorhinolaryngol. 127:109657, 2019 2- Çelikoyar M, Aktan E, Dogusoy G. Congenital midline cervical cleft: a case report. J Med Case Rep. 13(1):176. 2019 3- Riba M, Bejarano M, Hernández C, Moraleda I, Massaguer C, Ribalta T, Gómez M, Krauel L, Parri FJ, Albert A. Midline Cervical Cleft: An Anatomical Finding and a Proposal for a New Approach. Cleft Palate Craniofac J. 57(12):1422–1427, 2020 4- Moreno SD, Christopher P, Kloostra P. Congenital Midline Cervical Cleft (CMCC): Z-Plasty Versus Linear Cutaneous Repair. J Craniofac Surg. 34(1):e38–e41, 2023 5- Magalhães R, Louro M, Forny D, Sá Á, Franco D. Congenital midline cervical cleft: Management of a case series and literature review. J Plast Reconstr Aesthet Surg. 93:117–126, 2024 6- Hwang JC, Perry R. Midline Cervical Cleft: Case Report and Current Understanding. Cleft Palate Craniofac J. 62(12):2180–2186, 2025

Chance Fracture

A Chance fracture is an unstable spinal injury first described in 1948 as a distinctive flexion-distraction pattern that splits the vertebra horizontally. What makes this fracture conceptually important is that it involves all three columns of the spine as defined by the three-column model. The anterior column comprises the anterior longitudinal ligament, the anterior annulus, and the anterior two-thirds of the vertebral body; the middle column includes the posterior third of the vertebral body, the posterior annulus, and the posterior longitudinal ligament; and the posterior column encompasses the posterior bony elements and the posterior ligamentous complex. Because an injury involving at least two of these three columns is considered mechanically unstable, and because a Chance fracture typically produces a distraction injury of the middle and posterior columns with or without an anterior compression component, it is by definition an unstable lesion. The horizontal fracture line propagates from posterior to anterior, coursing through the spinous process, lamina, pedicles, and into the vertebral body, without lateral displacement or rotation of the fragments. The mechanism is a sudden deceleration of the trunk against a fixed point, most classically the lap portion of a seat belt during a motor vehicle collision. When the vehicle decelerates abruptly, the restrained pelvis remains anchored while the upper body is thrown violently forward, forcing the spine to hyperflex over the belt, which acts as a fulcrum. This generates tension across the posterior elements and, in more severe cases, compression of the anterior vertebral body. The association with lap belts, recognized in the 1960s, gave the injury its alternate name of "seat belt fracture." Although motor vehicle collisions dominate, the same flexion-distraction forces can arise from falls from height, assaults, extreme sports such as rodeo and snowboarding, and military blast events involving armored vehicles. A less familiar variant occurs iatrogenically at the ends of long spinal fusion constructs, where the pedicles split along their longitudinal axis into cranial and caudal fragments, producing proximal or distal junctional failure without any antecedent trauma. Epidemiologically, thoracolumbar spine fractures are common, and roughly 2% of blunt trauma patients sustain one. The thoracolumbar junction is the second most frequent site of spinal injury after the cervical spine. Chance fractures classically cluster at the T10–L2 transition in adults, whereas in children they tend to occur in the mid-lumbar region. There is a male predominance and a tendency to affect children and young adults, with reported mean ages in the mid-twenties. The location difference between age groups has direct clinical consequences: children are more likely to fracture L1–L3, while adults more often fracture the thoracic spine, including levels such as T4–T6 and T10–T12 that are rarely involved in children. The most clinically dangerous feature of Chance fractures is their strong association with intra-abdominal injury. The same belt that fractures the spine crushes the viscera between the restraint and the vertebral column, and the reported incidence of concomitant abdominal injury ranges widely from roughly a third to nearly nine out of ten cases, commonly cited as high as 50%. The most feared associated injuries are hollow viscus perforations and mesenteric lacerations, both of which carry substantial mortality. This risk is markedly higher in children. When a Chance fracture is present, pediatric patients have a concomitant intra-abdominal organ injury far more often than adults—about 63% versus 23%—and hollow viscus injury in particular is dramatically more frequent, roughly 51% versus 8%. Interestingly, rates of solid organ injury are nearly identical between children and adults, so the excess risk in children is driven specifically by bowel and mesenteric injury. This pattern holds even when the analysis is restricted to collisions alone, which argues that the difference reflects anatomy rather than mechanism. The likely explanation lies in belt positioning and body proportions: children's belts frequently ride too high across the abdomen rather than the pelvis, creating a fixation point over soft viscera, and the pediatric abdomen is more compliant with underdeveloped iliac crests. The higher rate of lumbar fractures in children mirrors this higher-riding belt. The practical result is a much greater need for surgical abdominal intervention in children. Clinically, the injury is treacherous because patients typically present with back pain but no neurological deficit, so the fracture can be overlooked. Neurological signs appear only when there is cord or cauda equina compromise. A critical physical finding is the "seatbelt sign"—linear bruising or abrasion across the abdomen in the belt's distribution—which should sharply raise suspicion for both the fracture and underlying visceral injury, and which can also appear on CT as fat stranding in the anterior abdominal wall. The great danger is delayed presentation of bowel injury. Several documented cases illustrate a patient arriving hemodynamically stable with only subtle initial imaging findings, then deteriorating hours later as a bowel perforation or complete transection declares itself. Peritoneal signs develop slowly with small bowel injury because luminal contents are relatively neutral and bacterial load is low; a rising heart rate as part of the systemic inflammatory response may be the first physical clue. For this reason, close serial monitoring of vital signs, laboratory values, and abdominal examination is essential, with repeat CT recommended when the picture is equivocal or the patient worsens, and exploratory laparoscopy or laparotomy when perforation is suspected. In the context of fever, abdominal pain, and tachycardia in a child with a Chance fracture, the appropriate action is to proceed to the operating room out of concern for hollow viscus injury. For diagnosis, CT is the imaging modality of choice in patients sixteen and older, superior to radiography for detecting the fracture and for evaluating associated abdominal injury; coronal and sagittal reformations are essential given the horizontal orientation. In younger children, radiography is often the recommended first study, though CT obtained for abdominal evaluation typically suffices. MRI outperforms CT for soft tissue and ligamentous injury and should be obtained when ligamentous disruption or cord compromise is suspected, because purely ligamentous Chance injuries are much harder to detect and more likely to be missed. A sternal fracture is an additional sign of instability. Management depends on fracture displacement and the nature of the injury. A purely osseous, non-displaced fracture without neurological deficit can be treated conservatively with a rigid brace or hyperextension cast, typically for eight to twelve weeks, with high union rates. Displaced fractures with a significant bony gap, neurological deficit, or ligamentous involvement require surgery, because soft tissue injuries will not heal with bracing and instability will persist. The mainstay of surgical treatment is posterior pedicle screw fixation to reconstruct the posterior tension band, achieving realignment, reduction, and stabilization; this may be performed open or percutaneously, with fusion reserved as the final goal when ligamentous injury is present. Reduction of kyphosis is important to prevent painful post-traumatic sagittal malalignment. Prognosis correlates with the degree of kyphosis, and outcomes after surgical stabilization are good in the great majority of patients, though residual low back pain remains a common long-term complaint. Ultimately, optimal care depends on an interprofessional team maintaining a high index of suspicion so that neither the fracture nor its dangerous abdominal companions are overlooked. References: 1- Bourghli A, Obeid I, Boissiere L, Vital JM, Tabboush Z, Al Sarawan M. Management of a high thoracic chance fracture. Eur Spine J. 27(7):1547–1552, 2018 2- Hazen BJ, Keane OA, Vandewalle RJ, Grady Z, Wetzel M, Chern JJ, Santore MT. Difference in Presentation and Concomitant Intra-Abdominal Injury with Chance Fracture in Pediatric and Adult Populations. Am Surg. 89(6):2486–2491, 2023 3- Comadoll SM, Holton KJ, Polly DW Jr, Schmitz MW, Haselhuhn JJ, Soriano PBO, Martin CT, Jones KE, Sembrano JN. Chance Fracture Pattern Presenting in Proximal Junctional Failure. J Am Acad Orthop Surg Glob Res Rev. 7(8):e23.00039, 2023 4- Koay J, Davis DD, Hogg JP. Chance Fractures. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Aug 14. 5- Destinval C, Larmure O, Journeau P, Lemelle JL. Seat belt syndrome with caecal perforation and Chance fracture in an adolescent. BMJ Case Rep. 16(10):e254631, 2023 6- Huang CK, Lee CC, Kwok CM. Chance fracture at L2 followed by delayed jejunal transection due to a motor vehicle collision: A case report and review. Trauma Case Rep. 48:100968, 2023

Minimally Invasive Repair Pectus Excavatum

Pectus excavatum is the most common congenital chest wall deformity, characterized by a posterior depression of the sternum and adjacent costal cartilages that gives the chest a sunken appearance. It occurs in approximately one in every 300 to 400 live births and shows a marked male predominance, affecting boys roughly four to five times more often than girls. This apparent sex disparity may be partly artificial, as breast tissue can conceal the severity of the defect in female patients, leading to underdiagnosis. The deformity typically becomes more pronounced during the pubertal growth spurt, when rapid skeletal development accentuates the depression, and the chest wall becomes progressively less flexible as the patient ages. The clinical significance of pectus excavatum spans a spectrum from a purely cosmetic concern to a source of meaningful cardiopulmonary compromise. The inward displacement of the sternum most commonly compresses the right-sided chambers of the heart, displacing the heart toward the left chest and reducing stroke volume, cardiac output, and both diastolic and systolic function. Many patients report exercise intolerance, fatigue, dyspnea on exertion, palpitations, and chest pain, and functional testing frequently demonstrates reduced maximal oxygen consumption. Historically the condition was dismissed as a cosmetic problem alone, but accumulating evidence now shows that cardiac performance may be reduced measurably and that surgical correction can restore normalized cardiopulmonary function. Importantly, the psychosocial burden should not be discounted; body image concerns, diminished self-confidence, social avoidance, anxiety, and depression are all well documented, and quality-of-life measures improve substantially following repair. For roughly half a century, surgical correction relied on open techniques involving resection of the deformed costal cartilages, sometimes combined with a sternal osteotomy, to reposition the sternum. This open approach, refined over decades, remained the standard until the late 1990s, when a minimally invasive technique was introduced. This method requires no cartilage resection or sternal osteotomy. Instead, a curved metal bar is passed behind the sternum under thoracoscopic guidance and rotated so that its convexity elevates the depressed area from within. The bar is secured to the chest wall and left in place for a period of years while the chest remodels, after which it is removed. The minimally invasive procedure rapidly became the standard of care, particularly for pediatric and adolescent patients, owing to its smaller incisions, shorter operative time, minimal blood loss, and excellent cosmetic outcomes. Its adoption fueled a dramatic rise in the number of patients seeking correction, driven in part by greater awareness spread through the internet. Preoperative assessment combines clinical examination with imaging. Thoracic imaging, using either computed tomography or magnetic resonance imaging, allows measurement of the Haller index, calculated by dividing the transverse diameter of the chest by the anteroposterior distance between the sternum and spine at the deepest point. A value above 3.2 to 3.25 has traditionally justified surgical repair, though this threshold has notable limitations. Abnormal values can appear in otherwise normal chests, and some severely affected patients fall below the cutoff, so the index does not correlate reliably with physiologic symptoms. The correction index has emerged as a more accurate estimate of severity. Additional evaluation may include electrocardiography, echocardiography, pulmonary function testing, and cardiopulmonary exercise testing, particularly when comorbidity such as a connective tissue disorder is suspected or when the case is more complex. Metal allergy screening is advisable when there is a personal or family history of allergic reactions, since the standard bars contain nickel and other components that can provoke sensitization. The optimal timing of surgery remains debated. Many surgeons prefer to operate near the onset of puberty, when the chest wall remains soft, elastic, and easily manipulated, with broad agreement that the best results are obtained between roughly 13 and 16 years of age. Operating too early carries a higher risk of recurrence if the bar is removed before puberty, while adult repair, though feasible, is technically more demanding. The calcification and rigidity that accompany aging make sternal elevation more difficult, increase the force distributed to the bars, and raise the risk of bar displacement. Nevertheless, successful repair has been reported in patients well into their seventies, and adult correction continues to increase, with symptom resolution and satisfying cosmetic results achievable when appropriate technical modifications are applied. Several modifications have improved the safety and durability of the procedure. Routine thoracoscopy provides direct visualization during the retrosternal dissection and has become widely accepted as a minimum standard to reduce cardiac injury, the most feared and potentially lethal complication. Cardiac perforation is most likely during dissection in patients with previous open cardiac surgery, in whom dense adhesions bind the sternum to the myocardium, and in those with very deep or stiff chests. Sternal elevation techniques—including crane systems, retractors, subxiphoid approaches, hooks, and the vacuum bell applied intraoperatively—expand the retrosternal space and facilitate safe bar passage, further decreasing the incidence of near-fatal events. The use of multiple bars distributes corrective forces across a broader area, improves correction in stiff or complex chests, and reduces displacement. The cross-bar technique, in which two bars are crossed beneath the sternum with their lateral ends resting on the lower rib cage, is particularly useful for severe deformities with costal flaring, avoiding lateral chest wall depression while correcting flaring. Bar stability depends heavily on correct positioning. The bar must enter and exit the chest medial to the highest point of the deformity; if placed too laterally, it fails to elevate the sternum and may strip the intercostal muscles, leading to instability and recurrence. Numerous fixation strategies—lateral and medial stabilizers, pericostal sutures, multipoint suture fixation, hinge plates, and claw fixators—have reduced the rate of bar displacement from historical highs to around one percent. Shorter bars have also been adopted to reduce the risk of flipping. Complications, though relatively uncommon in experienced hands, span a range of severity. Pleural effusion and pneumothorax are almost universally present but rarely require intervention. Bar displacement, once the most common late complication, has fallen dramatically with improved fixation. Overcorrection into a carinatum shape, metal allergy, wound infections, and, rarely, life-threatening hemorrhage from mediastinal or internal mammary vessels can occur. Notably, serious complications can arise not only during placement but also during bar removal, which should therefore be performed in a setting equipped to manage catastrophic bleeding. Recurrence rates remain low when bars are left in place for an adequate interval, generally two to three years or longer in complex cases. Postoperative pain is the defining challenge of this operation. Paradoxically, despite its minimally invasive nature, the procedure may cause more pain than its open counterpart, because the substernal bar exerts sustained upward force on an intact chest wall and acutely stretches the intercostal nerves until bone and cartilage remodel. Pain and opioid-related side effects are the principal determinants of hospital length of stay, making effective analgesia central to recovery. Traditional approaches relied on thoracic epidural analgesia and patient-controlled analgesia, but these have limitations including variable efficacy, prolonged stays, and complications. Modern practice emphasizes multimodal, opioid-sparing strategies. Regional techniques—including paravertebral, erector spinae plane, serratus anterior plane, and intercostal nerve blocks—and non-opioid adjuncts such as acetaminophen, nonsteroidal anti-inflammatory drugs, gabapentinoids, ketamine, dexmedetomidine, and methadone all contribute. The most transformative advance has been intercostal nerve cryoablation, which induces temporary degeneration of the intercostal nerve axons, halting pain transmission for weeks until regeneration occurs. Cryoablation has been shown to reduce hospital stay substantially—in one randomized trial from around five days to three—and to decrease opioid consumption compared with epidural analgesia. Its main limitation is a delayed onset of up to a day, so it is best combined with a direct-acting regional block or systemic analgesia to bridge the early postoperative period. When cryoablation is paired with an adjunct analgesic intervention, it appears superior to all other modalities in shortening hospitalization, and refined multimodal protocols have made same-day discharge feasible for a majority of patients. Neuropathic pain is a recognized risk, more common in adults than children, but rarely requires treatment. The incorporation of these techniques into enhanced recovery pathways—combining preoperative education, multimodal analgesia, early mobilization, early oral intake, and prompt removal of catheters—has standardized care and improved outcomes. Even so, roughly one-third of patients require a short course of opioids in the immediate postoperative period. Together, these surgical and analgesic refinements have made minimally invasive repair of pectus excavatum a safe, effective, and increasingly well-tolerated operation with excellent long-term cosmetic and functional results. References: 1- Pilegaard H, Licht PB. Minimal Invasive Repair of Pectus Excavatum and Carinatum. Thorac Surg Clin. 27(2):123-131, 2017 2- Goretsky MJ, McGuire MM. Complications associated with the minimally invasive repair of pectus excavatum. Semin Pediatr Surg. 27(3):151-155, 2018 3- Haecker FM, Krebs TF, Kleitsch KU. Current Development of Minimally Invasive Repair of Pectus Excavatum (MIRPE). Children (Basel). 9(4):478, 2022 4- Aly MR, Farina JM, Botros MM, Jaroszewski DE. Minimally invasive repair of pectus excavatum in adults: a review article of presentation, workup, and surgical treatment. J Thorac Dis. 15(9):5150-5173, 2023 5- Brussels AR, Kim MS. Perioperative considerations in anesthesia for minimally invasive repair of pectus excavatum, Nuss procedure. Semin Pediatr Surg. 33(5):151459, 2024 6- Chiu MZ, Li R, Koka A, Demehri FR. Pain management after pediatric minimally invasive repair of pectus excavatum: a narrative review. Transl Pediatr. 13(12):2267-2281, 2024 7- Van Polen EJ, Franssen CJ, Daemen JHT, Isabella AJ, Franssen AJPM, Hulsewé KWE, Vissers YLJ, de Loos ER. Postoperative Pain Management After Minimally Invasive Repair of Pectus Excavatum: A Systematic Review and Network Meta-analysis. J Pediatr Surg. 60(6):162282, 2025

 

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